ByBy

Ahmed BakhshAhmed Bakhshahmedbakhshgmailcomahmedbakhshgmailcom

Pseudotumor cerebriPseudotumor cerebri

Pseudotumor cerebri

Syndrome of raised intracranial pressure Syndrome of raised intracranial pressure without any without any clinicalclinical laboratory laboratory radiological evidence of radiological evidence of intracranial pathologyintracranial pathology Presents with symptoms of increased ICPPresents with symptoms of increased ICP headacheheadache pulsatile tinnituspulsatile tinnitus transitory visual obscuration transitory visual obscuration diplopiadiplopia

23-05-01 Bakhsh A 2

Obese femalesObese females Intractable headachesIntractable headaches vision problems vision problems PapilledemaPapilledema Think of Think of Pseudotumor cerebri

3Bakhsh A23-05-01

Pseudotumor cerebri

Syndrome of raised intracranial pressure Syndrome of raised intracranial pressure without any without any clinicalclinical laboratory laboratory radiological evidence of radiological evidence of intracranial pathologyintracranial pathology Presents with symptoms of increased ICPPresents with symptoms of increased ICP headacheheadache pulsatile tinnituspulsatile tinnitus transitory visual obscuration transitory visual obscuration diplopiadiplopia

23-05-01 Bakhsh A 2

Obese femalesObese females Intractable headachesIntractable headaches vision problems vision problems PapilledemaPapilledema Think of Think of Pseudotumor cerebri

3Bakhsh A23-05-01

Obese femalesObese females Intractable headachesIntractable headaches vision problems vision problems PapilledemaPapilledema Think of Think of Pseudotumor cerebri

3Bakhsh A23-05-01

23-05-01 Bakhsh A 4

5Bakhsh A23-05-01

6Bakhsh A23-05-01

USAUSA 09 to 10 100000 in general population09 to 10 100000 in general population 16-35 100000 in women 16-35 100000 in women 79-20 100000 in overweight women 79-20 100000 in overweight women UKUK 156100000year156100000year 286 100000 in women 286 100000 in women 119100000 in obese women119100000 in obese women

7Bakhsh A23-05-01

LibyaLibya 22100000 in general population 22100000 in general population 12100000 in women aged 15ndash44 years 12100000 in women aged 15ndash44 years 214 100000 in obese women 214 100000 in obese women OmanOman 218100000 in general population218100000 in general population 325100000 women in all age groups 325100000 women in all age groups 414 100000 in the age group of 15ndash44 years414 100000 in the age group of 15ndash44 years IsraelIsrael 202100000 in general population 202100000 in general population 317100000 in women 317100000 in women 549100000 in reproductive age group549100000 in reproductive age group

Sumayya J et el Sumayya J et el Idiopathic intracranial hypertension in the Idiopathic intracranial hypertension in the Middle East A growing concern Middle East A growing concern Saudi Journal of Ophthalmology Saudi Journal of Ophthalmology (2015) 29 26ndash31(2015) 29 26ndash31

23-05-01 Bakhsh A 8

Meningitis serosa Quincke 1893Meningitis serosa Quincke 1893

Pseudotumor cerebriPseudotumor cerebri Nonne 1904 Nonne 1904

Benign intracranial hypertensionBenign intracranial hypertension Foley 1955Foley 1955

Idiopathic intracranial hypertensionIdiopathic intracranial hypertension Corbett 1989 Corbett 1989

Primary intracranial hypertensionPrimary intracranial hypertension

Secondary intracranial hypertensionSecondary intracranial hypertension

9Bakhsh A23-05-01

Anemia Anemia Sleep ApneaSleep Apnea HypertensionHypertension HypoparathyridismHypoparathyridism Chronic renal failureChronic renal failure Cushings amp AddisonsCushings amp Addisons

23-05-01 Bakhsh A 10

Tetracycline MinocyclineTetracycline Minocycline Anabolic steroidsAnabolic steroids Growth hormoneGrowth hormone Nitrofurantoin Nitrofurantoin Nalidixic acidNalidixic acid IsotretinoinIsotretinoin TamoxifenTamoxifen Vitamin AVitamin A LithiumLithium Steroid Steroid

23-05-01 Bakhsh A 11

12Bakhsh A23-05-01

13Bakhsh A23-05-01

Priapism is pathological Priapism is pathological elevation of venouselevation of venouspressure pressure of the male genitalia due to venous of the male genitalia due to venous out flow out flow obstructionobstruction or or compressioncompression

Pathophysiology of idiopathic intracranialPathophysiology of idiopathic intracranialhypertension may be analogous to that of priapismhypertension may be analogous to that of priapism

Bateman GA1Idiopathic intracranial hypertension priapism of Idiopathic intracranial hypertension priapism of the brainthe brain Med Hypotheses 200463(3)549-52 200463(3)549-52

23-05-01 Bakhsh A 14

5Bakhsh A23-05-01

6Bakhsh A23-05-01

USAUSA 09 to 10 100000 in general population09 to 10 100000 in general population 16-35 100000 in women 16-35 100000 in women 79-20 100000 in overweight women 79-20 100000 in overweight women UKUK 156100000year156100000year 286 100000 in women 286 100000 in women 119100000 in obese women119100000 in obese women

7Bakhsh A23-05-01

LibyaLibya 22100000 in general population 22100000 in general population 12100000 in women aged 15ndash44 years 12100000 in women aged 15ndash44 years 214 100000 in obese women 214 100000 in obese women OmanOman 218100000 in general population218100000 in general population 325100000 women in all age groups 325100000 women in all age groups 414 100000 in the age group of 15ndash44 years414 100000 in the age group of 15ndash44 years IsraelIsrael 202100000 in general population 202100000 in general population 317100000 in women 317100000 in women 549100000 in reproductive age group549100000 in reproductive age group

Sumayya J et el Sumayya J et el Idiopathic intracranial hypertension in the Idiopathic intracranial hypertension in the Middle East A growing concern Middle East A growing concern Saudi Journal of Ophthalmology Saudi Journal of Ophthalmology (2015) 29 26ndash31(2015) 29 26ndash31

23-05-01 Bakhsh A 8

Meningitis serosa Quincke 1893Meningitis serosa Quincke 1893

Pseudotumor cerebriPseudotumor cerebri Nonne 1904 Nonne 1904

Benign intracranial hypertensionBenign intracranial hypertension Foley 1955Foley 1955

Idiopathic intracranial hypertensionIdiopathic intracranial hypertension Corbett 1989 Corbett 1989

Primary intracranial hypertensionPrimary intracranial hypertension

Secondary intracranial hypertensionSecondary intracranial hypertension

9Bakhsh A23-05-01

Anemia Anemia Sleep ApneaSleep Apnea HypertensionHypertension HypoparathyridismHypoparathyridism Chronic renal failureChronic renal failure Cushings amp AddisonsCushings amp Addisons

23-05-01 Bakhsh A 10

Tetracycline MinocyclineTetracycline Minocycline Anabolic steroidsAnabolic steroids Growth hormoneGrowth hormone Nitrofurantoin Nitrofurantoin Nalidixic acidNalidixic acid IsotretinoinIsotretinoin TamoxifenTamoxifen Vitamin AVitamin A LithiumLithium Steroid Steroid

23-05-01 Bakhsh A 11

12Bakhsh A23-05-01

13Bakhsh A23-05-01

Priapism is pathological Priapism is pathological elevation of venouselevation of venouspressure pressure of the male genitalia due to venous of the male genitalia due to venous out flow out flow obstructionobstruction or or compressioncompression

Pathophysiology of idiopathic intracranialPathophysiology of idiopathic intracranialhypertension may be analogous to that of priapismhypertension may be analogous to that of priapism

Bateman GA1Idiopathic intracranial hypertension priapism of Idiopathic intracranial hypertension priapism of the brainthe brain Med Hypotheses 200463(3)549-52 200463(3)549-52

23-05-01 Bakhsh A 14

6Bakhsh A23-05-01

USAUSA 09 to 10 100000 in general population09 to 10 100000 in general population 16-35 100000 in women 16-35 100000 in women 79-20 100000 in overweight women 79-20 100000 in overweight women UKUK 156100000year156100000year 286 100000 in women 286 100000 in women 119100000 in obese women119100000 in obese women

7Bakhsh A23-05-01

LibyaLibya 22100000 in general population 22100000 in general population 12100000 in women aged 15ndash44 years 12100000 in women aged 15ndash44 years 214 100000 in obese women 214 100000 in obese women OmanOman 218100000 in general population218100000 in general population 325100000 women in all age groups 325100000 women in all age groups 414 100000 in the age group of 15ndash44 years414 100000 in the age group of 15ndash44 years IsraelIsrael 202100000 in general population 202100000 in general population 317100000 in women 317100000 in women 549100000 in reproductive age group549100000 in reproductive age group

Sumayya J et el Sumayya J et el Idiopathic intracranial hypertension in the Idiopathic intracranial hypertension in the Middle East A growing concern Middle East A growing concern Saudi Journal of Ophthalmology Saudi Journal of Ophthalmology (2015) 29 26ndash31(2015) 29 26ndash31

23-05-01 Bakhsh A 8

Meningitis serosa Quincke 1893Meningitis serosa Quincke 1893

Pseudotumor cerebriPseudotumor cerebri Nonne 1904 Nonne 1904

Benign intracranial hypertensionBenign intracranial hypertension Foley 1955Foley 1955

Idiopathic intracranial hypertensionIdiopathic intracranial hypertension Corbett 1989 Corbett 1989

Primary intracranial hypertensionPrimary intracranial hypertension

Secondary intracranial hypertensionSecondary intracranial hypertension

9Bakhsh A23-05-01

Anemia Anemia Sleep ApneaSleep Apnea HypertensionHypertension HypoparathyridismHypoparathyridism Chronic renal failureChronic renal failure Cushings amp AddisonsCushings amp Addisons

23-05-01 Bakhsh A 10

Tetracycline MinocyclineTetracycline Minocycline Anabolic steroidsAnabolic steroids Growth hormoneGrowth hormone Nitrofurantoin Nitrofurantoin Nalidixic acidNalidixic acid IsotretinoinIsotretinoin TamoxifenTamoxifen Vitamin AVitamin A LithiumLithium Steroid Steroid

23-05-01 Bakhsh A 11

12Bakhsh A23-05-01

13Bakhsh A23-05-01

Priapism is pathological Priapism is pathological elevation of venouselevation of venouspressure pressure of the male genitalia due to venous of the male genitalia due to venous out flow out flow obstructionobstruction or or compressioncompression

Pathophysiology of idiopathic intracranialPathophysiology of idiopathic intracranialhypertension may be analogous to that of priapismhypertension may be analogous to that of priapism

Bateman GA1Idiopathic intracranial hypertension priapism of Idiopathic intracranial hypertension priapism of the brainthe brain Med Hypotheses 200463(3)549-52 200463(3)549-52

23-05-01 Bakhsh A 14

USAUSA 09 to 10 100000 in general population09 to 10 100000 in general population 16-35 100000 in women 16-35 100000 in women 79-20 100000 in overweight women 79-20 100000 in overweight women UKUK 156100000year156100000year 286 100000 in women 286 100000 in women 119100000 in obese women119100000 in obese women

7Bakhsh A23-05-01

LibyaLibya 22100000 in general population 22100000 in general population 12100000 in women aged 15ndash44 years 12100000 in women aged 15ndash44 years 214 100000 in obese women 214 100000 in obese women OmanOman 218100000 in general population218100000 in general population 325100000 women in all age groups 325100000 women in all age groups 414 100000 in the age group of 15ndash44 years414 100000 in the age group of 15ndash44 years IsraelIsrael 202100000 in general population 202100000 in general population 317100000 in women 317100000 in women 549100000 in reproductive age group549100000 in reproductive age group

