Management of Tarlov cysts: an uncommon but potentially serious spinal column disease-review of the literature and experience with over 1000 referrals.

Tarlov cysts were thought to be anatomic variants of uncertain etiology and clinical significance when initially described over 80 years ago. They are often detected in routine lumbosacral imaging and generally not reported in a differential diagnosis. There is increasing evidence that at least some Tarlov cysts are symptomatic and can have a significant adverse impact on patients' health and well-being. Women are disproportionately affected with this condition, often presenting with long-standing pain and neurological dysfunctions. Significant gender bias has been a concern in the management of these patients. Unfortunately, there is no consensus on patient selection or management approaches for symptomatic Tarlov cysts. This review article updates information on the prevalence, diagnosis, clinical significance, and treatments of these cysts. Based on these findings and experience with over 1000 patient referrals, a treatment decision algorithm for symptomatic Tarlov cysts was constructed to provide guidance for appropriate management of patients with these complex cysts.

Tarlov Cysts and Premature Ejaculation.

Tarlov cysts adjacent to the spinal cord are usually asymptomatic and found incidentally via magnetic resonance imaging. On rare occasions, they increase in size to produce symptoms resembling disk herniation. We report a rare case of a sacral cyst resulting in premature ejaculation in a 32-year-old man who presented with pelvic pain and acquired premature ejaculation. Spinal nerve root decompression, excision of intraspinal Tarlov cyst, and spinal nerve root adhesion release surgery significantly improved his pain and premature ejaculation at a six-month follow-up.

Sacral Tarlov cysts: Neurophysiology abnormalities and correlation with pelvic sensory and visceral symptoms.

BACKGROUND AND PURPOSE: Recent studies suggest a possible association between Tarlov cysts (TCs), usually considered as incidental radiological findings, and neurological symptoms such as pain, numbness and urogenital complaints. The aim was to explore the relationship between TCs and sacral nerve root functions using pelvic neurophysiology tests, and to correlate changes with clinical symptoms and magnetic resonance imaging (MRI) findings. METHODS: Consecutive patients with sacral TCs, referred for pelvic neurophysiology testing and presenting with at least one symptom related to the pelvic area, participated in a cross-sectional review of symptoms using validated questionnaires. Findings of pelvic neurophysiology (pudendal sensory evoked potentials, sacral dermatomal sensory evoked potentials, external anal sphincter electromyography) and urodynamics testing were collected retrospectively. The relationship between neurophysiology, MRI findings and patients' symptoms was assessed using Fisher and ANOVA tests. RESULTS: Sixty-five females were included (mean age 51.2 ± 12.1 years). The commonest symptom was pain (92%). Urinary (91%), bowel (71%) and sexual (80%) symptoms were also frequently reported. Thirty-seven patients (57%) had abnormal neurophysiology findings reflecting sacral root dysfunction. No association was seen between MRI findings (size, location of the cysts, severity of compression) and neurophysiology. A negative association was observed between neurophysiology abnormalities and occurrence of urgency urinary incontinence (p = 0.03), detrusor overactivity (p < 0.01) and stress urinary incontinence (p = 0.04); however, there was no association with voiding difficulties. CONCLUSIONS: Contrary to current understanding, TCs are associated with injury to the sacral somatic innervation in the majority of patients with presumed symptomatic cysts. However, urinary incontinence is unlikely to be related to TC-induced nerve damage.

Tarlov cyst with self-healing cauda equina syndrome following combined spinal-epidural anesthesia: a case report.

BACKGROUND: Cauda Equina Syndrome (CES) after Combined Spinal-Epidural Anesthesia (CSEA) is a rare disease that most of the time need surgery to relieve spinal cord compression. CASE PRESENTATION: A 34-year-old male patient underwent a procedure for prolapse and hemorrhoids (PPH) under CSEA. Anesthesia and surgery were uneventful. However, the patient gradually experienced urinary retention, lower abdomen and back pain, changes in bowel habits and neurological dysfunction of the lower limbs when the catheter was removed. It was later determined that the patient had Tarlov cyst at the left S1 level in the sacral canal. Finally, the patient completely recovered 20 days after drug conservative therapy onset. CONCLUSION: This case suggests that CES might occur even after ordinary CSEA. The risk factors are drug neurotoxicity to ropivacaine and Tarlov cyst, which helped to accumulate ropivacaine. The development of ultrasound-guided CSEA and an ultrasound atlas of the spinal canal are required.

