Communicating Hydrocephalus: A Reversible Complication of the Mustard Operation with Serial Hemodynamics and Long-term Follow-up

Abstract

Severe superior vena caval (SVC) obstruction following the Mustard operation resulted in substantially increased intracranial pressure in 3 patients. A pathophysiological mechanism is postulated to explain the cerebral edema in 1 and the communicating hydrocephalus in 2. Following surgical relief of the obstruction in the latter 2, there was a decrease in both the rate of head growth and the ventricular size. It is of note that despite the angiographic appearance of azygous decompression, the pressure elevation from the SVC obstruction was transmitted to the brain. The long-term follow-up at three and four years of the 2 infants with communicating hydrocephalus suggests that aggressive treatment is compatible with a favorable outcome.