Abstract

Children with myelodysplasia often develop tethering of their spinal cord within 2-3 years after correction of their initial birth defect, leading to either neurological or urological dysfunction. Surveillance of these children includes electrophysiologic/urodynamic (EMG/UDS) studies to monitor improvement or deterioration of their neurological function. We reviewed 39 patients who underwent surgical untethering from 1979 to 1994 to determine whether postoperative EMG/UDS studies were useful in assessing outcome after surgery. All patients had a complete neurologic examination and an EMG/UDS prior to and 3 months after surgery. Twenty patients (51%) showed improvement in their EMG/UDS study after untethering, 15 (39%) demonstrated no significant change, and 4 (10%) showed deterioration. Improvement consisted of either return of previously absent motor units, return of sacral reflexes, or improved bladder dynamics. In a further 3 patients, subsequent deterioration on serial postoperative EMG/UDS testing was the first indication of recurrent tethering. We conclude that serial EMG/UDS evaluation in patients at risk for tethering of the spinal cord may serve as a useful adjunct to close clinical observation.

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