CASE REPORT Indonesian Journal of Neurosurgery (IJN) 2020, Volume 3, Number 3: 115-118 P-ISSN.2089-1180, E-ISSN.2302-2914 Symptomatic Tarlov : A Case Report and Surgical Management

Sabri Ibrahim1, Victorio1*

ABSTRACT

Introduction: Perineural (Tarlov) are meningeal dilatations and bladder incontinence. Initially, she had been evaluated by her of the posterior spinal nerve root sheath that most often affect physician, and magnetic resonance imaging (MRI) was ordered. She sacral roots and can cause a progressive painful . had received physical therapy with little benefit and was referred Tarlov described a case of symptomatic perineural cyst and for physiatrists assessment. recommended its removal. Conclusion: Review of the patient’s original MRI scan showed the Case Presentation: A 30-year-old female physician presented presence of perineural (Tarlov) cysts within the sacral canal at the with a chief complaint of bilateral posterior thigh pain and low level of S1-S2, with compression of the adjacent nerve root. Tarlov back pain, which began insidiously approximately 6 months before cysts can be a rare cause of lumbosacral radiculopathy and should her initial examination. She had a one-month history of bowel be considered in the differential diagnosis of radicular leg pain.

Keywords: Arachnoid cysts; Case report; Radiculopathy; Tarlov cysts Cite This Article: Ibrahim, S., Victorio. 2020. Symptomatic Tarlov Cyst: A Case Report and Surgical Management. Indonesian Journal of Neurosurgery 3(3): 115-118. DOI: 10.15562/ijn.v3i3.83

1Division of Neurospine, Periveral INTRODUCTION: usually diagnosed on MRI, which reveals the lesion nerve and Pain Faculty of Medicine arising from the sacral nerve root close to the dorsal University of Sumatera Utara, Perineural (Tarlov) cysts are meningeal dilatations Medan, Indonesia root ganglion Tarlov advised extensive with of the posterior spinal nerve root sheath that most sacral laminectomy and excision of the cyst along often affect sacral roots and can cause a progressive with the nerve root.6 Paulsen reported CT-guided painful radiculopathy. The cyst can enlarge via a percutaneous aspiration of these perineural cysts net inflow of , eventually causing for the relief of . Recently, microsurgical symptoms by distorting, compressing, or stretching excision of the cyst has been advocated, combined adjacent nerve roots. Tarlov cysts were first described with duroplasty or plication of the cyst wall.12-13 by Tarlov in 1938, after he identified 5 cysts in 30 1 We report a case of a symptomatic Tarlov cyst adult patients at autopsy. Tarlov described a case presenting as bilateral posterior thigh pain and low of symptomatic perineural cyst and recommended back pain, to increase the awareness of this rare its removal. Since then, a few cases have been entity in the neurosurgical community. reported in the literature.2-4 In later writings, he described symptomatic cases of perineurial cysts PATIENT AND OBSERVATION that were successfully treated surgically with sacral laminectomy and cyst excision.5,6 Over the years, A 30-year-old woman presented with a 6-month * Corresponding to: others have reported cases of Tarlov cysts causing history of progressive, intractable pain of the Victorio; Division of Neurospine, 7 8-10 Periveral nerve and Pain Faculty of coccygodynia, sacral pain, sacral bilateral posterior thigh and low back pain and 11 Medicine University of Sumatera and sacral insufficiency fractures. numbness. Pain was aggravated on walking. At the Utara, Medan, Indonesia; The prevalence of Tarlov cysts has been time, he was becoming increasingly incapacitated, [email protected] estimated to be 1 to 4.6% among the general adult although he was still able to work. She rated his pain population.2-8 70% of the cysts are asymptomatic, as 8 of 10 possible points on a visual analogue scale. 17% have an additive effect on other pathological Pain was rapidly relieved in the recumbent position. entities, and only 13% are symptomatic.5 She had a one-month history of bowel and bladder The patient may present with low back pain, incontinence. The pain was not associated with Received: 2019-09-27 sciatica, or . specific time, posture and it used to get relieved by Accepted: 2020-10-17 Because of severe myelopathy, the patients non-steroidal anti-inflammatory drugs (NSAID). Published: 2020-12-01 sometimes suffer sleep disorder.6 The cysts are For the last three months, the intensity and duration

