csf leaks steven wright, m.d. matthew ryan, m.d. january 5, 2004

CSF LeaksCSF Leaks

Steven Wright, M.D.Steven Wright, M.D.

Matthew Ryan, M.D.Matthew Ryan, M.D.

January 5, 2004January 5, 2004

CSF LeaksCSF Leaks

Abnormal communication between the Abnormal communication between the subarachnoid space and the subarachnoid space and the tympanomastoid space or nasal cavity.tympanomastoid space or nasal cavity.

Presenting symptoms:Presenting symptoms: Middle ear effusion, hearing lossMiddle ear effusion, hearing loss Unilateral rhinorrheaUnilateral rhinorrhea

Risk of meningitis is highRisk of meningitis is high 2-88%2-88%

CSF RhinorrheaCSF Rhinorrhea

Diverse etiologyDiverse etiologyIdiopathicIdiopathicTrauma-SurgicalTrauma-Surgical

<1%<1%Trauma-NonsurgicalTrauma-Nonsurgical

3% of all closed head injuries3% of all closed head injuries30% of skull base fractures30% of skull base fracturesFrontal>Ethmoids>SphenoidsFrontal>Ethmoids>Sphenoids

InflammatoryInflammatoryCongenitalCongenitalNeoplasmNeoplasm

Testing of Nasal SecretionsTesting of Nasal Secretions

Beta-2-transferrin is highly sensitive and Beta-2-transferrin is highly sensitive and specificspecific 1/501/50thth of a drop of a drop

Electronic nose has shown early successElectronic nose has shown early success

ImagingImaging

High resolution CTHigh resolution CT CT CisternographyCT Cisternography MRIMRI

Heavily weighted T2Heavily weighted T2 Slow flow MRISlow flow MRI MRI cisternographyMRI cisternography

Radionuclide cisternographyRadionuclide cisternography Intrathecal flourescinIntrathecal flourescin

ImagingImaging

HRCTHRCT Volume averagingVolume averaging Congenital Congenital

dehiscences of dehiscences of Spenoid/cribiform Spenoid/cribiform niche.niche.

ImagingImaging

CT cisternographyCT cisternography

Currently the optimal Currently the optimal imaging modality (85% imaging modality (85% sensitive)sensitive)

Intrathecal Intrathecal administration of administration of iodine, prone 6hrsiodine, prone 6hrs

0% for inactive leaks0% for inactive leaks Substantial radiation Substantial radiation

exposureexposure ?neurotoxic potential?neurotoxic potential

ImagingImaging

MRI cisternographyMRI cisternography heavily weighted T2heavily weighted T2

Intrathecal gadoliniumIntrathecal gadolinium

ImagingImaging

Slow flow MRISlow flow MRI Diffusion weighted Diffusion weighted

MRIMRI Fluid motion down to Fluid motion down to

0.5mm/sec0.5mm/sec Ex. MRA/MRVEx. MRA/MRV

ImagingImaging

Radioisotope cisternographyRadioisotope cisternography Intrathecal administration of technitium 99mIntrathecal administration of technitium 99m Less spatial resolution and specificityLess spatial resolution and specificity Largely abandoned due to false positive and Largely abandoned due to false positive and

false negative resultsfalse negative results

Intrathecal FlourescinIntrathecal Flourescin

0.1ml of 10% 0.1ml of 10% flourescin solution flourescin solution mixed in 10cc of CSFmixed in 10cc of CSF

Blue light may Blue light may enhance the enhance the flourescinflourescin

Complications are lowComplications are low

Treatment of CSF RhinorrheaTreatment of CSF Rhinorrhea

Conservative measuresConservative measures Bed rest/Elev HOB>30Bed rest/Elev HOB>30 Stool softenersStool softeners No sneezing/coughingNo sneezing/coughing +/- lumbar drains+/- lumbar drains

Early failuresEarly failures Assoc with hydrocephalusAssoc with hydrocephalus Recurrent or persistent leaksRecurrent or persistent leaks

Treatment of CSF RhinorrheaTreatment of CSF Rhinorrhea

Prophylactic antibiotics:Prophylactic antibiotics: Two conflicting meta-analysis regarding Two conflicting meta-analysis regarding

basilar skull fractures.basilar skull fractures. Proponents argue less meningitis.Proponents argue less meningitis. Opponents argue organism resistance.Opponents argue organism resistance.