Endoscopic transforaminal treatment of a thoracic perineural cyst: a case report.

BACKGROUND AND IMPORTANCE: To describe the first case of a thoracic perineural cyst successfully treated using a direct thoracic transforaminal endoscopic approach. METHODS: Case report. CLINICAL PRESENTATION: A 66-year-old male presented with right-sided radicular pain in a T4 distribution. MRI of the thoracic spine revealed a right T4 perineural cyst caudally displacing the root in the T4-5 foramen. He had failed attempts at nonoperative management. The patient underwent an all endoscopic transforaminal perineural cyst decompression and resection as a same-day surgical procedure. Postoperatively, the patient noted near complete resolution of the preoperative radicular pain. A thoracic MRI with and without contrast was performed 3 months after surgery and showed no evidence of the preoperative perineural cyst and no symptom recurrence was noted by the patient. CONCLUSION: This case report presents the first safe and successful report of an all endoscopic transforaminal decompression and resection of a perineural cyst in the thoracic spine.

A novel surgical technique for treatment of symptomatic Tarlov cysts.

BACKGROUND: The aim of this retrospective study was to describe a novel, simple surgical technique for the treatment of symptomatic Tarlov cysts. METHODS: A total of 40 patients with symptomatic Tarlov cysts, admitted to our tertiary center between 1998 and 2019 constituted the study group. All patients underwent microsurgical puckering of the cyst, the technique we described to prevent a recurrence. Patients' symptoms, radiological findings, intraoperative findings, and clinical results were evaluated. RESULTS: Of the 40 patients (5 males, 35 females) whose charts were reviewed, the mean age was 28.4 (range, 17-61) years. The mean follow-up was 8 (range, 3 months to 21 years) years. Preoperatively, the most common symptoms were leg pain and numbness of the lower extremity. Postoperatively, no major complications were observed. Clinical progression was halted in all patients; 33 (82%) patients recovered completely and seven (17%) patients reported partial recovery. Cystic cavity persisted radiologically in five (12%) patients, decreased in size in 30 (75%) patients, and regressed completely in the remaining five (12%) patients. None of the patients had permanent neurological deficits. CONCLUSION: Puckering of the cyst membrane is a safe and easy-to-perform surgical technique for symptomatic Tarlov cysts. This technique can be used almost in all cases instead of the commonly used microsurgical cyst excision or cyst fenestration.

High Prevalence of Perineural Cysts in Patients with Fibromyalgia and Chronic Fatigue Syndrome.

OBJECTIVE: Pain in fibromyalgia (FM) and chronic fatigue syndrome (CFS) is assumed to originate from central sensitization. Perineural cysts or Tarlov cysts (TCs) are nerve root dilations resulting from pathologically increased cerebrospinal fluid pressure. These cysts initially affect sensory neurons and axons in dorsal root ganglia and produce sensory symptoms (pain and paresthesia). Symptomatic TC (STC) patients often complain about widespread pain and fatigue. Consequently, STC patients may initially be diagnosed with FM, CFS, or both. The objective of this study was to document the prevalence of TCs in patients diagnosed with FM or CFS. DESIGN: A retrospective study. SETTING: An outpatient clinic for musculoskeletal disorders. SUBJECTS: Patients diagnosed with FM according to the 1990 American College of Rheumatology criteria or with CFS according to the 1994 Centers for Disease Control criteria were selected. METHODS: Review of lumbar and sacral magnetic resonance imaging scans including TCs ≥5 mm in size. RESULTS: In total, 197 patients with FM, CFS, or both underwent magnetic resonance imaging. Ninety-one percent were women. The mean age was 48.1 (±11.9) years. TCs were observed in 39% of patients, with a mean size of 11.8 (±5.2) mm. In males, the prevalence was 12%, vs. 42% in females. CONCLUSIONS: In patients diagnosed with FM or CFS, the prevalence of TCs was three times higher than that in the general population. This observation supports the hypothesis that STCs, FM, and CFS may share the same pathophysiological mechanism, i.e., moderately increased cerebrospinal fluid pressure, causing irritation of neurons and axons in dorsal root ganglia.

