Complex management of hydrocephalus secondary to choroid plexus hyperplasia.

Abstract Hyperplasia of the choroid plexus represents a rare cause of communicating hydrocephalus in children. Recent work has come to associate such pathology with genetic abnormalities (e.g. most particularly, perturbations in chromosome 9). Given such extensive cerebrospinal fluid (CSF) overproduction, patients with choroid plexus hyperplasia often fail CSF diversion and therefore require adjuvant interventions. Herein, the authors present the case of a male infant with a ventriculoperitoneal shunt (VPS) and radiographic choroid hyperplasia that presented to our institution with a massive abdominal hydrocele caused by an inability to absorb the significant amount of CSF drainage into the abdomen. The child was eventually treated with an endoscopic third ventriculostomy (ETV) and choroid plexus coagulation (CPC); however, he still required CSF diversion via a ventriculoatrial shunt (VAS). Of note, a genetic work-up revealed tetraploidy of chromosome 9.