Tarlov Cysts and Associated Dural

Ectasia-When to Operate

William C. Welch MD, FAANS,FACS, FICS

Robert L. Bailey MD, Robert Whitmore MD

University of Pennsylvania School of Medicine

Donlin Long, MD, FAANS, FACS

Johns Hopkins Hospital

First of many water/sea quotes

"To be or not to be– that is the question:

Whether 'tis nobler in the mind to suffer

The slings and arrows of outrageous

fortune,

Or to take arms against a sea of troubles

And, by opposing, end them.”

William Shakespeare

Hamlet.

Case

• 59 yo female with 4 year hx of LBP, posterior leg

pain, and pelvic discomfort (“ground glass”)

• Multiple evaluations, multiple physicians

• Had undergone spinal cord untethering surgery

2 years ago

• No specific neurological complaints or deficits,

suggestion of bladder dysfunction

Thoughts?

• Diagnosis?

• Treatment options?

• Outcomes?

• Literature?

Introduction Definition:

• Collections of CSF between the endoneurium and

perineurium of the nerve root sheath near the dorsal root

ganglion in S1-S4 region.

• Microscopic evidence of neural injury

– Prevalence estimated to be 4.6% among general adult

population

• Definition has become liberalized to include all nerve root

associated cysts

Frequency: 1-2% of patients have nerve root

associated cysts on MRI

Historical Classification

• Identified on autopsy specimens in the late 1930‘s by Tarlov

and Rexed

• One subsequent autopsy description (NEJM Clinico-

pathologic Conference, Long)

• 1950‘s Tarlov described 8 patients in whom symptoms were

attributed to the cysts

Classification

• Nabors et al.

– Tarlov or perineural cysts are Type II

• Nerve tissue in the walls of the cyst, unclear if

communicating with perineural arachnoid space

– Type I:

• Extra-dural cysts without neural involvement, enlarge

foramen and scallop vertebral bodies

– Type III:

• Intradural, either congenital or caused by trauma

Comparison to Other Cysts

Potential communication with SA space Communicates freely with SA space

Delayed filling in myelograms Rapid filling in myelograms

Found distal to the junction of posterior

nerve root and dorsal root ganglion in

sacral region

Found proximal to dorsal root ganglion

throughout vertebral column

Walls contain nerve fibers Walls lined by arachnoid mater with no

signs of neural elements

Often multiple, extending around the

circumference of nerve root

No pattern of formation with regards to

multiplicity

Etiology Formation:

• Inflammation within nerve root cysts followed by inoculation

of CSF

• Congenital origin

• Arachnoidal proliferation along exiting sacral nerve root

• Blockage of venous drainage in the perineuria and

epineurium secondary to hemosiderin deposition after trauma

• Collagen vascular disorders

• Marfan‘s syndrome

Symptoms

• Usually asymptomatic, but may be attributable to symptoms in

15-30% of cases

• Tendency to increase in size over time, potentially causing

complications and eroding sacral bone

• 4 Categories of symptomatic patients:

– Group 1: Pain on tail bone that radiates to legs with potential weakness

– Group 2: Pain on bones, legs, groin area, sexual dysfunction and

dysfunctional bladder

– Group 3: Pain that radiates from cyst site across hips to lower abdomen

– Group 4: No pain, only sexual and bladder dysfunction

Symptoms

• Wide spectrum of symptoms including:

– Back pain, perineal pain, sciatica, cauda equina

syndrome, dysuria, urinary incontinence,

coccygodynia, sacral radiculopathy, headaches,

retrograde ejaculation, parathesia, hyperesthesia,

difficulty with ambulation, motor dysfunction in lower

extremities, abdominal pain

Symptoms Etiology of symptoms:

• Direct nerve injury

• Cyst pressure on adjacent nerve roots

• Transmssion of CSF pressure into cyst

• subsequent further compression of nerve root

• nerve root wall tension

• CSF leakage (functional pseudomeningocele)

• Sacral erosion

• Unclear etiological postulates

Symptoms Nerve root symptoms:

• Radiculopathy

• Typically S1 and S2

• May have localized pain at cyst site

• Can be thoracic, potentially cervical

Sacral and Pelvic pain

• Variable pelvic symptoms

• ʺpressure,“ ʺground-glass,“ others

• Bladder/bowel symptoms

• Sacral erosions

CSF hypotensive symptoms

• Postural headache

Physical Examination

General exam:

• Collagen disorder

• Neurofibromatosis

• CRPS

• Radiculopathy

• Hypesthesia

• Decreased reflexes

• Strength testing

• Perineal sensation

• Postural symptoms

Evaluation

• Evaluation

• Is there any other potentially reasonable cause of the patient’s

symptoms

• EMG testing to look for radiculopathy

• Plain films

• CT (may wish to include sacrum)

• Myelogram

• MRI of lumbar and sacral spine

• MRI of pelvis

• Urological studies

• Gynecological studies

Treatment Options

• Do Nothing

– Run Away!

• Expectant therapy (most common)

• PT/OT

• Epidural steroids

• Others

• Invasive Non-Surgical Therapy

• Surgical Options

“I pass with relief from the tossing sea of cause

and theory to the firm ground of result and fact.”

Winston Churchill

Literature

Neurosurgery. 40(4), April 1997, pp 861-865

• 3 patient cases in which lumbar drain was placed and resulted in

alleviation of symptoms

• 2/3 underwent lumboperitoneal shunt with resolution of symptoms for

11 and 9 months each

• Report data supporting the role of the hydrostatic and pulsatile forces

of CSF in the symptomatology of the cysts.

• External lumbar CSF drainage my be used as adjuvant diagnostic tool

if doubt exists about resolution of symptoms with intervention

• Although proposed by the authors as such, not considered a viable

long-term treatment option as symptoms recur

Literature

Prevalence and percutaneous drainage of cysts of the sacral

nerve root sheath (Tarlov cysts). Paulsen RD, Call GA, Murtagh FR. AJNR Am J Neuroradiol. 1994 Feb;15(2):293-7

• 7 cysts were drained in 5 patients using a percutaneous CT-guided

aspiration method

• Instant pain relief lasted from 3 weeks to 6 months

• Cysts repressurized and the patients' symptoms returned.

• Technique can be a quick and simple way of at least attaining a pain-

free interval

Literature

• Authors report a decrease in the intraprocedural severe pain that

develops during aspiration, which is thought to be related to the

negative pressure retraction on the dura

CT-guided biopsy and aspiration

of Tarlov cysts may help in

proving the cyst is the cause of

the symptoms and guiding

appropriate therapy.

Large Tarlov cyst, causing back pain

when patient coughed, treated by

endoscopic placement of shunt from cyst

to peritoneum, with resolution of

symptoms.

Literature

• 4 patients treated initially with CT-guided aspiration with recurrent

symptoms within 17-28 weeks

• Results: Improvement or resolution of pain with no recurrence during

follow-up of 7-23 month periods

• Complications: 3/4 patients developed aseptic meningitis

• Authors postulated that resolution and lack of recurrence of

symptoms following fibrin glue injection may be due to stimulation of

fibroblasts and subsequent fibrosis that occurs with fibrin glue

resorption

AJR February

1997 vol. 168 no.

2 367-370

Literature

AJNR 2011 32: 1469-1473 AJNR 2011 32: 1469-1473

Literature

AJNR 2011 32: 1469-1473

Literature

AJNR 2011 32: 1469-1473

• Numerous techniques/strategies have been proposed

• Simple posterior sacral bony decompression has low success

rates

• Microsurgical excision consists of sacral laminectomy or

laminoplasty followed be resection of the wall of the cyst

– Nervous fibers of the parental nerve roots may lie directly in the walls

of the cyst

• Suturing walls of the cyst, neck ligation to close

communication of the cyst with the subarachnoid space

• Excising the cyst and sacrificing parental root?

• Absorbable gelatin sponge and/or fibrin glue and muscle

or fat patching to fill the cyst cavity and cover dural defects

– Neurologic worsening and cauda equina syndrome have been

reported

Surgical Options

Literature

• Mummaneni et al. Microsurgical Treatment of

Symptomatic Sacral Tarlov Cysts. Neurosurgery,

47(1); 2000, p74-79. – Retrospective review of 8 adult patients with radicular pain

– Performed sacral laminectomies with cyst fenestration and imbrication

– Closure reinforced with epidural fat or muscle grafts with fibrin glue

application.