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of pain had increased, which was now unrelieved Sensory examination showed diminished sensory by taking NSAIDs. The pain had progressed to the perception to pinprick on the soles of his feet and lower back and bilateral upper thigh up to the ankle. in S1 distribution. She denied any prior history of The pain was aggravated by activity and prolonged trauma or back injury. There was sensory deficit over standing and was more bothersome in the evening. the perineum. Anal sphincter tone and constriction On physical examination, a grade of 4/5 strength were abnormal. Knee jerks were normal. Bilateral was demonstrated in All lower limbs. Heel and ankle jerk was grade+. toe walking and knee bends were well performed. Preoperative MR imaging demonstrated a large sacral cyst arising within the thecal sac at S-1- S2, of around 7 x 4 x 3 cm with expansion of the osseous sacral central canal and enlargement of S-1 and S-2 neural foramina causing the compression of all adjacent nerve roots. The cyst did not fill with contrast material and appeared to have no communication with the spinal subarachnoid space. X-ray of the lumbosacral spine did not reveal any abnormality

DISCUSSION Tarlov Cysts are common and usually detected incidentally during magnetic resonance imaging of the lumbosacral spine. Tarlov cysts are rare causes of low back pain. They are more common in females. It is generally agreed that asymptomatic Tarlov cysts do not require treatment. When symptomatic, the potential surgery-related benefit and the specific surgical intervention remain controversial. Tarlov’s initial descriptions of perineurial cysts were primarily localised to the posterior sacral or coccygeal nerve roots, most often the second or third sacral roots. These cysts generally occur at the junction of the posterior root Figure 1. MRI Lumbosacral Magnetic Resonance and the dorsal ganglion and are located between Imaging showing a Tarlov cyst at S1- S2 the perineurium and endoneurium. They are filled with cerebrospinal fluid (CSF) and are static in size. The clinical presentation of Tarlov cysts is variable. The cysts may cause local and/or . The dominant syndrome is preferable to the caudal nerve roots, either sciatica, sacral or buttocks pain, vaginal or penile paranesthaesia or sensory changes over the buttocks, perineal area and lower extremity. Depending on their location, size and relationship to the nerve roots, they may cause sensory disturbances or motor deficits to the point of bladder dysfunction. Tenderness on firm pressure over the sacrum may be present. Commonly, the symptomatology is intermittent at its onset and is most frequently exacerbated by standing, walking and coughing. Bed rest alleviates the discomfort.8 Microscopic features of the cyst were described by Tarlov. The early stage in cyst formation is that of a space between the arachnoid which covers the root or the perineurium and the outer layer of the pia cover of the root or the endoneurium. It usually begins in one portion of the circumference of the Figure 2. Axial MRI Revealing a Large Cyst in Sacral Area perineural space, with larger cysts compressing

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or of the sacral foramina.5 Myelography studies using oil-based (iophendylate) contrast resulted in delayed filling of the cyst. Current studies using water-soluble (metrizamide) contrast allow for a more rapid filling of the cyst.5,9,,10,12 Unenhanced CT scans may show sacral erosions, asymmetric epidural fat distribution, and cystic masses that are isodense with CSF.9-12 In the event that an MRI cannot be performed, a CT myelogram can be used. Treatment includes both conservative and surgical approaches. Conservative treatment generally consists of the appropriate use of pain medication, and a physical therapy program. Tarlov cysts are a documented cause of sacral radiculopathy and other radicular pain syndromes. They must be considered in the differential diagnosis of patients presenting with these clinical Figure 3. After Sacral laminectomy (S2) the thin transparent presentations and appropriately treated by cyst cyst wall membrane was eksposed excision. To relieve progressively incapacitating symptoms, surgery was recommended. In our case, after laminectomy of S2 in the prone position, a large cyst was identified at the S2 level of the cord. It was situated near the of S2. It was aspirated and discovered to be filled with cerebrospinal fluid; the Valsalva manoeuvre was used for approval. No inflow of cerebrospinal fluid was evident during a repeat Valsalva manoeuvre. The posterolateral wall of the cyst was resected after electrical stimulation verified that no motor nerve fibres were present. Because nerve fibres are often present in the cyst wall, risks include neurological deficits, urinary disturbances, as well as infection, spinal , and CSF leakage. Fibrin glue was then applied to fill the cyst cavity, to prevent cyst recurrence or CSF leakage, Although the cyst wall specimen was sent to the laboratory for pathological examination, its volume was inadequate to determine whether nerve root fibres were present Postoperatively, the patient reported marked Figure 4. The Tarlov cyst was aspirated pain relief. At month 3, the patient regained bowel and bladder control. Sensation and deep reflexes the nerve root to one side. The cyst occupies the were also improved. posterior root abutting the proximal portion of the dorsal ganglion. Its main part is bordered by a CONCLUSIONS reticulum or by nerve fibers.1 MRI is now the technique of choice in finding Tarlov cysts are a rare cause of low back pain perineural cysts, and is considered the imaging or lumbosacral radiculopathy and should be study of choice in identifying these cysts [5]. considered in the differential diagnosis of radicular Compared with CT scanning, MRI provides better symptoms. The diagnosis of a perineural cyst resolution of tissue density, an absence of bone should not be precluded based on location. MRI is interference, multiplanar capabilities, and is non- currently the gold standard modality for revealing invasive.5-11 Because these cysts are filled with CSF, such cysts and is a useful tool for evaluation and a low signal is seen on T1 and a high signal is noted surgical planning. Treatment is indicated only on T2.5-11 Plain films may show bony erosions of the when the cyst is symptomatic. Options include external cerebrospinal fluid drainage, percutaneous

