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Hydrocephalus in Patients with Neurofibromatosis Type 1: MR Imaging Findings and the Outcome of Endoscopic Third Ventriculostomy

Hydrocephalus in Patients with Neurofibromatosis Type 1: MR Imaging Findings and the Outcome of... SUMMARY: 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. Abbreviations cine PC cine phase-contrast CNS central nervous system ETV endoscopic third ventriculostomy 3D-CISS 3D constructive interference in steady state NF-1 neurofibromatosis type 1 TSE turbo spin-echo http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Neuroradiology American Journal of Neuroradiology

Hydrocephalus in Patients with Neurofibromatosis Type 1: MR Imaging Findings and the Outcome of Endoscopic Third Ventriculostomy

American Journal of Neuroradiology , Volume 32 (4): 643 – Apr 1, 2011

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Publisher
American Journal of Neuroradiology
Copyright
Copyright © 2011 by the American Society of Neuroradiology.
ISSN
0195-6108
eISSN
1936-959X
DOI
10.3174/ajnr.A2357
pmid
21330395
Publisher site
See Article on Publisher Site

Abstract

SUMMARY: 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. Abbreviations cine PC cine phase-contrast CNS central nervous system ETV endoscopic third ventriculostomy 3D-CISS 3D constructive interference in steady state NF-1 neurofibromatosis type 1 TSE turbo spin-echo

Journal

American Journal of NeuroradiologyAmerican Journal of Neuroradiology

Published: Apr 1, 2011

References