Abstract Introduction Perineural cysts, also known as Tarlov cysts, are cerebrospinal fluid-filled nerve root cysts predominantly located at the sacral spine. While typically asymptomatic, large Tarlov cysts may elicit discomforting symptoms. Diagnostic challenges stem from limited understanding and symptom overlap with various disorders. Objective This case report explores the presentation of a 41-year-old female patient exhibiting symptoms of vulvar pain and urinary disturbances, which were ultimately attributed to a large Tarlov cyst, highlighting the complexities of diagnosis and management associated with this condition. Methods A 41-year-old female patient with a past medical history of linear scleroderma, chronic left hip pain with leg length discrepancy, and family history of endometriosis presented to her primary care physician with vulvar pain and urinary symptoms. She had burning vulvar pain, predominantly on the left side, radiating from the vulvar vestibule to the perineum, which gradually worsened over 6 months. She also had lower back pain radiating into her legs and calves, along with increased urinary frequency, urgency, retention and burning upon micturition. Patient underwent a 11-month treatment regimen following a referral to pelvic pain specialist, including being initiated on gabapentin and vaginal suppositories, which provided significant pain relief. However, over time, her vulvar pain gradually worsened, and gabapentin was replaced with pregabalin. She underwent six weeks of pelvic floor physical therapy and received a series of peripheral nerve blocks, including multiple pudendal nerve blocks, trigger point injections and hydrodissection of connective tissue around the nerves, yielding little pain relief. Due to persistent vulvar pain, the patient was referred to our clinic. Her examination revealed tenderness upon palpation over the lumbar-sacral spine and sacroiliac joint and para-spinal muscle spasm on the left side. She had provoked vestibulodynia that was predominately on the posterior aspect of the vestibule with a Q-tip test 8/10, and about 4/10 at anterior vestibule. She also continued to have tenderness in her pelvic floor muscles. Given her history and physical exam, a spinal pathology was suspected, and lumbosacral MRI was ordered. Results The lumbosacral MRI revealed a Tarlov cyst measuring 7.7 cm x 3.9 cm x 4.2 cm, extending from the L5-S1 disc level to the S3-S4 vertebral body junction. The patient was subsequently referred to a neurosurgeon who performed a twelve-hour surgery, including a dissection of the intrasacral meningeal cyst that was causing sacral nerve root compression and subsequent wrapping of the weakened right S2-S3 nerve roots. Postoperatively, the pain, although decreased, persisted due to nerve root thinning and long-term compression of the sacral nerve roots. She is currently being managed by our clinic, pain specialists and rehabilitation. A two-year recovery period is anticipated. Conclusions This case underscores the diagnostic challenge of vulvar pain in the context of a Tarlov cyst and emphasizes the necessity for heightened clinical suspicion and comprehensive evaluation. Additionally, the case highlights the importance of considering MRI of the lumbosacral spine as an early investigation in patients presenting with otherwise unexplained chronic vulvar pain, offering a valuable diagnostic tool for identifying conditions like Tarlov cysts that might otherwise remain elusive. Disclosure No.
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