Sudden Death in Hydrocephalic Spina bifida aperta Patients

Twelve patients of 146 hydrocephalic spina bifida aperta patients, treated with a ventricular drainage system, died suddenly. In 7 of them the cause of death was most probably related to the use of a ventriculovascular shunt, causing pulmonary embolism. Thromboembolic complications were not seen in the 26 hydrocephalic spina bifida patients treated with a ventriculoperitoneal shunt. In 4 other patients shunt malfunction was considered as the cause of sudden death. The case reports of these 11 patients are presented. Pathophysiological mechanisms are discussed and measures for prevention of these two potentially lethal complications are described.