Rare Diseases Symptoms Automatic Extraction

Death in consequence of late failure of endoscopic third ventriculostomy.

[hydrocephalus with stenosis of the aqueduct of sylvius]

Late failure of originally successful endoscopic third ventriculostomy (ETV) is considerably less common than failure of ventriculo-peritoneal (V-P) shunt in treatment of hydrocephalus. Death in consequence of late ETV failure is a very rare complication, so far having been mentioned in literature in 13 patients only.We present the case of an 11-year-old girl who died in consequence of ETV failure 26 months after the endoscopic procedure. Histopathology proved the closure of stoma by gliotic tissue.We can assume that reduced compliance of the ventricular wall in long-term V-P shunt, aqueductal stenosis as a cause of hydrocephalus as well as rapid onset of clinical symptoms prior to ETV might be the cause of acute onset of intracranial hypertension after late ETV failure. Potential prevention of sudden death seems to be the implantation of subcutaneous reservoir during the endoscopic procedure, which allows simple and rapid reduction of intraventricular pressure.