Surgical OptionsSurgical Options

IntracranialIntracranial Direct visualizationDirect visualization Success rates 50-73%Success rates 50-73% Significant morbiditySignificant morbidity

• AnosmiaAnosmia• Cerebral edemaCerebral edema• SeizuresSeizures

Surgical OptionsSurgical Options

Extracranial approachExtracranial approach Improved success rates (80%)Improved success rates (80%) Significant morbiditySignificant morbidity Frontal osteoplastic flap/infratemporal Frontal osteoplastic flap/infratemporal

approachapproach

Endoscopic repairEndoscopic repair

Endoscopic intranasal repairEndoscopic intranasal repair Overall success rates:Overall success rates:

• 90% 1st attempt90% 1st attempt• 52-67% for 252-67% for 2ndnd attempt attempt• Overall 97%Overall 97%

Complications:Complications:• Meningitis (0.3%)Meningitis (0.3%)• Brain abscess (0.9%)Brain abscess (0.9%)• Subdural hematoma (0.3%)Subdural hematoma (0.3%)• Headache (0.3%)Headache (0.3%)

Endoscopic techniquesEndoscopic techniques

Overlay vs UnderlayOverlay vs Underlaytechniquetechnique

Meta-analysis Meta-analysis showed that both showed that both techniques have techniques have similar success ratessimilar success rates

Onlay: adjacent Onlay: adjacent structures at risk, or if structures at risk, or if the underlay is not the underlay is not possiblepossible

Surgical TechniquesSurgical Techniques

Use gelfoam and gelfilm (>90%)Use gelfoam and gelfilm (>90%) Use nasal packing (100%)Use nasal packing (100%) Consider fibrin glue (>50%)Consider fibrin glue (>50%) Consider lumbar drain for Consider lumbar drain for

idiopathathic/posttraumatic assoc with idiopathathic/posttraumatic assoc with increased ICPincreased ICP 3-5 days3-5 days Not requiredNot required

BR, stool softeners, antibioticsBR, stool softeners, antibiotics

CSF OtorrheaCSF Otorrhea

AcquiredAcquired Postoperative (58%)Postoperative (58%) Trauma (32%)Trauma (32%) Nontraumatic (11%)Nontraumatic (11%)

SpontaneousSpontaneous Bony defect theoryBony defect theory Arachnoid granulation theoryArachnoid granulation theory

Temporal bone fracturesTemporal bone fractures

LongitudinalLongitudinal 70%70% Anterior to otic capsuleAnterior to otic capsule 15-20% facial nerve 15-20% facial nerve

involvementinvolvement

Temporal bone fracturesTemporal bone fractures

TransverseTransverse 20%20% High rate of SNHLHigh rate of SNHL 50% facial nerve 50% facial nerve

involvementinvolvement

Temporal bone fracturesTemporal bone fractures

HRCT will demonstrate the fracture line HRCT will demonstrate the fracture line and the likely site of CSF leak.and the likely site of CSF leak.

Beta-2-transferrinBeta-2-transferrin TreatmentTreatment

BedrestBedrest Elev HOBElev HOB Stool softenersStool softeners +/- lumbar drain+/- lumbar drain

Temporal bone fracturesTemporal bone fractures

Brodie and Thompson et al.Brodie and Thompson et al. 820 T-bone fractures/122 CSF leaks820 T-bone fractures/122 CSF leaks Spontaneous resolutionSpontaneous resolution

95/122: within 7 days95/122: within 7 days 21/122: between 7-14 days21/122: between 7-14 days 5/122: Persisted beyond 2 weeks5/122: Persisted beyond 2 weeks

Temporal bone fracturesTemporal bone fractures

MeningitisMeningitis 9/121 (7%) developed meningitis.9/121 (7%) developed meningitis.