Sumayya J et el Sumayya J et el Idiopathic intracranial hypertension in the Idiopathic intracranial hypertension in the Middle East A growing concern Middle East A growing concern Saudi Journal of Ophthalmology Saudi Journal of Ophthalmology (2015) 29 26ndash31(2015) 29 26ndash31

23-05-01 Bakhsh A 8

Meningitis serosa Quincke 1893Meningitis serosa Quincke 1893

Pseudotumor cerebriPseudotumor cerebri Nonne 1904 Nonne 1904

Benign intracranial hypertensionBenign intracranial hypertension Foley 1955Foley 1955

Idiopathic intracranial hypertensionIdiopathic intracranial hypertension Corbett 1989 Corbett 1989

Primary intracranial hypertensionPrimary intracranial hypertension

Secondary intracranial hypertensionSecondary intracranial hypertension

9Bakhsh A23-05-01

Anemia Anemia Sleep ApneaSleep Apnea HypertensionHypertension HypoparathyridismHypoparathyridism Chronic renal failureChronic renal failure Cushings amp AddisonsCushings amp Addisons

23-05-01 Bakhsh A 10

Tetracycline MinocyclineTetracycline Minocycline Anabolic steroidsAnabolic steroids Growth hormoneGrowth hormone Nitrofurantoin Nitrofurantoin Nalidixic acidNalidixic acid IsotretinoinIsotretinoin TamoxifenTamoxifen Vitamin AVitamin A LithiumLithium Steroid Steroid

23-05-01 Bakhsh A 11

12Bakhsh A23-05-01

13Bakhsh A23-05-01

Priapism is pathological Priapism is pathological elevation of venouselevation of venouspressure pressure of the male genitalia due to venous of the male genitalia due to venous out flow out flow obstructionobstruction or or compressioncompression

Pathophysiology of idiopathic intracranialPathophysiology of idiopathic intracranialhypertension may be analogous to that of priapismhypertension may be analogous to that of priapism

Bateman GA1Idiopathic intracranial hypertension priapism of Idiopathic intracranial hypertension priapism of the brainthe brain Med Hypotheses 200463(3)549-52 200463(3)549-52

23-05-01 Bakhsh A 14

LibyaLibya 22100000 in general population 22100000 in general population 12100000 in women aged 15ndash44 years 12100000 in women aged 15ndash44 years 214 100000 in obese women 214 100000 in obese women OmanOman 218100000 in general population218100000 in general population 325100000 women in all age groups 325100000 women in all age groups 414 100000 in the age group of 15ndash44 years414 100000 in the age group of 15ndash44 years IsraelIsrael 202100000 in general population 202100000 in general population 317100000 in women 317100000 in women 549100000 in reproductive age group549100000 in reproductive age group

Sumayya J et el Sumayya J et el Idiopathic intracranial hypertension in the Idiopathic intracranial hypertension in the Middle East A growing concern Middle East A growing concern Saudi Journal of Ophthalmology Saudi Journal of Ophthalmology (2015) 29 26ndash31(2015) 29 26ndash31

23-05-01 Bakhsh A 8

Meningitis serosa Quincke 1893Meningitis serosa Quincke 1893

Pseudotumor cerebriPseudotumor cerebri Nonne 1904 Nonne 1904

Benign intracranial hypertensionBenign intracranial hypertension Foley 1955Foley 1955

Idiopathic intracranial hypertensionIdiopathic intracranial hypertension Corbett 1989 Corbett 1989

Primary intracranial hypertensionPrimary intracranial hypertension

Secondary intracranial hypertensionSecondary intracranial hypertension

9Bakhsh A23-05-01

Anemia Anemia Sleep ApneaSleep Apnea HypertensionHypertension HypoparathyridismHypoparathyridism Chronic renal failureChronic renal failure Cushings amp AddisonsCushings amp Addisons

23-05-01 Bakhsh A 10

Tetracycline MinocyclineTetracycline Minocycline Anabolic steroidsAnabolic steroids Growth hormoneGrowth hormone Nitrofurantoin Nitrofurantoin Nalidixic acidNalidixic acid IsotretinoinIsotretinoin TamoxifenTamoxifen Vitamin AVitamin A LithiumLithium Steroid Steroid

23-05-01 Bakhsh A 11

12Bakhsh A23-05-01

13Bakhsh A23-05-01

Priapism is pathological Priapism is pathological elevation of venouselevation of venouspressure pressure of the male genitalia due to venous of the male genitalia due to venous out flow out flow obstructionobstruction or or compressioncompression

Pathophysiology of idiopathic intracranialPathophysiology of idiopathic intracranialhypertension may be analogous to that of priapismhypertension may be analogous to that of priapism

Bateman GA1Idiopathic intracranial hypertension priapism of Idiopathic intracranial hypertension priapism of the brainthe brain Med Hypotheses 200463(3)549-52 200463(3)549-52

23-05-01 Bakhsh A 14

Meningitis serosa Quincke 1893Meningitis serosa Quincke 1893

Pseudotumor cerebriPseudotumor cerebri Nonne 1904 Nonne 1904

Benign intracranial hypertensionBenign intracranial hypertension Foley 1955Foley 1955

Idiopathic intracranial hypertensionIdiopathic intracranial hypertension Corbett 1989 Corbett 1989

Primary intracranial hypertensionPrimary intracranial hypertension

Secondary intracranial hypertensionSecondary intracranial hypertension

9Bakhsh A23-05-01

Anemia Anemia Sleep ApneaSleep Apnea HypertensionHypertension HypoparathyridismHypoparathyridism Chronic renal failureChronic renal failure Cushings amp AddisonsCushings amp Addisons

23-05-01 Bakhsh A 10

Tetracycline MinocyclineTetracycline Minocycline Anabolic steroidsAnabolic steroids Growth hormoneGrowth hormone Nitrofurantoin Nitrofurantoin Nalidixic acidNalidixic acid IsotretinoinIsotretinoin TamoxifenTamoxifen Vitamin AVitamin A LithiumLithium Steroid Steroid

23-05-01 Bakhsh A 11

12Bakhsh A23-05-01

13Bakhsh A23-05-01

Priapism is pathological Priapism is pathological elevation of venouselevation of venouspressure pressure of the male genitalia due to venous of the male genitalia due to venous out flow out flow obstructionobstruction or or compressioncompression

Pathophysiology of idiopathic intracranialPathophysiology of idiopathic intracranialhypertension may be analogous to that of priapismhypertension may be analogous to that of priapism

Bateman GA1Idiopathic intracranial hypertension priapism of Idiopathic intracranial hypertension priapism of the brainthe brain Med Hypotheses 200463(3)549-52 200463(3)549-52

23-05-01 Bakhsh A 14

Anemia Anemia Sleep ApneaSleep Apnea HypertensionHypertension HypoparathyridismHypoparathyridism Chronic renal failureChronic renal failure Cushings amp AddisonsCushings amp Addisons

23-05-01 Bakhsh A 10

Tetracycline MinocyclineTetracycline Minocycline Anabolic steroidsAnabolic steroids Growth hormoneGrowth hormone Nitrofurantoin Nitrofurantoin Nalidixic acidNalidixic acid IsotretinoinIsotretinoin TamoxifenTamoxifen Vitamin AVitamin A LithiumLithium Steroid Steroid

23-05-01 Bakhsh A 11

12Bakhsh A23-05-01

13Bakhsh A23-05-01

Priapism is pathological Priapism is pathological elevation of venouselevation of venouspressure pressure of the male genitalia due to venous of the male genitalia due to venous out flow out flow obstructionobstruction or or compressioncompression

Pathophysiology of idiopathic intracranialPathophysiology of idiopathic intracranialhypertension may be analogous to that of priapismhypertension may be analogous to that of priapism

Bateman GA1Idiopathic intracranial hypertension priapism of Idiopathic intracranial hypertension priapism of the brainthe brain Med Hypotheses 200463(3)549-52 200463(3)549-52

23-05-01 Bakhsh A 14

Tetracycline MinocyclineTetracycline Minocycline Anabolic steroidsAnabolic steroids Growth hormoneGrowth hormone Nitrofurantoin Nitrofurantoin Nalidixic acidNalidixic acid IsotretinoinIsotretinoin TamoxifenTamoxifen Vitamin AVitamin A LithiumLithium Steroid Steroid

23-05-01 Bakhsh A 11

12Bakhsh A23-05-01

13Bakhsh A23-05-01

Priapism is pathological Priapism is pathological elevation of venouselevation of venouspressure pressure of the male genitalia due to venous of the male genitalia due to venous out flow out flow obstructionobstruction or or compressioncompression

Pathophysiology of idiopathic intracranialPathophysiology of idiopathic intracranialhypertension may be analogous to that of priapismhypertension may be analogous to that of priapism

Bateman GA1Idiopathic intracranial hypertension priapism of Idiopathic intracranial hypertension priapism of the brainthe brain Med Hypotheses 200463(3)549-52 200463(3)549-52

23-05-01 Bakhsh A 14

12Bakhsh A23-05-01

13Bakhsh A23-05-01

Priapism is pathological Priapism is pathological elevation of venouselevation of venouspressure pressure of the male genitalia due to venous of the male genitalia due to venous out flow out flow obstructionobstruction or or compressioncompression

Pathophysiology of idiopathic intracranialPathophysiology of idiopathic intracranialhypertension may be analogous to that of priapismhypertension may be analogous to that of priapism

Bateman GA1Idiopathic intracranial hypertension priapism of Idiopathic intracranial hypertension priapism of the brainthe brain Med Hypotheses 200463(3)549-52 200463(3)549-52

23-05-01 Bakhsh A 14

13Bakhsh A23-05-01

Priapism is pathological Priapism is pathological elevation of venouselevation of venouspressure pressure of the male genitalia due to venous of the male genitalia due to venous out flow out flow obstructionobstruction or or compressioncompression

Pathophysiology of idiopathic intracranialPathophysiology of idiopathic intracranialhypertension may be analogous to that of priapismhypertension may be analogous to that of priapism

Bateman GA1Idiopathic intracranial hypertension priapism of Idiopathic intracranial hypertension priapism of the brainthe brain Med Hypotheses 200463(3)549-52 200463(3)549-52

23-05-01 Bakhsh A 14

Priapism is pathological Priapism is pathological elevation of venouselevation of venouspressure pressure of the male genitalia due to venous of the male genitalia due to venous out flow out flow obstructionobstruction or or compressioncompression

Pathophysiology of idiopathic intracranialPathophysiology of idiopathic intracranialhypertension may be analogous to that of priapismhypertension may be analogous to that of priapism

Bateman GA1Idiopathic intracranial hypertension priapism of Idiopathic intracranial hypertension priapism of the brainthe brain Med Hypotheses 200463(3)549-52 200463(3)549-52

23-05-01 Bakhsh A 14

23-05-01 Bakhsh A 15

23-05-01 Bakhsh A 16

1)Dandy WE 1)Dandy WE Intracranial pressure without brain tumormdashdiagnosis and treatment Ann Surg 1937106492ndash513Ann Surg 1937106492ndash513

2) Smith JLSmith JL Whence pseudotumor cerebri J Clin J Clin Neuroophthalmol 1985Neuroophthalmol 1985555ndash6

3) Friedman DI Friedman DI Diagnostic criteria for idiopathic intracranial hypertension Neurology 2002591492ndash5Neurology 2002591492ndash5

Awake patientAwake patientSymptoms amp signs of high ICPSymptoms amp signs of high ICPElevated ICP lateral decubitus position (gt20 cm HElevated ICP lateral decubitus position (gt20 cm H22O)O)Normal CSF compositionNormal CSF compositionNormal routine neuroimagingNormal routine neuroimaging

17Bakhsh A23-05-01

MR images from the case of a 9-year-old male patient with IIH without papilledema

Hiroko Suzuki et al AJNR Am J Neuroradiol 200122196-199

copy2001 by American Society of Neuroradiology18Bakhsh A23-05-01

With treatment there is gradual improvement but notWith treatment there is gradual improvement but notnecessarily recoverynecessarily recoveryMany patients have persistent papilledemaMany patients have persistent papilledemaHigh ICP on lumbar punctureHigh ICP on lumbar punctureResidual visual field deficitsResidual visual field deficits

