Abstract

Background: Tarlov cysts are sacral perineural cysts arising between the peri and endoneurium of the posterior spinal nerve root at the Dorsal Root Ganglion and have a global prevalence rate of 4.27%. These are primarily asymptomatic (only 1% with symptoms) and typically arise in females between the ages of 50–60. Patients’ symptoms include radicular pain, sensory dysesthesias, urinary and/or bowel symptoms, and sexual dysfunction. Non-surgical management with lumbar cerebrospinal fluid drainage and computerized tomography-guided cyst aspiration typically provide only months of improvement before recurring. Surgical treatment includes a laminectomy, cyst, and/or nerve root decompression with fenestration of the cyst and/ or imbrication. Early surgery for large cysts provides the longest symptom-free periods.

Case Description: A 30-year-old male presented with a very large magnetic resonance-documented Tarlov cyst (Nabors Type 2) arising from bilateral S2 nerve root sheaths with marked pelvic extension. Although he was initially treated with a S1, S2 laminectomy, closure of the dural defect, and excision/marsupialization of the cyst, he later required placement of a thecoperitoneal shunt (TP shunt).

Conclusion: A 30-year-old male with large Nabors Type 2 Tarlov cyst arising from both S2 nerve root sheaths required a S1-S2 laminectomy, dural closure/marsupialization, and imbrication of the cyst, eventually followed by placement of a TP shunt.

Keywords: Nabor cyst, Pelvic cyst, Spine, Tarlov cyst, Theco-peritoneal shunt

INTRODUCTION

We present a 30-year-old male whose lower extremity radicular and sphincteric complaints attributed to an magnetic resonance (MR)-documented Type 2 Tarlov cyst arising from both S2 nerve root sheaths. Although he underwent a laminectomy, closure of the bilateral dural fistulas with imbrication/marsupialization of the sacral cysts, he ultimately required placement of a thecoperitoneal (TP) shunt.

CASE DETAILS

A 30-year-old male presented with posterior left thigh pain of 18 months’ duration and 1 year of urinary frequency/urgency, constipation, and scrotal/perineal pain. The lumbosacral MR imaging (MRI) revealed a Tarlov cyst (Nabors Type 2) arising from bilateral S2 nerve root sheaths with marked pelvic extension.

Computerized tomography (CT) and MR findings

A Nabors-2 Tarlov cyst was diagnosed based on both lumbosacral CT and MR studies. The CT showed a hypodense lesion filling the pelvic cavity origination from bilateral S1 sacral roots, accompanied by chronic bony erosion of the sacrum at multiple levels (i.e., specially from S2 to 4) [ Figure 1 ]. The MR showed cerebrospinal fluid [CSF] signal cystic lesions with air-fluid levels, communicating with the thecal sac bilaterally, involving bilateral S1 and S2 neural foramina, and demonstrating anterior extension [ Figure 2 ]. The right lesion contained the S2 nerve root (measured 4.5 × 3.3 × 3.7 cm [Antero-Posterior (AP) x Transverse (TR) x Cranio-caudal (CC)]), while the left lesion (11 × 9.5 × 9.7 cm [AP × TR × CC]) contained the left S2 nerve root. The extremely large cyst displaced the urinary bladder and adjacent bowel loops inferolaterally. Notably, the cyst additionally contained irregular T2 hypointense septae. Further, the dural sac herniated bilaterally through the posterior lamina of the S1 and S2 vertebrae.

Figure 1:

Axial and sagittal computerized tomography scan of Lumbo-Sacral (LS) spine. Hypodense Expansile cystic lesion is seen filling the pelvic cavity reaching almost up to the anterior abdomen wall. It displaces the bladder and rectum posteroinferior with bony erosions of the sacrum from S2-4. Note the simultaneous spinal canal expansion at the S2-4 level.

Figure 2:

Preoperative axial and sagittal T2-W magnetic resonance imaging scan Lumbo-Sacral (LS) spine. The T2-hypointense lesion originates from bilateral neural foramen at the level of S2. Appreciate the septa between two sides of the cavities within the pelvis. Bony elements are seen missing at levels S2-4.

Surgery

Following a S1, S2 laminectomy, dural fistula closure, and excision/marsupialization of the cyst, he remained asymptomatic for a few months. However, 6 months later symptoms recurred at which point he underwent, lumbar drainage that relieved his urinary complaints; a TP shunt was then placed. At surgery, two separate large tense cysts were encountered extradurally surrounding the bilateral S2; these cysts extended ventrally into the pelvis. Another small cyst compressed the left S1 root as it exited the canal. The three cysts were incised and drained (i.e., around 1.5 L of Cerebro-Spinal Fluid (CSF). Biopsies were taken from the cyst walls, and the cysts were marsupialized. In addition, gel foam was placed over the neck of the bilateral cysts bilaterally in conjunction with the application of fibrin glue (i.e., over the dura and cyst necks).

Postoperative MR imaging

The postoperative spine/pelvic MR revealed air-fluid levels in both cysts which were reduced in size vs. the preoperative scans.

Postoperative symptoms

Postoperatively, the patient had severe headaches that resolved within the 1^st postoperative day by keeping the head of bed flat. By postoperative day 2, there was complete resolution of the deep pelvic pain and no further urinary symptoms. The remainder of the postoperative course was uneventful, and the patient was discharged on postoperative day 5.

