Abstract
BACKGROUND: Plexiform neurofibromas are extremely rarely found in the region of cauda equina and can pose a significant challenge in the diagnostic and management sense. To our knowledge, only 7 cases of cauda equina neurofibromatosis (CENF) have been reported up-to-date. CASE SUMMARY: We describe a case of a 55-year-old man with a 10 years history of progressive lower extremities weakness and bladder dysfunction. Before presenting, patient was misdiagnosed with idiopathic polyneuropathy. Lumbar spine MRI revealed a tortuous tumorous masses in the cauda equina region, extending through the Th12-L4 vertebrae. The patient underwent Th12-L3 Laminectomy with duraplasty. During the operation, the most enlarged electroneurographically silent nerve root was resected, anticipating inadequate decompression if nerve root was spared. The patient's neurological condition improved post-operatively, but urinary retention became the major complaint. We provide a follow-up period of 10 years. During this time, the patient's condition progressively worsened despite extensive decompression. The consequent MRI scans showed progressive enlargement of cauda equina roots and increasing lumbar stenosis, predominantly affecting L3-L4 segment. During the follow-up 8 years after the operation, the patient complained of worsening lower extremities sensorimotor function and neurogenic claudication. Subsequent MRI revealed lumbar spine stenosis at the level of L3-L4, requiring further decompression. The patient underwent a second surgery involving L4-L5 Laminectomy with duraplasty and L2-L5 transpedicular fixation. The post-operative period was uneventful. Latest follow-up 18 mo after the second surgery revealed substantial improvement in patient's well-being. CONCLUSION: CENF should be kept in mind during the differential diagnostic work-up for polyneuropathies. Management with an extensive decompression, duraplasty and primary spinal fixation represents a rational approach to achieve a sustained symptomatic improvement and superior overall outcome.