57 patients were followed for 5 to 41 years 57 patients were followed for 5 to 41 years 24 developed blindness 24 developed blindness

Corbett JJ Corbett JJ Visual loss in pseudotumor cerebri Follow-up of 57 patients from

five to 41 years five to 41 years and a profile of 14 patients with permanent severe visual loss

Arch Neurol Arch Neurol 1982 39461

19Bakhsh A23-05-01

40 recurrence rate over period of 62 years40 recurrence rate over period of 62 years

20 patients were followed up for over 10 years20 patients were followed up for over 10 years

3 patients had recurrence about 12ndash78 months3 patients had recurrence about 12ndash78 months

6 patients experienced delayed worsening6 patients experienced delayed worsening

about 28ndash135 months after an initial stable courseabout 28ndash135 months after an initial stable course

23-05-01 Bakhsh A 20

No evidence based guidelinesNo evidence based guidelines

Alleviation of headacheAlleviation of headache

Preservation of visionPreservation of vision

Early referral to ophthalmologistEarly referral to ophthalmologist

21Bakhsh A23-05-01

23-05-01 Bakhsh A 22

Patients continue to have headaches Patients continue to have headaches despite improvement in papilledema and despite improvement in papilledema and visual functionvisual function

Analgesic overuse or rebound headaches Analgesic overuse or rebound headaches may be common in patients may be common in patients

23Bakhsh A23-05-01

A low-sodium weight reduction program alleviate symptoms but A low-sodium weight reduction program alleviate symptoms but not in all patients not in all patients

Visual fields amp papilledema improve more quickly in weight loss Visual fields amp papilledema improve more quickly in weight loss groupgroup

Weight loss takes some time to achieve other treatments Weight loss takes some time to achieve other treatments are required at the same time are required at the same time

Kupersmith MJ Kupersmith MJ Effects of weight loss on the course of idiopathic intracranial hypertension in women Neurology 1998Neurology 1998 501094

Johnson LNJohnson LN The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri) Ophthalmology 1998Ophthalmology 1998 1052313

24Bakhsh A23-05-01

First line treatment First line treatment 1- 4 g day 1- 4 g day Effective in 47 to 67 Effective in 47 to 67 MethazolamideMethazolamide( carbonic anhydrase Inhibitors) can ( carbonic anhydrase Inhibitors) can

be used in acetazolamide intolerant patients be used in acetazolamide intolerant patients Diamox sequels Diamox sequels sustained release formulationsustained release formulationexpensive expensive Sulfa allergy is relative contraindication Sulfa allergy is relative contraindication

25Bakhsh A

Anorexia Anorexia Metallic taste Metallic taste Kidney stonesKidney stones Metabolic acidosis Metabolic acidosis Nausea amp vomiting Nausea amp vomiting Electrolytes change Electrolytes change Digital amp oral paresthesias Digital amp oral paresthesias

26Bakhsh A23-05-01

Treatment options are limitedTreatment options are limited Caloric restriction Caloric restriction amp amp diureticsdiuretics are are

contraindicatedcontraindicated Acetazolamide is a contraindication in first Acetazolamide is a contraindication in first

20 weeks20 weeks TeratogenicTeratogenic effects have been reported effects have been reported

with high doses in animals and a single with high doses in animals and a single case of acase of a teratoma teratoma was seen in humans was seen in humans

27Bakhsh A23-05-01

Pregnant patientsPregnant patientsOnly Only diagnostic diagnostic not not therapeutictherapeutic CSF reforms within 6 hours CSF reforms within 6 hours Uncomfortable amp painful Uncomfortable amp painful Technically difficult in obeseTechnically difficult in obeseComplications Complications Low pressure headaches (30)Low pressure headaches (30)Bakhsh A Role of conventional lumbar myelography in the managementof sciatica An experience from Pakistan Asian J Neurosurg 2012Jan7(1)25-8

28Bakhsh A23-05-01

Commonly used in the past Commonly used in the past Long-term side effects weight gainLong-term side effects weight gainWithdrawal causes rebound intracranialWithdrawal causes rebound intracranialhypertensionhypertensionSteroids are not routinely recommendedSteroids are not routinely recommendedShort course Short course of intravenous corticosteroidsof intravenous corticosteroidsin conjunction with acetazolamide severein conjunction with acetazolamide severeacute visual lossacute visual lossLiu GT Liu GT High-dose methylprednisolone andHigh-dose methylprednisolone andacetazolamide for visual loss in pseudotumor cerebriacetazolamide for visual loss in pseudotumor cerebriAm J Ophthalmol 1994Am J Ophthalmol 1994 11888 11888

29Bakhsh A23-05-01

Deteriorating vision is a universally Deteriorating vision is a universally

accepted indicationaccepted indication

IntractableIntractable headache unresponsive to headache unresponsive to medicationmedication

30Bakhsh A23-05-01

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

23-05-01 Bakhsh A 16

1)Dandy WE 1)Dandy WE Intracranial pressure without brain tumormdashdiagnosis and treatment Ann Surg 1937106492ndash513Ann Surg 1937106492ndash513

2) Smith JLSmith JL Whence pseudotumor cerebri J Clin J Clin Neuroophthalmol 1985Neuroophthalmol 1985555ndash6

3) Friedman DI Friedman DI Diagnostic criteria for idiopathic intracranial hypertension Neurology 2002591492ndash5Neurology 2002591492ndash5

Awake patientAwake patientSymptoms amp signs of high ICPSymptoms amp signs of high ICPElevated ICP lateral decubitus position (gt20 cm HElevated ICP lateral decubitus position (gt20 cm H22O)O)Normal CSF compositionNormal CSF compositionNormal routine neuroimagingNormal routine neuroimaging

17Bakhsh A23-05-01

MR images from the case of a 9-year-old male patient with IIH without papilledema

Hiroko Suzuki et al AJNR Am J Neuroradiol 200122196-199

copy2001 by American Society of Neuroradiology18Bakhsh A23-05-01

With treatment there is gradual improvement but notWith treatment there is gradual improvement but notnecessarily recoverynecessarily recoveryMany patients have persistent papilledemaMany patients have persistent papilledemaHigh ICP on lumbar punctureHigh ICP on lumbar punctureResidual visual field deficitsResidual visual field deficits

57 patients were followed for 5 to 41 years 57 patients were followed for 5 to 41 years 24 developed blindness 24 developed blindness

Corbett JJ Corbett JJ Visual loss in pseudotumor cerebri Follow-up of 57 patients from

five to 41 years five to 41 years and a profile of 14 patients with permanent severe visual loss

Arch Neurol Arch Neurol 1982 39461

19Bakhsh A23-05-01

40 recurrence rate over period of 62 years40 recurrence rate over period of 62 years

20 patients were followed up for over 10 years20 patients were followed up for over 10 years

3 patients had recurrence about 12ndash78 months3 patients had recurrence about 12ndash78 months

6 patients experienced delayed worsening6 patients experienced delayed worsening

about 28ndash135 months after an initial stable courseabout 28ndash135 months after an initial stable course

23-05-01 Bakhsh A 20

No evidence based guidelinesNo evidence based guidelines

Alleviation of headacheAlleviation of headache

Preservation of visionPreservation of vision

Early referral to ophthalmologistEarly referral to ophthalmologist

21Bakhsh A23-05-01

23-05-01 Bakhsh A 22

Patients continue to have headaches Patients continue to have headaches despite improvement in papilledema and despite improvement in papilledema and visual functionvisual function

Analgesic overuse or rebound headaches Analgesic overuse or rebound headaches may be common in patients may be common in patients

23Bakhsh A23-05-01

A low-sodium weight reduction program alleviate symptoms but A low-sodium weight reduction program alleviate symptoms but not in all patients not in all patients

Visual fields amp papilledema improve more quickly in weight loss Visual fields amp papilledema improve more quickly in weight loss groupgroup

Weight loss takes some time to achieve other treatments Weight loss takes some time to achieve other treatments are required at the same time are required at the same time

Kupersmith MJ Kupersmith MJ Effects of weight loss on the course of idiopathic intracranial hypertension in women Neurology 1998Neurology 1998 501094

Johnson LNJohnson LN The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri) Ophthalmology 1998Ophthalmology 1998 1052313

24Bakhsh A23-05-01

First line treatment First line treatment 1- 4 g day 1- 4 g day Effective in 47 to 67 Effective in 47 to 67 MethazolamideMethazolamide( carbonic anhydrase Inhibitors) can ( carbonic anhydrase Inhibitors) can

be used in acetazolamide intolerant patients be used in acetazolamide intolerant patients Diamox sequels Diamox sequels sustained release formulationsustained release formulationexpensive expensive Sulfa allergy is relative contraindication Sulfa allergy is relative contraindication

25Bakhsh A

Anorexia Anorexia Metallic taste Metallic taste Kidney stonesKidney stones Metabolic acidosis Metabolic acidosis Nausea amp vomiting Nausea amp vomiting Electrolytes change Electrolytes change Digital amp oral paresthesias Digital amp oral paresthesias

26Bakhsh A23-05-01

Treatment options are limitedTreatment options are limited Caloric restriction Caloric restriction amp amp diureticsdiuretics are are

contraindicatedcontraindicated Acetazolamide is a contraindication in first Acetazolamide is a contraindication in first

20 weeks20 weeks TeratogenicTeratogenic effects have been reported effects have been reported

with high doses in animals and a single with high doses in animals and a single case of acase of a teratoma teratoma was seen in humans was seen in humans

27Bakhsh A23-05-01

Pregnant patientsPregnant patientsOnly Only diagnostic diagnostic not not therapeutictherapeutic CSF reforms within 6 hours CSF reforms within 6 hours Uncomfortable amp painful Uncomfortable amp painful Technically difficult in obeseTechnically difficult in obeseComplications Complications Low pressure headaches (30)Low pressure headaches (30)Bakhsh A Role of conventional lumbar myelography in the managementof sciatica An experience from Pakistan Asian J Neurosurg 2012Jan7(1)25-8

28Bakhsh A23-05-01

Commonly used in the past Commonly used in the past Long-term side effects weight gainLong-term side effects weight gainWithdrawal causes rebound intracranialWithdrawal causes rebound intracranialhypertensionhypertensionSteroids are not routinely recommendedSteroids are not routinely recommendedShort course Short course of intravenous corticosteroidsof intravenous corticosteroidsin conjunction with acetazolamide severein conjunction with acetazolamide severeacute visual lossacute visual lossLiu GT Liu GT High-dose methylprednisolone andHigh-dose methylprednisolone andacetazolamide for visual loss in pseudotumor cerebriacetazolamide for visual loss in pseudotumor cerebriAm J Ophthalmol 1994Am J Ophthalmol 1994 11888 11888

29Bakhsh A23-05-01

Deteriorating vision is a universally Deteriorating vision is a universally

accepted indicationaccepted indication

IntractableIntractable headache unresponsive to headache unresponsive to medicationmedication

30Bakhsh A23-05-01

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

1)Dandy WE 1)Dandy WE Intracranial pressure without brain tumormdashdiagnosis and treatment Ann Surg 1937106492ndash513Ann Surg 1937106492ndash513

2) Smith JLSmith JL Whence pseudotumor cerebri J Clin J Clin Neuroophthalmol 1985Neuroophthalmol 1985555ndash6

3) Friedman DI Friedman DI Diagnostic criteria for idiopathic intracranial hypertension Neurology 2002591492ndash5Neurology 2002591492ndash5

Awake patientAwake patientSymptoms amp signs of high ICPSymptoms amp signs of high ICPElevated ICP lateral decubitus position (gt20 cm HElevated ICP lateral decubitus position (gt20 cm H22O)O)Normal CSF compositionNormal CSF compositionNormal routine neuroimagingNormal routine neuroimaging