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cyst drainage, percutaneous fibrin glue injection, 2. Chaiyabud P, Suwanpratheep K. Symptomatic Tarlov cyst: insertion of a cyst-subarachnoid shunt, a cyst- Report and review. J Med Assoc Thai. 2006; 89(7): 1047–50 3. Acosta FL, Jr, Quinones-Hinojosa A, Schmidt MH, peritoneal shunt or a lumboperitoneal shunt, Weinstein PR. Diagnosis and management of sacral Tarlov simple decompressive laminectomy, resection cysts Case report and review of the literature. Neurosurg of the cyst neck, cyst wall resection, and cyst Focus. 2003; 15(2):E15. imbrications. Our patient appreciated the relief of 4. Paulsen RD, Call GA, Murtagh FR. Prevalence and pain immediately after surgery. The postoperative percutaneous drainage of cysts of the sacral nerve root sheath (Tarlov cysts) AJNR Am J Neuroradiol. 1994; 15(2): period was uneventful and the patient made a 293–299 prompt recovery. At the three month follow-up, the 5. Langdown AJ, Grundy JR, Birch NC. The clinical relevance patient had no pain in their lower limbs and back. of Tarlov cysts. J Spinal Disord Tech 2005; 18(1): 29–33. The patient is back at their job and is asymptomatic. 6. Tarlov IM. Spinal perineural and meningeal cysts. J Neurol Neurosurg Psychiatry 1970; 33(6): 833-843. Tarlov cysts are well treatable entities and significant 7. Ziegler DK, Batnitzky S. Coccygodynia caused by a symptomatic relief is achievable. perineurial cyst. 1984; 34(6): 829-30. 8. Paulsen RD, Call GA, Murtagh FR. Prevalence and COMPETING INTEREST percutaneous drainage of cysts of the sacral nerve root sheath (Tarlov cysts). Am J Neuroradiol 1994; 15(2): 293- The authors declare no conflicts of interest 297 9. Davis DH, Wilkinson TJ, Teaford DA, Smigiel MR. Sciatica produced by a sacral perineurial cyst. Tex Med 1987; AUTHORS CONTRIBUTIONS 83(1):55-56. 10. Schreiber F, Haddad B. Lumbar and sacral perineural cysts Conception and design: SI, V causing pain. J Neurosurg 1951; 8(1): 504-509. Data Collection: SI, V 11. Peh WC, Evans NS. Tarlov cysts—another cause of sacral Drafting the article: SI, V insufficiency fractures? Clin Radiol 1992; 46(5): 329-330­­ Critically revising the article: SI, V 12. Rodziewicz GS, Kaufman B, Spetzler RF. Diagnosis of sacral perineural cysts by nuclear magnetic resonance. Surg Reviewed submitted version of manuscript: all Neurol. 1984; 22(2): 50–52. authors. 13. Caspar W, Papavero L, Nabhan A, Loew C, Ahlhelm F. Approved the final version of the manuscript: all Microsurgical excision of symptomatic sacral perineurial authors. cysts: A study of 15 cases. Surg Neurol. 2003; 59(2): 101– 106. 14. Klkarni RA. A case of symptomatic lumbar perineural cyst. ACKNOWLEDGEMENTS J Recent Adv pain 2015; 1(2): 95-96. 15. Haddadi K, Ganjeh QHR, Shayan MH, Saleh AM. A Rare All Author thank to The 1st Asia Australasian Huge Sacral Tarlov Cyst with Progressive Neurologic Neuro and Health Science International Conference Deficit: A Case Report. IrJNS. 2016; 2(2): 30-33 (AANHS).

REFERENCES 1. Tarlov IM. Perineural cysts of spinal nerve roots. Arch Neurol Psychiatry. 1938; 40(2): 1067-1074.

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