A later meta-analysis by the same author A later meta-analysis by the same author did reveal a statistically significant did reveal a statistically significant reduction in the incidence of meningitis reduction in the incidence of meningitis with the use of prophylactic antibiotics.with the use of prophylactic antibiotics.

Pediatric temporal bone fracturesPediatric temporal bone fractures

Much lower incidence (10:1, adult:pedi)Much lower incidence (10:1, adult:pedi) Undeveloped sinuses, skull flexibilityUndeveloped sinuses, skull flexibility

otorrhea>> rhinorrheaotorrhea>> rhinorrhea Prophylactic antibiotics did not influence Prophylactic antibiotics did not influence

the development of meningitis.the development of meningitis.

Spontaneous CSF otorrheaSpontaneous CSF otorrhea

Congenital Defect Theory:Congenital Defect Theory: 1) enlarged petrosal fallopian canal1) enlarged petrosal fallopian canal 2) patent tympanomeningeal (Hyrtl’s) fissure2) patent tympanomeningeal (Hyrtl’s) fissure 3) Comminication of the IAC with the vestibule 3) Comminication of the IAC with the vestibule

(Mondini’s dysplasia)-most common(Mondini’s dysplasia)-most common Childhood presentationChildhood presentation

82% SNHL82% SNHL 93% Meningitis93% Meningitis 83% Mondini Dysplasia83% Mondini Dysplasia

Congenital bony defectCongenital bony defect

Spontaneous CSF otorrheaSpontaneous CSF otorrhea

Arachnoid granulation theoryArachnoid granulation theory Enlargement of arachnoid villi due to Enlargement of arachnoid villi due to

congenital entrapments/pressure variationscongenital entrapments/pressure variations PresentationPresentation

Unilateral serous otitis mediaUnilateral serous otitis media Meningitis (36%)Meningitis (36%) No SNHL or Mondini dysplasiaNo SNHL or Mondini dysplasia Sites are multiple, floor of the middle fossa Sites are multiple, floor of the middle fossa

most commonmost common

Arachnoid GranulationArachnoid Granulation

Spontaneous CSF otorrheaSpontaneous CSF otorrhea

Stone et al.Stone et al. HRCT vs. CT cisternography/radionuclide HRCT vs. CT cisternography/radionuclide

cisternography.cisternography. HRCT showed bony defects in 71%.HRCT showed bony defects in 71%. 100% intraoperative findings correlated with 100% intraoperative findings correlated with

HRCT.HRCT. HRCT significantly identified more patients HRCT significantly identified more patients

with CSF leak than radionuclide with CSF leak than radionuclide cisternography or CT cisternography.cisternography or CT cisternography.

Surgical approachesSurgical approaches

TransmastoidTransmastoid Not ideal for large Not ideal for large

defects (>2cm), defects (>2cm), multiple defects, or multiple defects, or defects that extend defects that extend anteriorlyanteriorly

Middle cranial fossaMiddle cranial fossa Technically Technically

challengingchallenging Best exposureBest exposure

Combined approachCombined approach

Technique of closureTechnique of closure

Muscle, fascia, fat, bone wax, etc..Muscle, fascia, fat, bone wax, etc.. The success rate is significantly higher for The success rate is significantly higher for

those patients who undergo primary those patients who undergo primary closure with a multi-layer technique versus closure with a multi-layer technique versus those patients who only get single-layer those patients who only get single-layer closure.closure.

Refractory cases may require closure of Refractory cases may require closure of the EAC and obliteration.the EAC and obliteration.

ConclusionsConclusions

The clinical presentations of CSF leaks The clinical presentations of CSF leaks may be very subtle.may be very subtle.

The clinician must keep a low threshold for The clinician must keep a low threshold for further testing with Beta-2-Transferrin.further testing with Beta-2-Transferrin.

Imaging studies should be performed to Imaging studies should be performed to anatomically localize the site.anatomically localize the site.

Success rates may be over 90% with Success rates may be over 90% with proper patient and surgical selectionproper patient and surgical selection