Delayed tetraplegia after thoracolumbar scoliosis surgery in Stuve-Wiedemann syndrome

Objective: We present 2 patients with Stuve-Wiedemann syndrome (SWS) who suffered delayed tetraparesis following posterior spinal surgery for scoliosis. Background Summary: Delayed tetraparesis after a syndromic thoracic scoliosis correction has never been reported. A cord injury distant from the surgical site is rare, and intraoperative neuromonitoring should be used to detect and prevent neurologic impairment. Methods: Review of medical charts. Results: Two patients with SWS suffered delayed tetraparesis 20 and 40 hours respectively after thoracolumbar posterior surgery. In one patient distal motor evoked potentials fell and recovered partially during surgery. In both patients, early postoperative neurologic examination was normal (in one of them except for the extensor hallucis 2/5). CT scan showed correct instrumentation placement and no compressive haematoma. MRI ruled out cord anomalies, but revealed in both patients identical cervical edema that was most likely secondary to ischemia. Angiogram revealed an absence of anterior cord vascular supply. Conclusions: Correction of severe deformities in syndromic patients may lead to stretch injuries of the spinal cord and its vascular supply. This in turn may lead to a neurological deficit extending beyond the limits of the spinal instrumentation. Abrupt postoperative neck pain may be an alert to this impending development. Close surveillance in the early postoperative period should be maintained in patients with SWS because a delayed neurological deficit can be present even hours afterwards and may be cranial to the surgical level.