Over a nine-year period, 63 viable newborns with myelomeningocele were consecutively treated, of whom 11 (17%) developed brainstem symptoms assessed to be potentially life-threatening. All 11 underwent brainstem decompression by cervical laminectomy with stent placement between the fourth ventricle and the spinal subarachnoid space, at a median age of 8 months. 86% survived to 60 months of age. Those with brainstem dysfunction had a significantly greater mortality than those without, despite aggressive neurosurgical management by brainstem decompression.