17Bakhsh A23-05-01

MR images from the case of a 9-year-old male patient with IIH without papilledema

Hiroko Suzuki et al AJNR Am J Neuroradiol 200122196-199

copy2001 by American Society of Neuroradiology18Bakhsh A23-05-01

With treatment there is gradual improvement but notWith treatment there is gradual improvement but notnecessarily recoverynecessarily recoveryMany patients have persistent papilledemaMany patients have persistent papilledemaHigh ICP on lumbar punctureHigh ICP on lumbar punctureResidual visual field deficitsResidual visual field deficits

57 patients were followed for 5 to 41 years 57 patients were followed for 5 to 41 years 24 developed blindness 24 developed blindness

Corbett JJ Corbett JJ Visual loss in pseudotumor cerebri Follow-up of 57 patients from

five to 41 years five to 41 years and a profile of 14 patients with permanent severe visual loss

Arch Neurol Arch Neurol 1982 39461

19Bakhsh A23-05-01

40 recurrence rate over period of 62 years40 recurrence rate over period of 62 years

20 patients were followed up for over 10 years20 patients were followed up for over 10 years

3 patients had recurrence about 12ndash78 months3 patients had recurrence about 12ndash78 months

6 patients experienced delayed worsening6 patients experienced delayed worsening

about 28ndash135 months after an initial stable courseabout 28ndash135 months after an initial stable course

23-05-01 Bakhsh A 20

No evidence based guidelinesNo evidence based guidelines

Alleviation of headacheAlleviation of headache

Preservation of visionPreservation of vision

Early referral to ophthalmologistEarly referral to ophthalmologist

21Bakhsh A23-05-01

23-05-01 Bakhsh A 22

Patients continue to have headaches Patients continue to have headaches despite improvement in papilledema and despite improvement in papilledema and visual functionvisual function

Analgesic overuse or rebound headaches Analgesic overuse or rebound headaches may be common in patients may be common in patients

23Bakhsh A23-05-01

A low-sodium weight reduction program alleviate symptoms but A low-sodium weight reduction program alleviate symptoms but not in all patients not in all patients

Visual fields amp papilledema improve more quickly in weight loss Visual fields amp papilledema improve more quickly in weight loss groupgroup

Weight loss takes some time to achieve other treatments Weight loss takes some time to achieve other treatments are required at the same time are required at the same time

Kupersmith MJ Kupersmith MJ Effects of weight loss on the course of idiopathic intracranial hypertension in women Neurology 1998Neurology 1998 501094

Johnson LNJohnson LN The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri) Ophthalmology 1998Ophthalmology 1998 1052313

24Bakhsh A23-05-01

First line treatment First line treatment 1- 4 g day 1- 4 g day Effective in 47 to 67 Effective in 47 to 67 MethazolamideMethazolamide( carbonic anhydrase Inhibitors) can ( carbonic anhydrase Inhibitors) can

be used in acetazolamide intolerant patients be used in acetazolamide intolerant patients Diamox sequels Diamox sequels sustained release formulationsustained release formulationexpensive expensive Sulfa allergy is relative contraindication Sulfa allergy is relative contraindication

25Bakhsh A

Anorexia Anorexia Metallic taste Metallic taste Kidney stonesKidney stones Metabolic acidosis Metabolic acidosis Nausea amp vomiting Nausea amp vomiting Electrolytes change Electrolytes change Digital amp oral paresthesias Digital amp oral paresthesias

26Bakhsh A23-05-01

Treatment options are limitedTreatment options are limited Caloric restriction Caloric restriction amp amp diureticsdiuretics are are

contraindicatedcontraindicated Acetazolamide is a contraindication in first Acetazolamide is a contraindication in first

20 weeks20 weeks TeratogenicTeratogenic effects have been reported effects have been reported

with high doses in animals and a single with high doses in animals and a single case of acase of a teratoma teratoma was seen in humans was seen in humans

27Bakhsh A23-05-01

Pregnant patientsPregnant patientsOnly Only diagnostic diagnostic not not therapeutictherapeutic CSF reforms within 6 hours CSF reforms within 6 hours Uncomfortable amp painful Uncomfortable amp painful Technically difficult in obeseTechnically difficult in obeseComplications Complications Low pressure headaches (30)Low pressure headaches (30)Bakhsh A Role of conventional lumbar myelography in the managementof sciatica An experience from Pakistan Asian J Neurosurg 2012Jan7(1)25-8

28Bakhsh A23-05-01

Commonly used in the past Commonly used in the past Long-term side effects weight gainLong-term side effects weight gainWithdrawal causes rebound intracranialWithdrawal causes rebound intracranialhypertensionhypertensionSteroids are not routinely recommendedSteroids are not routinely recommendedShort course Short course of intravenous corticosteroidsof intravenous corticosteroidsin conjunction with acetazolamide severein conjunction with acetazolamide severeacute visual lossacute visual lossLiu GT Liu GT High-dose methylprednisolone andHigh-dose methylprednisolone andacetazolamide for visual loss in pseudotumor cerebriacetazolamide for visual loss in pseudotumor cerebriAm J Ophthalmol 1994Am J Ophthalmol 1994 11888 11888

29Bakhsh A23-05-01

Deteriorating vision is a universally Deteriorating vision is a universally

accepted indicationaccepted indication

IntractableIntractable headache unresponsive to headache unresponsive to medicationmedication

30Bakhsh A23-05-01

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

MR images from the case of a 9-year-old male patient with IIH without papilledema

Hiroko Suzuki et al AJNR Am J Neuroradiol 200122196-199

copy2001 by American Society of Neuroradiology18Bakhsh A23-05-01

With treatment there is gradual improvement but notWith treatment there is gradual improvement but notnecessarily recoverynecessarily recoveryMany patients have persistent papilledemaMany patients have persistent papilledemaHigh ICP on lumbar punctureHigh ICP on lumbar punctureResidual visual field deficitsResidual visual field deficits

57 patients were followed for 5 to 41 years 57 patients were followed for 5 to 41 years 24 developed blindness 24 developed blindness

Corbett JJ Corbett JJ Visual loss in pseudotumor cerebri Follow-up of 57 patients from

five to 41 years five to 41 years and a profile of 14 patients with permanent severe visual loss

Arch Neurol Arch Neurol 1982 39461

19Bakhsh A23-05-01

40 recurrence rate over period of 62 years40 recurrence rate over period of 62 years

20 patients were followed up for over 10 years20 patients were followed up for over 10 years

3 patients had recurrence about 12ndash78 months3 patients had recurrence about 12ndash78 months

6 patients experienced delayed worsening6 patients experienced delayed worsening

about 28ndash135 months after an initial stable courseabout 28ndash135 months after an initial stable course

23-05-01 Bakhsh A 20

No evidence based guidelinesNo evidence based guidelines

Alleviation of headacheAlleviation of headache

Preservation of visionPreservation of vision

Early referral to ophthalmologistEarly referral to ophthalmologist

21Bakhsh A23-05-01

23-05-01 Bakhsh A 22

Patients continue to have headaches Patients continue to have headaches despite improvement in papilledema and despite improvement in papilledema and visual functionvisual function

Analgesic overuse or rebound headaches Analgesic overuse or rebound headaches may be common in patients may be common in patients

23Bakhsh A23-05-01

A low-sodium weight reduction program alleviate symptoms but A low-sodium weight reduction program alleviate symptoms but not in all patients not in all patients

Visual fields amp papilledema improve more quickly in weight loss Visual fields amp papilledema improve more quickly in weight loss groupgroup

Weight loss takes some time to achieve other treatments Weight loss takes some time to achieve other treatments are required at the same time are required at the same time

Kupersmith MJ Kupersmith MJ Effects of weight loss on the course of idiopathic intracranial hypertension in women Neurology 1998Neurology 1998 501094

Johnson LNJohnson LN The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri) Ophthalmology 1998Ophthalmology 1998 1052313

24Bakhsh A23-05-01

First line treatment First line treatment 1- 4 g day 1- 4 g day Effective in 47 to 67 Effective in 47 to 67 MethazolamideMethazolamide( carbonic anhydrase Inhibitors) can ( carbonic anhydrase Inhibitors) can

be used in acetazolamide intolerant patients be used in acetazolamide intolerant patients Diamox sequels Diamox sequels sustained release formulationsustained release formulationexpensive expensive Sulfa allergy is relative contraindication Sulfa allergy is relative contraindication

25Bakhsh A

Anorexia Anorexia Metallic taste Metallic taste Kidney stonesKidney stones Metabolic acidosis Metabolic acidosis Nausea amp vomiting Nausea amp vomiting Electrolytes change Electrolytes change Digital amp oral paresthesias Digital amp oral paresthesias

26Bakhsh A23-05-01

Treatment options are limitedTreatment options are limited Caloric restriction Caloric restriction amp amp diureticsdiuretics are are

contraindicatedcontraindicated Acetazolamide is a contraindication in first Acetazolamide is a contraindication in first

20 weeks20 weeks TeratogenicTeratogenic effects have been reported effects have been reported

with high doses in animals and a single with high doses in animals and a single case of acase of a teratoma teratoma was seen in humans was seen in humans

27Bakhsh A23-05-01

Pregnant patientsPregnant patientsOnly Only diagnostic diagnostic not not therapeutictherapeutic CSF reforms within 6 hours CSF reforms within 6 hours Uncomfortable amp painful Uncomfortable amp painful Technically difficult in obeseTechnically difficult in obeseComplications Complications Low pressure headaches (30)Low pressure headaches (30)Bakhsh A Role of conventional lumbar myelography in the managementof sciatica An experience from Pakistan Asian J Neurosurg 2012Jan7(1)25-8

28Bakhsh A23-05-01

Commonly used in the past Commonly used in the past Long-term side effects weight gainLong-term side effects weight gainWithdrawal causes rebound intracranialWithdrawal causes rebound intracranialhypertensionhypertensionSteroids are not routinely recommendedSteroids are not routinely recommendedShort course Short course of intravenous corticosteroidsof intravenous corticosteroidsin conjunction with acetazolamide severein conjunction with acetazolamide severeacute visual lossacute visual lossLiu GT Liu GT High-dose methylprednisolone andHigh-dose methylprednisolone andacetazolamide for visual loss in pseudotumor cerebriacetazolamide for visual loss in pseudotumor cerebriAm J Ophthalmol 1994Am J Ophthalmol 1994 11888 11888

29Bakhsh A23-05-01

Deteriorating vision is a universally Deteriorating vision is a universally

accepted indicationaccepted indication

IntractableIntractable headache unresponsive to headache unresponsive to medicationmedication

30Bakhsh A23-05-01

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

With treatment there is gradual improvement but notWith treatment there is gradual improvement but notnecessarily recoverynecessarily recoveryMany patients have persistent papilledemaMany patients have persistent papilledemaHigh ICP on lumbar punctureHigh ICP on lumbar punctureResidual visual field deficitsResidual visual field deficits

57 patients were followed for 5 to 41 years 57 patients were followed for 5 to 41 years 24 developed blindness 24 developed blindness

Corbett JJ Corbett JJ Visual loss in pseudotumor cerebri Follow-up of 57 patients from

five to 41 years five to 41 years and a profile of 14 patients with permanent severe visual loss

Arch Neurol Arch Neurol 1982 39461

19Bakhsh A23-05-01

40 recurrence rate over period of 62 years40 recurrence rate over period of 62 years

20 patients were followed up for over 10 years20 patients were followed up for over 10 years

3 patients had recurrence about 12ndash78 months3 patients had recurrence about 12ndash78 months

6 patients experienced delayed worsening6 patients experienced delayed worsening

about 28ndash135 months after an initial stable courseabout 28ndash135 months after an initial stable course

23-05-01 Bakhsh A 20

No evidence based guidelinesNo evidence based guidelines

Alleviation of headacheAlleviation of headache

Preservation of visionPreservation of vision

Early referral to ophthalmologistEarly referral to ophthalmologist

21Bakhsh A23-05-01

23-05-01 Bakhsh A 22

Patients continue to have headaches Patients continue to have headaches despite improvement in papilledema and despite improvement in papilledema and visual functionvisual function

