Neurofibromatosis and Hydrocephalus Bahattin Tanrıkulu and M. Memet Özek Contents Introduction ....................................................................................... 2 Neurofibromatosis 1 .............................................................................. 2 Diagnosis of Neurofibromatosis 1 ............................................................ 3 NF1 Disease Manifestations .................................................................. 4 Neurofibromatosis 1 and Hydrocephalus ..................................................... 5 Tumoral Causes ............................................................................... 6 Non-tumoral Causes .......................................................................... 7 Neurofibromatosis 2 .............................................................................. 9 Diagnosis of Neurofibromatosis 2 ............................................................ 10 Clinical Manifestations ........................................................................ 10 Neurofibromatosis 2 and Hydrocephalus ..................................................... 10 Treatment of Hydrocephalus in NF2 ......................................................... 11 References ........................................................................................ 13 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 hydroceph- alus 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 B. Tanrıkulu · M. M. Özek (*) Department of Neurosurgery, Division of Pediatric Neurosurgery, Acibadem University School of Medicine, Istanbul, Turkey e-mail: bahattintanrikulu@gmail.com; memetozek@gmail.com # Springer International Publishing AG, part of Springer Nature 2018 G. Cinalli et al. (eds.), Pediatric Hydrocephalus, https://doi.org/10.1007/978-3-319-31889-9_65-1 1