Bilateral choroid plexus hyperplasia: a case report and management strategies

Choroid plexus hyperplasia (CPH) is a rare cause of cerebrospinal fluid (CSF) overproduction and shunt-resistant hydrocephalus in infants. If treated with a ventriculoperitoneal (VP) shunt, these patients secondarily develop CSF accumulation along the shunt tract and within the peritoneum. The surgical management of this condition is not as clearly defined as in the case of a choroid plexus papilloma or carcinoma.

An 8-day-old male patient presented with bulging fontanelle, head circumference = 42 cm (>98th percentile), and cranial ultrasound demonstrating communicating hydrocephalus with enlarged choroid plexuses. A VP shunt was inserted, and secondarily progressive hydrocephalus, shunt tract fluid accumulation, and ascites developed. The infant underwent staged bilateral plexectomy and is tolerating CSF diversion. The pathology confirmed CPH.

Of the cases reported in the literature, treatments have included CSF shunting, endoscopic coagulation, and craniotomy with plexectomy. CSF shunting was required in the majority but not all. Only those having undergone bilateral choroid plexectomy have been rendered shunt free.