Recurrent symptoms requiring placement of a TP shunt

Within 6 postoperative months, the patient presented with recurrent symptoms (i.e., pelvic pain and urinary dysfunction).The lumbosacral MR spine now revealed a posterior dural sac-CSF collection, measuring 4 × 4.4 × 10 cm (AP × TR × CC) with recurrent bilateral presacral tarlov cysts communicating with the thecal sac (i.e., thorough the neural foramina of S1-S2, right Cyst 4.6 × 3.3 × 4.2 cm, left cyst 12.3 × 12 × 15.4 cm [AP × TR × CC]) [ Figure 3 ]. Cysts extended into the pelvis, causing compression of the urinary bladder/ rectum, and were notably larger than the initial cysts, but did not contain the previously noted septae. A lumbar drain was inserted at the L3-L4 disk space, that improved the patient’s symptoms, and was followed by application of a TP shunt at the L4-L5 level [ Figure 4 ]. The postoperative course was uneventful, the patient remained asymptomatic up to the 1-year follow-up examination.

Figure 3:

Axial and sagittal magnetic resonance imaging scan of Lumbo-Sacral (LS) spine 6 months after index surgery. T1-hypointense and T2-hyperintense lesion is seen reappearing in the pelvic cavity. Posteriorly, the collection of cerebrospinal fluid can be noted at the level of surgery/laminectomy.

Figure 4:

Axial and sagittal computerized tomography scan revealing Theco-peritoneal (TP) shunt tip in the cavity of the spinal canal at the level of L1 vertebral body and reduction in the size of the cyst within the pelvic cavity. The white dots of the TP shunt can be seen within the peritoneal cavity.

DISCUSSION

Tarlov cysts are CSF-filled cavities contained by an epithelium first described in cadaveric surgery by Tarlov.[ 5 , 7 ] With a global prevalence rate of 4.27%,[ 3 , 14 ] they form between perineurium and endoneurium at dorsal nerve roots (DRG) and mostly at sacral levels.[ 16 ] They are reported as incidental findings 99% times because they are asymptomatic at the same percentage. If symptomatic, radiculopathy and perineal pain is the usual presentation along with urinary disturbances. Symptomatic patients constitute only 1% of the cases and can present with radicular pain, sensory dysesthesias, urinary and/or bowel symptoms, and sexual dysfunction.[ 4 , 5 , 10 ] Rare presentation of Tarlov cyst being mistaken for hydrosalpinx had also been reported.[ 1 , 29 ] Non-surgical management with lumbar CSF drainage and CT-guided cyst aspiration is scarcely reported to provide months of symptom-free periods before recurrence.[ 20 ] Neurosurgical interventions include laminectomy, cyst and/or nerve root excision, and fenestration of cyst and imbrication.[ 19 , 22 , 26 , 28 ] Interestingly, a double crush syndrome of concomitant Tarlov cyst with epidermoid in a 42-year-old woman was reported in year 2020. The case becomes more interesting when it is revealed that the epidermoid is Iatrogenic and acquired.[ 23 ] Although, there are few reports of Tarlov cyst presenting as adnexal mass in females, our case being a young male salpingo-ovarian mass was not a possibility. MRI makes the investigation of choice in diagnosing Tarlov cyst because it gives hyperintense signals to Tarlov cysts as it gives to CSF and helps explore fenestral continuation with subarachnoid space. X-ray, although a financially better investigation, detects Tarlov cysts at advanced stages of bone degradation and tissue infiltration-paravertebral round shadows.[ 14 ] Laminectomy and marsupialization of sacral Tarlov cyst have shown promising outcomes with low recurrence; thus, our case was managed likewise. Large Tarlov cysts (>3.5 cm) have been reported up to sizes as large as 5 cm (largest diameter),[ 16 , 18 , 34 ] while our case presents a rare Giant Tarlov cyst of 12.3 x 12 x 15.4 cm (APXTRXCC). Tarlov cysts are rare at thoracic level with only few case reports.[ 2 ] Authors have done a literature review[ 9 , 11 - 13 , 15 , 17 , 21 , 24 , 25 , 27 ] in Table-1 for all cases of Tarlov cysts published with emphasis on clinical parameters, treatment, and recurrence. Total of 39 cases were reported of which only 10 were males. Current case belongs to less common gender of Tarlov cyst. A common differential of Tarlov cyst to be kept in mind is spinal arachnoid cysts. Authors have discussed in detail about this rare spinal pathology in another review.[ 6 ]

Table 1:

Literature Review on Tarlov Cyst with emphasis on Treatment, Recurrence, and special observations.

Literature review

A review of the literature on Tarlov cysts showed that of the total 39 cases, the majority were females [ Tables 1 and 2 ].[ 30 - 33 , 35 - 39 ] They are attributed to either musculoskeletal defects (supported by Tarlov cysts in collagen disorders like Marfan) that enlarge due to the hydrodynamic forces of CSF (i.e., continuous or pulsatile). In this series, patients averaged 40.35 years of age with a female threefold predominance. While all (100%) have lumbar/sacral radiculopathy, only 50% have bladder and bowel complaints including perineal pain (10%). Over 40% present with multiple Tarlov cysts, only rare of these may be giant lesions extending into the pelvis. Laminectomies, direct dural occlusion of dural/subarachnoid fistulas, imbrication and marsupialization often result in cyst resolution. If and when they recur, TP shunts or lumboperitoneal shunts[ 8 ] may become an option.

Table 2:

Analysis of literature review with exceptional reports.

CONCLUSION

Tarlov cysts may become extremely large in the presacral region to the point where they contribute to significant pelvic pain and genitourinary symptoms mimicking a pelvic cavity tumor. Treatment typically consists of laminectomy, occlusion of the dural/subarchnoid fistula, and imbrication/ marsupialization of the cyst with select cases warranting the placement of TP shunts.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

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