Analgesic overuse or rebound headaches Analgesic overuse or rebound headaches may be common in patients may be common in patients

23Bakhsh A23-05-01

A low-sodium weight reduction program alleviate symptoms but A low-sodium weight reduction program alleviate symptoms but not in all patients not in all patients

Visual fields amp papilledema improve more quickly in weight loss Visual fields amp papilledema improve more quickly in weight loss groupgroup

Weight loss takes some time to achieve other treatments Weight loss takes some time to achieve other treatments are required at the same time are required at the same time

Kupersmith MJ Kupersmith MJ Effects of weight loss on the course of idiopathic intracranial hypertension in women Neurology 1998Neurology 1998 501094

Johnson LNJohnson LN The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri) Ophthalmology 1998Ophthalmology 1998 1052313

24Bakhsh A23-05-01

First line treatment First line treatment 1- 4 g day 1- 4 g day Effective in 47 to 67 Effective in 47 to 67 MethazolamideMethazolamide( carbonic anhydrase Inhibitors) can ( carbonic anhydrase Inhibitors) can

be used in acetazolamide intolerant patients be used in acetazolamide intolerant patients Diamox sequels Diamox sequels sustained release formulationsustained release formulationexpensive expensive Sulfa allergy is relative contraindication Sulfa allergy is relative contraindication

25Bakhsh A

Anorexia Anorexia Metallic taste Metallic taste Kidney stonesKidney stones Metabolic acidosis Metabolic acidosis Nausea amp vomiting Nausea amp vomiting Electrolytes change Electrolytes change Digital amp oral paresthesias Digital amp oral paresthesias

26Bakhsh A23-05-01

Treatment options are limitedTreatment options are limited Caloric restriction Caloric restriction amp amp diureticsdiuretics are are

contraindicatedcontraindicated Acetazolamide is a contraindication in first Acetazolamide is a contraindication in first

20 weeks20 weeks TeratogenicTeratogenic effects have been reported effects have been reported

with high doses in animals and a single with high doses in animals and a single case of acase of a teratoma teratoma was seen in humans was seen in humans

27Bakhsh A23-05-01

Pregnant patientsPregnant patientsOnly Only diagnostic diagnostic not not therapeutictherapeutic CSF reforms within 6 hours CSF reforms within 6 hours Uncomfortable amp painful Uncomfortable amp painful Technically difficult in obeseTechnically difficult in obeseComplications Complications Low pressure headaches (30)Low pressure headaches (30)Bakhsh A Role of conventional lumbar myelography in the managementof sciatica An experience from Pakistan Asian J Neurosurg 2012Jan7(1)25-8

28Bakhsh A23-05-01

Commonly used in the past Commonly used in the past Long-term side effects weight gainLong-term side effects weight gainWithdrawal causes rebound intracranialWithdrawal causes rebound intracranialhypertensionhypertensionSteroids are not routinely recommendedSteroids are not routinely recommendedShort course Short course of intravenous corticosteroidsof intravenous corticosteroidsin conjunction with acetazolamide severein conjunction with acetazolamide severeacute visual lossacute visual lossLiu GT Liu GT High-dose methylprednisolone andHigh-dose methylprednisolone andacetazolamide for visual loss in pseudotumor cerebriacetazolamide for visual loss in pseudotumor cerebriAm J Ophthalmol 1994Am J Ophthalmol 1994 11888 11888

29Bakhsh A23-05-01

Deteriorating vision is a universally Deteriorating vision is a universally

accepted indicationaccepted indication

IntractableIntractable headache unresponsive to headache unresponsive to medicationmedication

30Bakhsh A23-05-01

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

40 recurrence rate over period of 62 years40 recurrence rate over period of 62 years

20 patients were followed up for over 10 years20 patients were followed up for over 10 years

3 patients had recurrence about 12ndash78 months3 patients had recurrence about 12ndash78 months

6 patients experienced delayed worsening6 patients experienced delayed worsening

about 28ndash135 months after an initial stable courseabout 28ndash135 months after an initial stable course

23-05-01 Bakhsh A 20

No evidence based guidelinesNo evidence based guidelines

Alleviation of headacheAlleviation of headache

Preservation of visionPreservation of vision

Early referral to ophthalmologistEarly referral to ophthalmologist

21Bakhsh A23-05-01

23-05-01 Bakhsh A 22

Patients continue to have headaches Patients continue to have headaches despite improvement in papilledema and despite improvement in papilledema and visual functionvisual function

Analgesic overuse or rebound headaches Analgesic overuse or rebound headaches may be common in patients may be common in patients

23Bakhsh A23-05-01

A low-sodium weight reduction program alleviate symptoms but A low-sodium weight reduction program alleviate symptoms but not in all patients not in all patients

Visual fields amp papilledema improve more quickly in weight loss Visual fields amp papilledema improve more quickly in weight loss groupgroup

Weight loss takes some time to achieve other treatments Weight loss takes some time to achieve other treatments are required at the same time are required at the same time

Kupersmith MJ Kupersmith MJ Effects of weight loss on the course of idiopathic intracranial hypertension in women Neurology 1998Neurology 1998 501094

Johnson LNJohnson LN The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri) Ophthalmology 1998Ophthalmology 1998 1052313

24Bakhsh A23-05-01

First line treatment First line treatment 1- 4 g day 1- 4 g day Effective in 47 to 67 Effective in 47 to 67 MethazolamideMethazolamide( carbonic anhydrase Inhibitors) can ( carbonic anhydrase Inhibitors) can

be used in acetazolamide intolerant patients be used in acetazolamide intolerant patients Diamox sequels Diamox sequels sustained release formulationsustained release formulationexpensive expensive Sulfa allergy is relative contraindication Sulfa allergy is relative contraindication

25Bakhsh A

Anorexia Anorexia Metallic taste Metallic taste Kidney stonesKidney stones Metabolic acidosis Metabolic acidosis Nausea amp vomiting Nausea amp vomiting Electrolytes change Electrolytes change Digital amp oral paresthesias Digital amp oral paresthesias

26Bakhsh A23-05-01

Treatment options are limitedTreatment options are limited Caloric restriction Caloric restriction amp amp diureticsdiuretics are are

contraindicatedcontraindicated Acetazolamide is a contraindication in first Acetazolamide is a contraindication in first

20 weeks20 weeks TeratogenicTeratogenic effects have been reported effects have been reported

with high doses in animals and a single with high doses in animals and a single case of acase of a teratoma teratoma was seen in humans was seen in humans

27Bakhsh A23-05-01

Pregnant patientsPregnant patientsOnly Only diagnostic diagnostic not not therapeutictherapeutic CSF reforms within 6 hours CSF reforms within 6 hours Uncomfortable amp painful Uncomfortable amp painful Technically difficult in obeseTechnically difficult in obeseComplications Complications Low pressure headaches (30)Low pressure headaches (30)Bakhsh A Role of conventional lumbar myelography in the managementof sciatica An experience from Pakistan Asian J Neurosurg 2012Jan7(1)25-8

28Bakhsh A23-05-01

Commonly used in the past Commonly used in the past Long-term side effects weight gainLong-term side effects weight gainWithdrawal causes rebound intracranialWithdrawal causes rebound intracranialhypertensionhypertensionSteroids are not routinely recommendedSteroids are not routinely recommendedShort course Short course of intravenous corticosteroidsof intravenous corticosteroidsin conjunction with acetazolamide severein conjunction with acetazolamide severeacute visual lossacute visual lossLiu GT Liu GT High-dose methylprednisolone andHigh-dose methylprednisolone andacetazolamide for visual loss in pseudotumor cerebriacetazolamide for visual loss in pseudotumor cerebriAm J Ophthalmol 1994Am J Ophthalmol 1994 11888 11888

29Bakhsh A23-05-01

Deteriorating vision is a universally Deteriorating vision is a universally

accepted indicationaccepted indication

IntractableIntractable headache unresponsive to headache unresponsive to medicationmedication

30Bakhsh A23-05-01

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

No evidence based guidelinesNo evidence based guidelines

Alleviation of headacheAlleviation of headache

Preservation of visionPreservation of vision

Early referral to ophthalmologistEarly referral to ophthalmologist

21Bakhsh A23-05-01

23-05-01 Bakhsh A 22

Patients continue to have headaches Patients continue to have headaches despite improvement in papilledema and despite improvement in papilledema and visual functionvisual function

Analgesic overuse or rebound headaches Analgesic overuse or rebound headaches may be common in patients may be common in patients

23Bakhsh A23-05-01

A low-sodium weight reduction program alleviate symptoms but A low-sodium weight reduction program alleviate symptoms but not in all patients not in all patients

Visual fields amp papilledema improve more quickly in weight loss Visual fields amp papilledema improve more quickly in weight loss groupgroup

Weight loss takes some time to achieve other treatments Weight loss takes some time to achieve other treatments are required at the same time are required at the same time

Kupersmith MJ Kupersmith MJ Effects of weight loss on the course of idiopathic intracranial hypertension in women Neurology 1998Neurology 1998 501094

Johnson LNJohnson LN The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri) Ophthalmology 1998Ophthalmology 1998 1052313

24Bakhsh A23-05-01

First line treatment First line treatment 1- 4 g day 1- 4 g day Effective in 47 to 67 Effective in 47 to 67 MethazolamideMethazolamide( carbonic anhydrase Inhibitors) can ( carbonic anhydrase Inhibitors) can

be used in acetazolamide intolerant patients be used in acetazolamide intolerant patients Diamox sequels Diamox sequels sustained release formulationsustained release formulationexpensive expensive Sulfa allergy is relative contraindication Sulfa allergy is relative contraindication

25Bakhsh A

Anorexia Anorexia Metallic taste Metallic taste Kidney stonesKidney stones Metabolic acidosis Metabolic acidosis Nausea amp vomiting Nausea amp vomiting Electrolytes change Electrolytes change Digital amp oral paresthesias Digital amp oral paresthesias

26Bakhsh A23-05-01

Treatment options are limitedTreatment options are limited Caloric restriction Caloric restriction amp amp diureticsdiuretics are are

contraindicatedcontraindicated Acetazolamide is a contraindication in first Acetazolamide is a contraindication in first

20 weeks20 weeks TeratogenicTeratogenic effects have been reported effects have been reported

with high doses in animals and a single with high doses in animals and a single case of acase of a teratoma teratoma was seen in humans was seen in humans

27Bakhsh A23-05-01

Pregnant patientsPregnant patientsOnly Only diagnostic diagnostic not not therapeutictherapeutic CSF reforms within 6 hours CSF reforms within 6 hours Uncomfortable amp painful Uncomfortable amp painful Technically difficult in obeseTechnically difficult in obeseComplications Complications Low pressure headaches (30)Low pressure headaches (30)Bakhsh A Role of conventional lumbar myelography in the managementof sciatica An experience from Pakistan Asian J Neurosurg 2012Jan7(1)25-8

28Bakhsh A23-05-01

Commonly used in the past Commonly used in the past Long-term side effects weight gainLong-term side effects weight gainWithdrawal causes rebound intracranialWithdrawal causes rebound intracranialhypertensionhypertensionSteroids are not routinely recommendedSteroids are not routinely recommendedShort course Short course of intravenous corticosteroidsof intravenous corticosteroidsin conjunction with acetazolamide severein conjunction with acetazolamide severeacute visual lossacute visual lossLiu GT Liu GT High-dose methylprednisolone andHigh-dose methylprednisolone andacetazolamide for visual loss in pseudotumor cerebriacetazolamide for visual loss in pseudotumor cerebriAm J Ophthalmol 1994Am J Ophthalmol 1994 11888 11888