– Improvement in symptoms in 7/8, bladder control improved in 2/3. No

CSF leaks or new deficits.

Mummaneni

Literature

Literature

Literature

Literature

• Yucesoy et al. Filling of a

Sacral Bone Defect From a

Perineural Cyst by

Cementation. JSDT, 15(6),

2002, p. 523-525.

– Case Report: Radicular

symptoms with Tarlov Cyst

– Partial excision, cyst imbrication,

methylmethacrylate filling of

sacral bone defect

Literature

• D. Long (in preparation)

– 456 consecutive patients (90% female, age 27-68

years) referred for evaluation and treatment of

perineural cysts

– 424 patients had identifiable cysts

• 53% unilateral, single root

• 37% bilateral, single level

• 10% bilateral, multiple nerve roots

• 32 patients had other cysts (dural ectasia and internal

meningocele)

Literature (Long, cont.)

• 220 patients excluded from study due to loss of

follow-up at one year, pain generator not felt to be

cyst-related, other causes

• 204 patients included in study (90% female)

• 113 patients had repeated diagnostic root block with

anesthetics of different half-lives

• 193 patients had pain relief with aspiration of cyst

Literature (Long, cont.)

• 75% of patients had specific or generalized lower

back pain and/or sciatica and/or perineal pain and/or

bladder/bowel dysfunction. 42% of patients had

decreased perineal sensation/pain with intercourse

• Follow-up MRI scans (all pts at 1 year, 100 pts at 2

years, 36 pts at 3 yrs and 28 pts at 5 years)

demonstrated that 96% unchanged in size

• CSF DRAINAGE!!! – In different series: 1/11, 1/3, 1/13 patients with postop CSF leaks

– Treatment: prolonged lumbar drainage

– Some authors have recommended routine postop lumbar drainage for

3-7 days to prevent CSF leakage and allow for graft healing

• Nerve root damage during excision

– Electrophysiological monitoring recommended by many authors

Complications

Personal Experience

• 23 cases over 21 years (last 12 in 5 years)

• Approach generally the same

– Osteoplastic laminotomy

– Imbrication of cyst

– Sealant

– +/- CSF drainage

– +/- Paraspinous muscle flap

• 2 patients no improvement

– both sealed well

• 59 yo F with severe low back pain, bilateral

posterior leg pain radiating into feet. Low

sacrum feels like “a hot torch”. Admits to

vaginal cramping, pelvic discomfort

• PSH: Cord detethering 4 years ago

• Exam: Neurologically intact, no deficits

appreciated

Case Presentation

• Previously underwent cyst aspiration followed by

fibrin glue injection with alleviation of symptoms

for 11 months

Case Presentation

Photos courtesy

of Dr. Don

Koenigsberg,

D.O.

Recent MRI

Case Presentation

Recent MRI

Case Presentation

Intraop

Case Presentation

Suggestions?

• Surgery included S1-3 osteoplastic laminotomy

• The dura was redundant, extremely thinned and

clearly leaking CSF into the sacral defect

• Pseudomeningocele?

• Tarlov cyst?

• Closed primarily (still leaking), subcutaneous

drains, bed rest, paraspinous muscle flap

Results

• “Ground glass” sensation completely resolved

• Leaked through incision after two weeks

Suggestions?

• Surgery included removal of S1-3 osteoplastic

laminotomy

• The dura was re-explored and could not be

primarily repaired any better than had been

done

• Plastic surgery closed over 4 drains with

paraspinous muscle flaps

• Subarachnoid drain placed

Outcome

• Bed rest for 2 weeks after discharge

• Dry, satisfied (overall) with results

Conclusions

• Relatively broad morphological criteria

• Similar symptoms

• Most appropriately not treated

• Interventional radiology treatments provide

some prognostication

• Surgery is customized, involved

• Generally speaking, outcomes good

Thank you for your

attention.

Neuro/Ortho Spine

Fellowship

available for

7/1/2014