1135 - Adult Presentation of Spinal Dysraphism and Tethered Cord Syndrome: A Case Report

A 61-year-old male with a reported history of type two diabetes, and  “sacral cyst removal” at age eighteen presented to clinic with progressive lumbar and leg pain, bilateral leg weakness, bilateral hand numbness, chronic urinary urgency, and atypical gait. Examination revealed diffuse lower extremity weakness (left worse than right), brisk deep tendon reflexes, clawed toes, wide-based antalgic gait, and diminished sensation to light touch in the bilateral hands. Advanced neuroimaging of the spinal axis revealed a significantly low-lying conus medullaris terminating at S1-S2, a small non-expansile syrinx at L3-L4, missing posterior lumbar vertebral elements and upper sacral region, with hypoplastic spinous processes of L4-5 vertebrae, and multilevel cervical and lumbar spondylosis. He was evaluated by neurosurgery and recommended to undergo S1-S2 laminectomy and spinal cord detethering. 

Discussions: This case illustrates a rare late adulthood presentation of spinal dysraphism and tethered cord syndrome (TCS).  The majority of cases of TCS are diagnosed in childhood with adult cases accounting for roughly 15%. Older adults (age 65 or above) represent less than 2% of adult cases.  Spinal dysraphism, as well as previous spinal surgery, are key risk factors for TCS.  In this patient,  a remote history of sacral cyst excision without clear diagnosis of spinal dysraphism was reported, as well as upper extremity symptoms which would have been accounted for by TCS. 

Conclusions: This case highlights the importance of recognizing late presentations of symptomatic tethered cord syndrome in adults, particularly in the setting of childhood spinal dysraphism, which may be unrecognized.  Timely advanced imaging is essential for diagnosis and neurosurgical referral for pain relief and stabilization of neurological symptoms.