29Bakhsh A23-05-01

Deteriorating vision is a universally Deteriorating vision is a universally

accepted indicationaccepted indication

IntractableIntractable headache unresponsive to headache unresponsive to medicationmedication

30Bakhsh A23-05-01

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

23-05-01 Bakhsh A 22

Patients continue to have headaches Patients continue to have headaches despite improvement in papilledema and despite improvement in papilledema and visual functionvisual function

Analgesic overuse or rebound headaches Analgesic overuse or rebound headaches may be common in patients may be common in patients

23Bakhsh A23-05-01

A low-sodium weight reduction program alleviate symptoms but A low-sodium weight reduction program alleviate symptoms but not in all patients not in all patients

Visual fields amp papilledema improve more quickly in weight loss Visual fields amp papilledema improve more quickly in weight loss groupgroup

Weight loss takes some time to achieve other treatments Weight loss takes some time to achieve other treatments are required at the same time are required at the same time

Kupersmith MJ Kupersmith MJ Effects of weight loss on the course of idiopathic intracranial hypertension in women Neurology 1998Neurology 1998 501094

Johnson LNJohnson LN The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri) Ophthalmology 1998Ophthalmology 1998 1052313

24Bakhsh A23-05-01

First line treatment First line treatment 1- 4 g day 1- 4 g day Effective in 47 to 67 Effective in 47 to 67 MethazolamideMethazolamide( carbonic anhydrase Inhibitors) can ( carbonic anhydrase Inhibitors) can

be used in acetazolamide intolerant patients be used in acetazolamide intolerant patients Diamox sequels Diamox sequels sustained release formulationsustained release formulationexpensive expensive Sulfa allergy is relative contraindication Sulfa allergy is relative contraindication

25Bakhsh A

Anorexia Anorexia Metallic taste Metallic taste Kidney stonesKidney stones Metabolic acidosis Metabolic acidosis Nausea amp vomiting Nausea amp vomiting Electrolytes change Electrolytes change Digital amp oral paresthesias Digital amp oral paresthesias

26Bakhsh A23-05-01

Treatment options are limitedTreatment options are limited Caloric restriction Caloric restriction amp amp diureticsdiuretics are are

contraindicatedcontraindicated Acetazolamide is a contraindication in first Acetazolamide is a contraindication in first

20 weeks20 weeks TeratogenicTeratogenic effects have been reported effects have been reported

with high doses in animals and a single with high doses in animals and a single case of acase of a teratoma teratoma was seen in humans was seen in humans

27Bakhsh A23-05-01

Pregnant patientsPregnant patientsOnly Only diagnostic diagnostic not not therapeutictherapeutic CSF reforms within 6 hours CSF reforms within 6 hours Uncomfortable amp painful Uncomfortable amp painful Technically difficult in obeseTechnically difficult in obeseComplications Complications Low pressure headaches (30)Low pressure headaches (30)Bakhsh A Role of conventional lumbar myelography in the managementof sciatica An experience from Pakistan Asian J Neurosurg 2012Jan7(1)25-8

28Bakhsh A23-05-01

Commonly used in the past Commonly used in the past Long-term side effects weight gainLong-term side effects weight gainWithdrawal causes rebound intracranialWithdrawal causes rebound intracranialhypertensionhypertensionSteroids are not routinely recommendedSteroids are not routinely recommendedShort course Short course of intravenous corticosteroidsof intravenous corticosteroidsin conjunction with acetazolamide severein conjunction with acetazolamide severeacute visual lossacute visual lossLiu GT Liu GT High-dose methylprednisolone andHigh-dose methylprednisolone andacetazolamide for visual loss in pseudotumor cerebriacetazolamide for visual loss in pseudotumor cerebriAm J Ophthalmol 1994Am J Ophthalmol 1994 11888 11888

29Bakhsh A23-05-01

Deteriorating vision is a universally Deteriorating vision is a universally

accepted indicationaccepted indication

IntractableIntractable headache unresponsive to headache unresponsive to medicationmedication

30Bakhsh A23-05-01

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Patients continue to have headaches Patients continue to have headaches despite improvement in papilledema and despite improvement in papilledema and visual functionvisual function

Analgesic overuse or rebound headaches Analgesic overuse or rebound headaches may be common in patients may be common in patients

23Bakhsh A23-05-01

A low-sodium weight reduction program alleviate symptoms but A low-sodium weight reduction program alleviate symptoms but not in all patients not in all patients

Visual fields amp papilledema improve more quickly in weight loss Visual fields amp papilledema improve more quickly in weight loss groupgroup

Weight loss takes some time to achieve other treatments Weight loss takes some time to achieve other treatments are required at the same time are required at the same time

Kupersmith MJ Kupersmith MJ Effects of weight loss on the course of idiopathic intracranial hypertension in women Neurology 1998Neurology 1998 501094

Johnson LNJohnson LN The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri) Ophthalmology 1998Ophthalmology 1998 1052313

24Bakhsh A23-05-01

First line treatment First line treatment 1- 4 g day 1- 4 g day Effective in 47 to 67 Effective in 47 to 67 MethazolamideMethazolamide( carbonic anhydrase Inhibitors) can ( carbonic anhydrase Inhibitors) can

be used in acetazolamide intolerant patients be used in acetazolamide intolerant patients Diamox sequels Diamox sequels sustained release formulationsustained release formulationexpensive expensive Sulfa allergy is relative contraindication Sulfa allergy is relative contraindication

25Bakhsh A

Anorexia Anorexia Metallic taste Metallic taste Kidney stonesKidney stones Metabolic acidosis Metabolic acidosis Nausea amp vomiting Nausea amp vomiting Electrolytes change Electrolytes change Digital amp oral paresthesias Digital amp oral paresthesias

26Bakhsh A23-05-01

Treatment options are limitedTreatment options are limited Caloric restriction Caloric restriction amp amp diureticsdiuretics are are

contraindicatedcontraindicated Acetazolamide is a contraindication in first Acetazolamide is a contraindication in first

20 weeks20 weeks TeratogenicTeratogenic effects have been reported effects have been reported

with high doses in animals and a single with high doses in animals and a single case of acase of a teratoma teratoma was seen in humans was seen in humans

27Bakhsh A23-05-01

Pregnant patientsPregnant patientsOnly Only diagnostic diagnostic not not therapeutictherapeutic CSF reforms within 6 hours CSF reforms within 6 hours Uncomfortable amp painful Uncomfortable amp painful Technically difficult in obeseTechnically difficult in obeseComplications Complications Low pressure headaches (30)Low pressure headaches (30)Bakhsh A Role of conventional lumbar myelography in the managementof sciatica An experience from Pakistan Asian J Neurosurg 2012Jan7(1)25-8

28Bakhsh A23-05-01

Commonly used in the past Commonly used in the past Long-term side effects weight gainLong-term side effects weight gainWithdrawal causes rebound intracranialWithdrawal causes rebound intracranialhypertensionhypertensionSteroids are not routinely recommendedSteroids are not routinely recommendedShort course Short course of intravenous corticosteroidsof intravenous corticosteroidsin conjunction with acetazolamide severein conjunction with acetazolamide severeacute visual lossacute visual lossLiu GT Liu GT High-dose methylprednisolone andHigh-dose methylprednisolone andacetazolamide for visual loss in pseudotumor cerebriacetazolamide for visual loss in pseudotumor cerebriAm J Ophthalmol 1994Am J Ophthalmol 1994 11888 11888

29Bakhsh A23-05-01

Deteriorating vision is a universally Deteriorating vision is a universally

accepted indicationaccepted indication

IntractableIntractable headache unresponsive to headache unresponsive to medicationmedication

30Bakhsh A23-05-01

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

A low-sodium weight reduction program alleviate symptoms but A low-sodium weight reduction program alleviate symptoms but not in all patients not in all patients

Visual fields amp papilledema improve more quickly in weight loss Visual fields amp papilledema improve more quickly in weight loss groupgroup

Weight loss takes some time to achieve other treatments Weight loss takes some time to achieve other treatments are required at the same time are required at the same time

Kupersmith MJ Kupersmith MJ Effects of weight loss on the course of idiopathic intracranial hypertension in women Neurology 1998Neurology 1998 501094

Johnson LNJohnson LN The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri) Ophthalmology 1998Ophthalmology 1998 1052313

24Bakhsh A23-05-01

First line treatment First line treatment 1- 4 g day 1- 4 g day Effective in 47 to 67 Effective in 47 to 67 MethazolamideMethazolamide( carbonic anhydrase Inhibitors) can ( carbonic anhydrase Inhibitors) can

be used in acetazolamide intolerant patients be used in acetazolamide intolerant patients Diamox sequels Diamox sequels sustained release formulationsustained release formulationexpensive expensive Sulfa allergy is relative contraindication Sulfa allergy is relative contraindication

25Bakhsh A

Anorexia Anorexia Metallic taste Metallic taste Kidney stonesKidney stones Metabolic acidosis Metabolic acidosis Nausea amp vomiting Nausea amp vomiting Electrolytes change Electrolytes change Digital amp oral paresthesias Digital amp oral paresthesias

26Bakhsh A23-05-01

Treatment options are limitedTreatment options are limited Caloric restriction Caloric restriction amp amp diureticsdiuretics are are

contraindicatedcontraindicated Acetazolamide is a contraindication in first Acetazolamide is a contraindication in first

20 weeks20 weeks TeratogenicTeratogenic effects have been reported effects have been reported

with high doses in animals and a single with high doses in animals and a single case of acase of a teratoma teratoma was seen in humans was seen in humans

27Bakhsh A23-05-01

Pregnant patientsPregnant patientsOnly Only diagnostic diagnostic not not therapeutictherapeutic CSF reforms within 6 hours CSF reforms within 6 hours Uncomfortable amp painful Uncomfortable amp painful Technically difficult in obeseTechnically difficult in obeseComplications Complications Low pressure headaches (30)Low pressure headaches (30)Bakhsh A Role of conventional lumbar myelography in the managementof sciatica An experience from Pakistan Asian J Neurosurg 2012Jan7(1)25-8

28Bakhsh A23-05-01

Commonly used in the past Commonly used in the past Long-term side effects weight gainLong-term side effects weight gainWithdrawal causes rebound intracranialWithdrawal causes rebound intracranialhypertensionhypertensionSteroids are not routinely recommendedSteroids are not routinely recommendedShort course Short course of intravenous corticosteroidsof intravenous corticosteroidsin conjunction with acetazolamide severein conjunction with acetazolamide severeacute visual lossacute visual lossLiu GT Liu GT High-dose methylprednisolone andHigh-dose methylprednisolone andacetazolamide for visual loss in pseudotumor cerebriacetazolamide for visual loss in pseudotumor cerebriAm J Ophthalmol 1994Am J Ophthalmol 1994 11888 11888

29Bakhsh A23-05-01

Deteriorating vision is a universally Deteriorating vision is a universally

accepted indicationaccepted indication

IntractableIntractable headache unresponsive to headache unresponsive to medicationmedication

30Bakhsh A23-05-01

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

First line treatment First line treatment 1- 4 g day 1- 4 g day Effective in 47 to 67 Effective in 47 to 67 MethazolamideMethazolamide( carbonic anhydrase Inhibitors) can ( carbonic anhydrase Inhibitors) can

be used in acetazolamide intolerant patients be used in acetazolamide intolerant patients Diamox sequels Diamox sequels sustained release formulationsustained release formulationexpensive expensive Sulfa allergy is relative contraindication Sulfa allergy is relative contraindication

25Bakhsh A

Anorexia Anorexia Metallic taste Metallic taste Kidney stonesKidney stones Metabolic acidosis Metabolic acidosis Nausea amp vomiting Nausea amp vomiting Electrolytes change Electrolytes change Digital amp oral paresthesias Digital amp oral paresthesias

26Bakhsh A23-05-01

Treatment options are limitedTreatment options are limited Caloric restriction Caloric restriction amp amp diureticsdiuretics are are

contraindicatedcontraindicated Acetazolamide is a contraindication in first Acetazolamide is a contraindication in first