Elimination of the check-valve mechanism of the sacral Tarlov cyst using a rotation flap technique in a pediatric patient: technical note.

OBJECTIVE: Symptomatic Tarlov cysts in children are not sufficiently reported and treatment methods for Tarlov cysts are still controversial. The goal of this manuscript is to introduce a new variation of the surgical technique. METHODS: We performed surgery to eliminate the one-way check valve mechanism of the Tarlov cyst in a 7-year-old female who presented with urinary and fecal incontinence. A relatively large S3 nerve root cyst showed a one-way check valve on computed tomography myelography. The inlet of the check valve was enlarged with rotation flap reconstruction. RESULTS: Two months after surgery, the patient had established normal sphincter control. MRI performed two years later showed that the treated cyst was collapsing, and no recurrence occurred. CONCLUSIONS: Rotation flap enlargement of the check valve inlet is a safe and efficacious option for the treatment of pediatric patients with sacral Tarlov cysts.

Evaluating the discordant relationship between Tarlov cysts and symptoms of pudendal neuralgia.

BACKGROUND: Pudendal neuralgia is a painful neuropathic condition involving the pudendal nerve dermatome. Tarlov cysts have been reported in the literature as another potential cause of chronic lumbosacral and pelvic pain. Notably, they are often located in the distribution of the pudendal nerve origin at the S2, S3, and S4 sacral nerve roots and it has been postulated that they may cause similar symptoms to pudendal neuralgia. Literature has been inconsistent on the clinical relevance of the cysts and if they are responsible for symptoms. OBJECTIVE: To evaluate the prevalence of S2-S4 Tarlov cysts at the pudendal nerve origin (S2-S4 sacral nerve roots) in patients specifically diagnosed with pudendal neuralgia, and establish association of patient symptoms with location of Tarlov cyst. STUDY DESIGN: A retrospective study was performed on 242 patients with pudendal neuralgia referred for pelvic magnetic resonance imaging from January 2010 to November 2012. Dedicated magnetic resonance imaging review evaluated for presence, level, site, and size of Tarlov cysts. Among those with demonstrable cysts, subsequent imaging data were collected and correlated with the patients' clinical site of symptoms. Statistical analysis was performed using χ2, Pearson χ2, and Fisher exact tests to assess significance. RESULTS: Thirty-nine (16.1%) patients demonstrated at least 1 sacral Tarlov cyst; and of the 38 patients with complete pain records, 31 (81.6%) had a mismatch in findings. A total of 50 Tarlov cysts were identified in the entire patient cohort. The majority of the Tarlov cysts were found at the S2-S3 level (32/50; 64%). Seventeen patients (44.7%) revealed unilateral discordant findings: unilateral symptoms on the opposite side as the Tarlov cyst. In addition, 14 (36.8%) patients were detected with bilateral discordant findings: 11 (28.9%) had bilateral symptoms with a unilateral Tarlov cyst, and 3 (7.9%) had unilateral symptoms with bilateral cysts. Concordant findings were only demonstrated in 7 patients (18.4%). No significant association was found between cyst size and pain laterality (P = .161), cyst volume and pain location (P = .546), or cyst size and unilateral vs bilateral pain (P = .997). CONCLUSION: The increased prevalence of Tarlov cysts is likely not the etiology of pudendal neuralgia, yet both could be due to similar pathogenesis from part of a focal or generalized condition.

Perrault syndrome with amenorrhea, infertility, Tarlov cyst, and degenerative disc.

Perrault syndrome is a rare autosomal recessive disorder that affects both males and females. The syndrome causes deafness in males, however females display gonadal dysgenesis along with sensorineural hearing loss. Herein, we present a 27-year-old female patient who is deaf and mute along with primary amenorrhea. Hormonal assays revealed hypergonadotropic hypogonadism and the karyotype was 46 XX. Pelvic ultrasound described a hypoplastic uterus and streak ovaries. MRI of the spine showed degenerative discs and Tarlov cysts. Whole exome sequencing identified a LARS2 mutation and the patient was diagnosed with Perrault syndrome type four (PRLTS4).

Management of perineural (Tarlov) cysts: a population-based cohort study and algorithm for the selection of surgical candidates.

