title: Hydrocephalus in patients with neurofibromatosis type 1: MR imaging findings and the outcome of endoscopic third ventriculostomy.
abstract: Although hydrocephalus associated with NF-1 is not rare, up to now the MR imaging findings in these patients and the role of ETV in the treatment of hydrocephalus associated with NF-1 have not been investigated thoroughly. We present the MR imaging findings of hydrocephalus associated with NF-1 in 7 of 54 patients with NF-1. Although the types of obstruction were various, including aqueductal web, superior velum medullary synechia, periaqueductal/tectal hamartomas, cerebellar and pontine tegmentum hamartomas, brain stem glioma, or a combination, the presence of hamartomas was a consistent finding in patients with NF-1 with hydrocephalus. In 5 cases, 8 ETV procedures were performed and followed for up to 53 months. All children treated with ETV were shunt-free at their most recent examinations. ETV may be the primary procedure for the treatment of hydrocephalus associated with NF-1, regardless of the cause and the level of the obstruction.
authors.affiliation: Department of Radiology, School of Medicine, Acibadem University, Istanbul, Turkey. adincer@asg.com.tr
authors.forename: A
authors.initials: A
authors.lastname: Dinçer
authors.forename: U
authors.initials: U
authors.lastname: Yener
authors.forename: M M
authors.initials: MM
authors.lastname: Özek
mesh_terms: D002648:Child; D002675:Child, Preschool; D005500:Follow-Up Studies; D006222:Hamartoma / Q000209:etiology / Q000473:pathology / Q000601:surgery; D006801:Humans; D006849:Hydrocephalus / Q000209:etiology / Q000473:pathology / Q000601:surgery; D008279:Magnetic Resonance Imaging; D009456:Neurofibromatosis 1 / Q000150:complications*; D013812:Therapeutics; D020542:Third Ventricle / Q000473:pathology / Q000601:surgery; D014696:Ventriculostomy