20 weeks20 weeks TeratogenicTeratogenic effects have been reported effects have been reported

with high doses in animals and a single with high doses in animals and a single case of acase of a teratoma teratoma was seen in humans was seen in humans

27Bakhsh A23-05-01

Pregnant patientsPregnant patientsOnly Only diagnostic diagnostic not not therapeutictherapeutic CSF reforms within 6 hours CSF reforms within 6 hours Uncomfortable amp painful Uncomfortable amp painful Technically difficult in obeseTechnically difficult in obeseComplications Complications Low pressure headaches (30)Low pressure headaches (30)Bakhsh A Role of conventional lumbar myelography in the managementof sciatica An experience from Pakistan Asian J Neurosurg 2012Jan7(1)25-8

28Bakhsh A23-05-01

Commonly used in the past Commonly used in the past Long-term side effects weight gainLong-term side effects weight gainWithdrawal causes rebound intracranialWithdrawal causes rebound intracranialhypertensionhypertensionSteroids are not routinely recommendedSteroids are not routinely recommendedShort course Short course of intravenous corticosteroidsof intravenous corticosteroidsin conjunction with acetazolamide severein conjunction with acetazolamide severeacute visual lossacute visual lossLiu GT Liu GT High-dose methylprednisolone andHigh-dose methylprednisolone andacetazolamide for visual loss in pseudotumor cerebriacetazolamide for visual loss in pseudotumor cerebriAm J Ophthalmol 1994Am J Ophthalmol 1994 11888 11888

29Bakhsh A23-05-01

Deteriorating vision is a universally Deteriorating vision is a universally

accepted indicationaccepted indication

IntractableIntractable headache unresponsive to headache unresponsive to medicationmedication

30Bakhsh A23-05-01

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Anorexia Anorexia Metallic taste Metallic taste Kidney stonesKidney stones Metabolic acidosis Metabolic acidosis Nausea amp vomiting Nausea amp vomiting Electrolytes change Electrolytes change Digital amp oral paresthesias Digital amp oral paresthesias

26Bakhsh A23-05-01

Treatment options are limitedTreatment options are limited Caloric restriction Caloric restriction amp amp diureticsdiuretics are are

contraindicatedcontraindicated Acetazolamide is a contraindication in first Acetazolamide is a contraindication in first

20 weeks20 weeks TeratogenicTeratogenic effects have been reported effects have been reported

with high doses in animals and a single with high doses in animals and a single case of acase of a teratoma teratoma was seen in humans was seen in humans

27Bakhsh A23-05-01

Pregnant patientsPregnant patientsOnly Only diagnostic diagnostic not not therapeutictherapeutic CSF reforms within 6 hours CSF reforms within 6 hours Uncomfortable amp painful Uncomfortable amp painful Technically difficult in obeseTechnically difficult in obeseComplications Complications Low pressure headaches (30)Low pressure headaches (30)Bakhsh A Role of conventional lumbar myelography in the managementof sciatica An experience from Pakistan Asian J Neurosurg 2012Jan7(1)25-8

28Bakhsh A23-05-01

Commonly used in the past Commonly used in the past Long-term side effects weight gainLong-term side effects weight gainWithdrawal causes rebound intracranialWithdrawal causes rebound intracranialhypertensionhypertensionSteroids are not routinely recommendedSteroids are not routinely recommendedShort course Short course of intravenous corticosteroidsof intravenous corticosteroidsin conjunction with acetazolamide severein conjunction with acetazolamide severeacute visual lossacute visual lossLiu GT Liu GT High-dose methylprednisolone andHigh-dose methylprednisolone andacetazolamide for visual loss in pseudotumor cerebriacetazolamide for visual loss in pseudotumor cerebriAm J Ophthalmol 1994Am J Ophthalmol 1994 11888 11888

29Bakhsh A23-05-01

Deteriorating vision is a universally Deteriorating vision is a universally

accepted indicationaccepted indication

IntractableIntractable headache unresponsive to headache unresponsive to medicationmedication

30Bakhsh A23-05-01

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Treatment options are limitedTreatment options are limited Caloric restriction Caloric restriction amp amp diureticsdiuretics are are

contraindicatedcontraindicated Acetazolamide is a contraindication in first Acetazolamide is a contraindication in first

20 weeks20 weeks TeratogenicTeratogenic effects have been reported effects have been reported

with high doses in animals and a single with high doses in animals and a single case of acase of a teratoma teratoma was seen in humans was seen in humans

27Bakhsh A23-05-01

Pregnant patientsPregnant patientsOnly Only diagnostic diagnostic not not therapeutictherapeutic CSF reforms within 6 hours CSF reforms within 6 hours Uncomfortable amp painful Uncomfortable amp painful Technically difficult in obeseTechnically difficult in obeseComplications Complications Low pressure headaches (30)Low pressure headaches (30)Bakhsh A Role of conventional lumbar myelography in the managementof sciatica An experience from Pakistan Asian J Neurosurg 2012Jan7(1)25-8

28Bakhsh A23-05-01

Commonly used in the past Commonly used in the past Long-term side effects weight gainLong-term side effects weight gainWithdrawal causes rebound intracranialWithdrawal causes rebound intracranialhypertensionhypertensionSteroids are not routinely recommendedSteroids are not routinely recommendedShort course Short course of intravenous corticosteroidsof intravenous corticosteroidsin conjunction with acetazolamide severein conjunction with acetazolamide severeacute visual lossacute visual lossLiu GT Liu GT High-dose methylprednisolone andHigh-dose methylprednisolone andacetazolamide for visual loss in pseudotumor cerebriacetazolamide for visual loss in pseudotumor cerebriAm J Ophthalmol 1994Am J Ophthalmol 1994 11888 11888

29Bakhsh A23-05-01

Deteriorating vision is a universally Deteriorating vision is a universally

accepted indicationaccepted indication

IntractableIntractable headache unresponsive to headache unresponsive to medicationmedication

30Bakhsh A23-05-01

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Pregnant patientsPregnant patientsOnly Only diagnostic diagnostic not not therapeutictherapeutic CSF reforms within 6 hours CSF reforms within 6 hours Uncomfortable amp painful Uncomfortable amp painful Technically difficult in obeseTechnically difficult in obeseComplications Complications Low pressure headaches (30)Low pressure headaches (30)Bakhsh A Role of conventional lumbar myelography in the managementof sciatica An experience from Pakistan Asian J Neurosurg 2012Jan7(1)25-8

28Bakhsh A23-05-01

Commonly used in the past Commonly used in the past Long-term side effects weight gainLong-term side effects weight gainWithdrawal causes rebound intracranialWithdrawal causes rebound intracranialhypertensionhypertensionSteroids are not routinely recommendedSteroids are not routinely recommendedShort course Short course of intravenous corticosteroidsof intravenous corticosteroidsin conjunction with acetazolamide severein conjunction with acetazolamide severeacute visual lossacute visual lossLiu GT Liu GT High-dose methylprednisolone andHigh-dose methylprednisolone andacetazolamide for visual loss in pseudotumor cerebriacetazolamide for visual loss in pseudotumor cerebriAm J Ophthalmol 1994Am J Ophthalmol 1994 11888 11888

29Bakhsh A23-05-01

Deteriorating vision is a universally Deteriorating vision is a universally

accepted indicationaccepted indication

IntractableIntractable headache unresponsive to headache unresponsive to medicationmedication

30Bakhsh A23-05-01

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Commonly used in the past Commonly used in the past Long-term side effects weight gainLong-term side effects weight gainWithdrawal causes rebound intracranialWithdrawal causes rebound intracranialhypertensionhypertensionSteroids are not routinely recommendedSteroids are not routinely recommendedShort course Short course of intravenous corticosteroidsof intravenous corticosteroidsin conjunction with acetazolamide severein conjunction with acetazolamide severeacute visual lossacute visual lossLiu GT Liu GT High-dose methylprednisolone andHigh-dose methylprednisolone andacetazolamide for visual loss in pseudotumor cerebriacetazolamide for visual loss in pseudotumor cerebriAm J Ophthalmol 1994Am J Ophthalmol 1994 11888 11888

29Bakhsh A23-05-01

Deteriorating vision is a universally Deteriorating vision is a universally

accepted indicationaccepted indication

IntractableIntractable headache unresponsive to headache unresponsive to medicationmedication

30Bakhsh A23-05-01

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Deteriorating vision is a universally Deteriorating vision is a universally

accepted indicationaccepted indication

IntractableIntractable headache unresponsive to headache unresponsive to medicationmedication

30Bakhsh A23-05-01

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

bull Ventriculoperitoneal shuntVentriculoperitoneal shuntbull Lumboperitoneal shunt Lumboperitoneal shunt bull Repeated lumbar puncturesRepeated lumbar puncturesbull Bariatric surgeryBariatric surgerybull Optic nerve sheath fenestrationOptic nerve sheath fenestrationbull Dural venous sinus stentingDural venous sinus stenting

23-05-01 Bakhsh A 31

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

HeadacheHeadache relief occurs in all patients relief occurs in all patients 50 having recurrent severe headaches50 having recurrent severe headacheswithin 3 years of surgery despite a workingwithin 3 years of surgery despite a workingshunt shunt 95 to 100 achieve remission of 95 to 100 achieve remission of visualvisualProblemsProblems

Vision continued to worsen in 32 Vision continued to worsen in 32

32Bakhsh A23-05-01

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Provide long-term relief in majority of Provide long-term relief in majority of patientspatients

Endoscopic operative techniques have Endoscopic operative techniques have improved our ability to place catheters improved our ability to place catheters

Shunt revision 40 to 60 Shunt revision 40 to 60

McGirt M Frameless stereotactic ventriculoperitoneal shunting for pseudotumor cerebri an outcomes comparison versus lumboperitoneal shunting Neurosurgery 2004 55458-9

33Bakhsh A23-05-01

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Shunt failure 86 Shunt failure 86 Shunt revisions 38 Shunt revisions 38 Low pressureLow pressureheadachesheadaches

Burgett RA Lumboperitoneal shunting for pseudotumor cerebri Neurology 1997 49734-9

23-05-01 Bakhsh A 34

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at oneinstitution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewedBased on their 30-year experience authors found thatBased on their 30-year experience authors found thatCSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acutetreatment providing long-term relief in the majority oftreatment providing long-term relief in the majority ofpatientspatientsThe use of ventricular shunts was associated with aThe use of ventricular shunts was associated with alower risk of shunt obstruction amp revision than the uselower risk of shunt obstruction amp revision than the useof of LP shuntsLP shunts

McGirt MJMcGirt MJ Cerebrospinal fluid shunt placement for pseudotumor cerebri-Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache predictors of treatment response associated intractable headache predictors of treatment response and an analysis of long-term outcomesand an analysis of long-term outcomes J Neurosurg J Neurosurg 2004 101(4)627-32 2004 101(4)627-32

23-05-01 Bakhsh A 35

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Remission of symptoms 92 Remission of symptoms 92 Papilledema resolves 97 Papilledema resolves 97 Effects start after 1 to 3 years after surgery Effects start after 1 to 3 years after surgery With mean weight loss of 45 to 58 kg With mean weight loss of 45 to 58 kg 12 studies class IV have been published 12 studies class IV have been published

with 66 patients with 66 patients

Jared Fridley Jared Fridley Bariatric surgery for the treatment of Bariatric surgery for the treatment of idiopathic intracranial hypertension J idiopathic intracranial hypertension J Neurosurg Neurosurg 2010 2010

36Bakhsh A23-05-01

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

37Bakhsh A23-05-01

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

OOptic ptic NNerve erve SSheath heath FFenestration enestration Preservation of vision is primary goalPreservation of vision is primary goal

It does not reduce ICPIt does not reduce ICP

Patients with bilateral papilledema needPatients with bilateral papilledema needbilateral bilateral OONNSSFF

