Intraoperative Neuromonitoring Assists in Detecting Positioning-Associated Ischemia in Non-Spine Surgery in Morquio Syndrome: A Case Report

Morquio syndrome, a lysosomal storage disease, is associated with an increased risk of spinal cord ischemia with positioning changes.1 Though uncommon in non-spine surgeries, intraoperative neuromonitoring (IONM) can facilitate prompt detection of ischemia.2,3 We utilized IONM in an 11-year-old undergoing bilateral distal femoral osteotomies and lumbar epidural placement. After induction and supine baseline signal acquisition, the patient was positioned laterally. Signals were lost, and hypotension ensued. Phenylephrine and fluids were administered, but hypotension resolved only after supine repositioning. Without IONM, spinal cord ischemia may have caused permanent injury. This case emphasizes the importance of pre-positioning IONM in patients with Morquio Syndrome.