Neurofibromatosis and hydrocephalus

Abstract

Neurofibromatosis(NF) is one of the hereditary neurocutaneous syndromes characterized by multiple tumors or hamartomas of central and peripheral nervous system and also concomitant skin and musculoskeletal system manifestations. There are two main types of NF, NF1 and NF2. Tumors, hamartomas, and other developmental pathologies that interfere with the circulation or absorption of cerebrospinal fluid (CSF) may cause hydrocephalus in NF1. Large bilateral vestibular schwannomas and any other intracranial tumors related to NF2 may lead hydrocephalus by narrowing or obstructing the CSF passages. Treatment of hydrocephalus in NF depends on the underlying pathology that interferes with cerebrospinal fluid dynamics. Treatment strategies for hydrocephalus in NF include endoscopic third ventriculostomy, ventriculoperitoneal shunt implantation, and removal of the obstructive lesion.