OBJECTIVE: Perineural cysts, also known as Tarlov cysts, are cerebrospinal fluid-filled growths that develop at the intersection of a dorsal root ganglion and posterior nerve root. They are typically an asymptomatic and incidental finding during routine spine imaging. For symptomatic perineural cysts, there is little evidence on which treatment is most effective or when it is indicated. The aim of this study was to review our experience from a population-based cohort of patients with symptomatic perineural cysts and to propose an algorithm that could be used in the selection of surgical candidates. METHODS: A retrospective review was conducted of all adult (≥ 15 years) patients with symptomatic perineural cysts who were referred to Karolinska University Hospital between 2002 and 2018. RESULTS: Thirty-nine patients were included. The most common symptom was sciatica (n = 22). Cyst aspiration was performed in 28 patients, 24 of whom showed clinical improvement and were offered surgery. Microsurgical cyst fenestration was performed in 17 patients, 16 of whom showed clinical improvement at long-term follow-up. There were no surgical complications. Ten of the patients who were offered surgery chose to be treated conservatively instead, four of whom showed progression of symptoms at long-term follow-up. CONCLUSIONS: Microsurgical cyst fenestration seems to be a safe and effective option for symptomatic relief in patients with perineural cysts. Based on the results from our series and those of others, we propose an algorithm for the selection of surgical candidates.

Pelvic occupation: the challenges of treating a symptomatic tarlov cyst.

Tarlov (perineural) cysts are meningeal dilations of the posterior spinal nerve root sheath located in between the peri- and endoneurium. We present a patient with a symptomatic sacral Tarlov cyst and the technical challenges faced by surgically treating the lesion by disconnection of the cyst from the subarachnoid space.

Can patients with symptomatic Tarlov cysts be differentiated from patients with specific low back pain based on comprehensive history taking?

BACKGROUND: Tarlov cysts (TCs) are expanded nerve root sheaths that occur near the dorsal root ganglion and result from increased intraspinal hydrostatic pressure. TCs most frequently affect the lumbosacral plexus and therefore may cause specific symptoms such as perineal pain and neurogenic bladder, bowel, and sphincter problems. It has been estimated that 1% of the population has symptomatic Tarlov cysts (STCs). However, STCs appear to be underdiagnosed, with the pain reported by patients commonly attributed to degenerative alterations seen on MRI. The aim of the present study is to investigate the utility of a comprehensive questionnaire for use by physicians in establishing the diagnosis of STCs. METHODS: We compared questionnaire responses regarding patient history between 33 patients diagnosed with symptomatic TCs and 42 patients with chronic low back pain and sciatica due to disc problems or degenerative or inflammatory disorders. The diagnosis of STCs was confirmed using nerve conduction studies (NCS) and electromyography (EMG) of the sacral myotomes by an expert neurophysiologist. RESULTS: The questionnaire responses revealed specific differences in perineal symptoms (perineal pain, dyspareunia, coccygodynia), bowel symptoms (constipation, diarrhea), bladder symptoms (hesitation, retention, frequency), and anal sphincter problems (anal pain, mild fecal incontinence). Additionally, sitting, walking, and straining aggravated pain more frequently in STC patients, and STC patients were more often forced to stop working and/or reduce their social activities. CONCLUSIONS: Including the above-listed items in the patient history might facilitate differentiation of low back pain and sciatica due to STCs from that due to disc problems or degenerative or inflammatory disorders.

Atypical findings of perineural cysts on postmyelographic computed tomography: a case report of intermittent intercostal neuralgia caused by thoracic perineural cysts.

BACKGROUND: Perineural cysts are sometimes found incidentally with magnetic resonance imaging, and clinical symptoms requiring treatment are rare. Perineural cysts typically exhibit delayed filling with contrast medium on myelography, which is one of the criteria used by Tarlov to distinguish perineural cysts from meningeal diverticula. We present a case of multiple thoracolumbar perineural cysts, one of which was considered the cause of intermittent intercostal neuralgia with atypical findings on postmyelographic computed tomography seen as selective filling of contrast medium. CASE PRESENTATION: A 61-year-old woman presented with intermittent pain on her left chest wall with distribution of the pain corresponding to the T10 dermatome. Magnetic resonance imaging showed multiple thoracolumbar perineural cysts with the largest located at the left T10 nerve root. On postmyelographic computed tomography immediately after contrast medium injection, the largest cyst and another at left T9 showed selective filling of contrast medium, suggesting that inflow of cerebrospinal fluid to the cyst exceeded outflow. Three hours after the injection, the intensity of the cysts was similar to the intensity of the thecal sac, and by the next day, contrast enhancement was undetectable. The patient was treated with an intercostal nerve block at T10, and the pain subsided. However, after 9 months of observation, the neuralgia recurred, and the nerve block was repeated with good effect. There was no recurrence 22 months after the last nerve block. CONCLUSIONS: We concluded that intermittent elevation of cerebrospinal fluid pressure in the cyst caused the neuralgia because of an imbalance between cerebrospinal fluid inflow and outflow, and repeated intercostal nerve blocks resolved the neuralgia. Our case demonstrates the mechanism of cyst expansion.