Shunting may still be required Shunting may still be required Alsuhaibani AH et el Alsuhaibani AH et el Effect of optic nerve sheath fenestration on Effect of optic nerve sheath fenestration on

papilledema of thepapilledema of theoperated and the contralateral nonoperated eyes in idiopathic intracranial operated and the contralateral nonoperated eyes in idiopathic intracranial

hypertensionhypertensionOphthalmology 2011Ophthalmology 2011 118412ndash414 118412ndash414

38Bakhsh A23-05-01

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Diplopia Diplopia Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve orblood supply) in 29 to 35 blood supply) in 29 to 35 Pupillary dysfunction 11 Pupillary dysfunction 11 Transient Vision loss 11 Transient Vision loss 11 Permanent in 15 to 26Permanent in 15 to 26Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF

39Bakhsh A23-05-01

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Many patients havetransverse sinus narrowing

atDistal transverse sinusDistal transverse sinus

Transversesigmoid sinusTransversesigmoid sinusJunctionJunction

Unilaterally Unilaterally

OrOr

BilaterallyBilaterally

23-05-01 Bakhsh A 40

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Cerebral venography and manometry in 99 patients with idiopathic intracranial hypertension consistently showed

venous hypertension venous hypertension in superior sagittal sinus amp superior sagittal sinus amp proximal transverse sinusesproximal transverse sinuses significant drop in venous pressure at the level of lateral third significant drop in venous pressure at the level of lateral third

of transverse sinus of transverse sinus The abnormality clearlyclearly demonstrated by manometry was not well

shown on the venous phase of cerebral angiography The appearance of the transverse sinus on venography varied from

smooth tapered narrowing to discrete intraluminal filling defects

King JOKing JO11Cerebral venography and manometry in idiopathic Cerebral venography and manometry in idiopathic intracranial hypertensionintracranial hypertension Neurology Neurology 1995 1995 45(12)2224-845(12)2224-8

23-05-01 Bakhsh A 41

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

May 1 2023 Bakhsh ABakhsh A 42

Farb have identified venous sinus stenosis in Farb have identified venous sinus stenosis in gtgt9090 of patients with PTC of patients with PTC

6868 in the control asymptomatic group in the control asymptomatic group

In another recent study In another recent study 9090 of 51 PTC of 51 PTC patients had bilateral transverse sinus patients had bilateral transverse sinus stenosis on MR venography with stenosis on MR venography with ATECO MRV ATECO MRV techniquetechnique

Farb RI Farb RI Idiopathic intracranial hypertension the prevalence Idiopathic intracranial hypertension the prevalenceand morphology of sinovenous stenosis and morphology of sinovenous stenosis Neurology 2003Neurology 2003601418ndash1424601418ndash1424

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

May 1 2023 Bakhsh A 43

The conventional MR venography suffers from The conventional MR venography suffers from artifacts in the region of the distal transverse sinus artifacts in the region of the distal transverse sinus This is why venous stenosis in PTC has been missed This is why venous stenosis in PTC has been missed in the past in the past

Higgins et al Higgins et al reanalyzedreanalyzed the MRVs of 20 PTC patients the MRVs of 20 PTC patients that were initially interpreted as that were initially interpreted as normal normal

Bilateral lateral Bilateral lateral sinus flow gaps sinus flow gaps were identified in were identified in 13 of 20 patients with PTC13 of 20 patients with PTC

None of 40 controls None of 40 controls

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

23-05-01 Bakhsh A 46

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

May 1 2023 Bakhsh A 47

In venous sinuses In venous sinuses increaseincrease in in numbernumber andand sizesize with advancing age and can with advancing age and can obstruct transverse sinusesobstruct transverse sinuses

Cause focal intra-luminal filling defects in Cause focal intra-luminal filling defects in 24 of CT and 13 of contrast enhanced 24 of CT and 13 of contrast enhanced MR studies in normal populationsMR studies in normal populations

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging studyA Sagittal reconstruction image obtained from 3D contrast-

enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect c

Luxia Liang et al AJNR Am J Neuroradiol 2002231739-1746

copy2002 by American Society of Neuroradiology

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

20 transverse sinuses were explored (in a pilot study 20 transverse sinuses were explored (in a pilot study of 10 human cadavers) in order to determine the of 10 human cadavers) in order to determine the anatomical basis of this stenosisanatomical basis of this stenosis

The presence of septa of varying sizes was The presence of septa of varying sizes was observed observed

We conclude might be one of the aetiological factors We conclude might be one of the aetiological factors involved in idiopathic intracranial hypertensioninvolved in idiopathic intracranial hypertension

Subramaniam RM Transverse sinus septum a new aetiology of idiopathic intracranial hypertension Australas Radiol 2004 Jun48(2)114-6

23-05-01 Bakhsh A 49

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

A total of A total of 102 cadavers 102 cadavers amp amp living patients living patients were used were used 53 of the subjects had structures in their53 of the subjects had structures in theirtransverse sinuses that could be potential venoustransverse sinuses that could be potential venousfilling defectsfilling defects

The septa were found to be more dominant inThe septa were found to be more dominant incentral (30) and lateral (22) thirds of central (30) and lateral (22) thirds of right transverse sinusesright transverse sinuses

30 of the subjects presented with arachnoid30 of the subjects presented with arachnoidgranulations in the right transverse sinusgranulations in the right transverse sinus

Strydom MA et el Strydom MA et el The anatomical basis of venographic filling The anatomical basis of venographic filling defects of the transverse sinus defects of the transverse sinus Clin Anat 2010Clin Anat 201023(2)153-923(2)153-9

50Bakhsh A23-05-01

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

23-05-01 Bakhsh A 51

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

23-05-01 Bakhsh A 52

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

23-05-01 Bakhsh A 53

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Mechanism by which transverse sinus stenosis leads to increase intracranial pressure

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

May 1 2023 Bakhsh ABakhsh A 55

Transverse sinus stenosis may occur as a secondary phenomenon in response to elevated ICP

Resolved stenosis with CSF drainage reversal of the venous sinus stenoses either by means of lumbar puncture or by CSF shunting

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension

Valeacuterie Biousse et al J Neurol Neurosurg Psychiatry 201283488-494

copy2012 by BMJ Publishing Group Ltd

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

May 1 2023 Bakhsh A 57

The first stent placement in the transverse The first stent placement in the transverse sinus for the treatment of IIH was sinus for the treatment of IIH was attempted in 2002 by Higgins in an obese attempted in 2002 by Higgins in an obese woman with bilateral stenosis of the sinuses woman with bilateral stenosis of the sinuses and intracranial hypertension refractory to and intracranial hypertension refractory to any form of treatmentany form of treatment

Higgins JN Higgins JN Idiopathic intracranial hypertension12 cases treated byIdiopathic intracranial hypertension12 cases treated byvenous sinus stenting venous sinus stenting J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003

741662-741662-

050123 Bakhsh A 57

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

May 1 2023 Bakhsh A 58

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

May 1 2023 Bakhsh A 59

Outcomes in 207 patients Outcomes in 207 patients 2 Months to 136 Months 2 Months to 136 Months 81 headaches 81 headaches 87 papilledema87 papilledema 95 pulsatile tinnitus95 pulsatile tinnitus Follow up periodsFollow up periods

Albuquerque FC et alAlbuquerque FC et al Intracranial venous sinus stenting Intracranial venous sinus stenting for benign intracranial hypertension clinical indications for benign intracranial hypertension clinical indications technique and preliminary results technique and preliminary results World Neurosurg World Neurosurg 2011 2011 75648ndash65275648ndash652

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

May 1 2023 Bakhsh A 60

Stent migrationStent migration

Sinus perforationSinus perforation In-stent thrombosisIn-stent thrombosis Subdural hemorrhageSubdural hemorrhage Intracranial hemorrhageIntracranial hemorrhage

Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent

Puffer RC Puffer RC Venous sinus stenting for idiopathicVenous sinus stenting for idiopathicintracranial hypertension a review of the literatureintracranial hypertension a review of the literature JJNeurointerv Surg 2013Neurointerv Surg 2013 5483 5483

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

May 1 2023 Bakhsh ABakhsh A 61

Stent patency may be evaluated by CT Stent patency may be evaluated by CT venographyvenography

Six-month period of anticoagulation is Six-month period of anticoagulation is required post stentingrequired post stenting

Be Be alert to the recurrence of PTC symptoms alert to the recurrence of PTC symptoms

Require re-stentingRequire re-stenting

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

May 1 2023 Bakhsh A 62

Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444million year in U S Amillion year in U S A

A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSFshunting procedures shunting procedures versus versus venous sinus stentingvenous sinus stenting

There was no cost difference for the initial procedureThere was no cost difference for the initial procedurefor both shunts and stentsfor both shunts and stents

The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related toshunt infections made the shunting procedureshunt infections made the shunting procedureapproximately approximately five times more costly overall five times more costly overall

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

May 1 2023 Bakhsh A 63

The Idiopathic IntracranialHypertension Treatment Trial

A multicenter double-blind placebo-controlled clinical trial is currently enrolling patients in the US (httpwwwnordicclinicaltrialscom)

This trial compares the efficacy of acetazolamide and placebo in the treatment of IIH patients with moderate visual

field defects All patients are also treated with a low-sodium diet and

participate in a standardized weight loss program This trial will clarify the efficacy of acetazolamide efficacy of acetazolamide and weight weight

loss loss in IIH Additional outcomes measured yearly up to 4 years Wall et al The Idiopathic Intracranial Hypertension Wall et al The Idiopathic Intracranial Hypertension

Treatment Trial JAMA Neurology 2014 Vol 71 No 6Treatment Trial JAMA Neurology 2014 Vol 71 No 6

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

The importance of venous sinus disease in the etiology of The importance of venous sinus disease in the etiology of idiopathic intracranial hypertension is probably idiopathic intracranial hypertension is probably underestimated underestimated

Patients in whom a venous sinus stenosis is Patients in whom a venous sinus stenosis is demonstrated by a noninvasive radiologic workup demonstrated by a noninvasive radiologic workup should be evaluated with direct retrograde cerebral should be evaluated with direct retrograde cerebral venography amp manometryvenography amp manometry

In patients with a In patients with a lesion of the venous sinuses lesion of the venous sinuses who who experienced experienced medical treatment failuremedical treatment failure endovascular endovascular stent placement seems to be an stent placement seems to be an interesting interesting alternative alternative to to classic surgical approachesclassic surgical approaches

Donnet ADonnet A Endovascular treatment of idiopathic Endovascular treatment of idiopathic intracranial hypertension clinical and radiologic outcome intracranial hypertension clinical and radiologic outcome of 10 consecutive patientsof 10 consecutive patients Neurology 2008 70641 Neurology 2008 70641

23-05-01 Bakhsh A 64

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65

23-05-01 65

• Slide 1
• Pseudotumor cerebri
• Slide 3
• Slide 4
• Slide 5
• Slide 6
• Epidemiology
• Middle East
• History amp Nomenclature
• Slide 10
• Slide 11
• Slide 12
• Slide 13
• Slide 14
• Slide 15
• Slide 16
• Modified Dandy criteria by Smith in 1985
• Slide 18
• Slide 19
• Slide 20
• Slide 22
• Slide 24
• Slide 25
• Slide 27
• Slide 28
• Slide 29
• Slide 30
• Slide 31
• Slide 32
• Slide 33
• Slide 34
• Slide 35
• Slide 36
• Slide 37
• Optic Nerve Sheath Fenestration
• Slide 39
• Slide 40
• Slide 41
• Slide 42
• Slide 43
• Slide 44
• Slide 45
• Arachnoid granulations
• Slide 47
• Slide 48
• Slide 49
• Slide 50
• Slide 51
• Slide 52
• Slide 53
• Slide 54
• Slide 55
• Slide 56
• Slide 57
• Slide 58
• Slide 59
• Slide 60
• Slide 61
• Slide 62
• The Idiopathic Intracranial Hypertension Treatment Trial
• Slide 64
• Slide 65