Endoscopic diagnosis and treatment of far distal obstructive hydrocephalus

Obstruction of the CSF circulation distal to the fourth ventricle is a rare cause of noncommunicating hydrocephalus. Endoscopic third ventriculostomy (ETV) represents one of the treatment options, but reports of results are rare.

Between March 1997 and June 2008, 20 ETVs in 20 patients (mean 32.4 years, range 1 month-79 years) for noncommunicating hydrocephalus distal to the fourth ventricle were undertaken. All patients suffered from severe internal hydrocephalus and typical clinical symptoms. In addition to the standard ETV, a transaqueductal inspection of the posterior fossa with a flexible scope was performed. All patients were prospectively followed.

An ETV was achieved in all patients. It was clinically successful in 15 of 20 patients (75%) with an improvement of 50% (three out of six) of the pediatric and of 83% (12 out of 14) of the adult population. A reduction of ventricle size was found in ten (50%). Five patients (25%) received ventriculoperitoneal shunting. A transaqueductal inspection of the posterior fossa cerebrospinal fluid (CSF) pathways was performed in 16. In the remaining four patients, no inspection with the flexible scope was done. One clinically silent fornix contusion and one CSF fistula which was treated conservatively occurred. There was no permanent morbidity.

ETV is a successful treatment option in CSF pathway obstructions distal to the fourth ventricle. Although the success rate particularly of the pediatric population appears to be lower than with other indications of obstructive hydrocephalus, a relevant part of the patient population improves after ventriculostomy and shunting can be avoided.