Prevalence of simple and complex sacral perineural Tarlov cysts in a French cohort of adults and children.

OBJECTIVE: To determine the prevalence of simple and complex sacral perineural Tarlov cysts (TCs) in a cohort of children and adults. MATERIAL AND METHODS: Retrospective observational epidemiological study assessing 1100 consecutive sacral magnetic resonance (MR) studies, including 100 children and adolescents. All patients underwent 1.5T MR imaging with T1 and T2 weighted image acquisitions in sagittal and axial planes. All perineural cysts affecting the sacral nerve roots S1-S4 were quantitatively and qualitatively assessed. RESULTS: Two hundred and sixty-three sacral TCs were found in 132 adult patients (13.2%), with a female predominance (68%). None was found in children. The prevalence of TCs increased with age. The average number of cysts per patient was 2.0±1.2 with a maximum of 6 cysts in a single patient. Most of the cysts (87.5%) showed a homogenous central fluid collection and a parietal course of the nerve fibers. Complex patterns were present in 33 cysts (12.5%) within which 28 cysts showed endocystic crossing of nerve fibers and 5 cysts contained internal septations. Seventy cysts (26.6%) eroded the adjacent bone and 13 cysts (4.9%) extended to the pelvis. CONCLUSION: The prevalence of sacral TCs in our cohort corresponded to 13%, with a female predominance. Interestingly no TCs were found in children or adolescents (<18 years). In relation to the non-negligible percentage of complex cysts with internal septations, or endocystic crossing of nerve fibers, pre-interventional characterization of sacral TCs might help to choose an appropriate procedure in the treatment of rare symptomatic variants.

Giant cystic sacral schwannoma mimicking tarlov cyst: a case report.

PURPOSE: To present a rare case of a giant schwannoma of the sacrum mimicking a Tarlov cyst. METHODS: A 58-year-old woman had a 1-year history of low back pain. MRI revealed a large cystic mass in the sacral canal with bony erosion. Radiological diagnosis of Tarlov cyst was made. RESULTS: The patient underwent surgical treatment for the lesion, which revealed a solid mass. Histopathological examination of the tumor confirmed the diagnosis of schwannoma. The postoperative course was uneventful and the patient has had significant improvement in her pain 1 month postoperatively. CONCLUSION: Giant cystic schwannoma of the sacrum is a very rare diagnosis overlooked by practitioners for more common cystic etiologies, but its treatment is significantly different. Care should be taken to include this diagnosis in a differential for a cystic sacral mass.

Electromyographic Abnormalities Associated with Symptomatic Sacral Tarlov Cysts.

Tarlov or perineural cysts (TC) are commonly overlooked as a cause of sacral and ischial pain, and urogenital and bowel problems. TC can be seen on MRI, but are often considered asymptomatic. This is especially true for smaller cysts. Moreover, there are only few diagnostic characteristics that can be used to confirm that the cysts are the cause of the symptoms. As a consequence, a lot of controversy remains regarding the clinical importance of TC. Because of this underdiagnosed condition, patients often suffer for several years from unrecognized chronic neuropathic pain and neurological conditions. In this article, case reports of three patients with giant and smaller symptomatic sacral cysts are presented, in which electromyographic testing was performed to demonstrate nerve damage. We suggest that electromyography of the sacral nerve roots can be a reasonable tool for the diagnosis of symptomatic TC, as well as for the differentiation from other pathological entities causing sacral and ischial pain. Moreover, using electromyography it was also documented that smaller cysts of < 1 cm can cause nerve damage. Therefore incidence of symptomatic TC may be higher than initially thought.