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 Endoscopic Third Ventriculostomy
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DOI：
10.3174/ajnr.a2357
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发表时间：
2011-04-01
影响因子：
3.5
通讯作者：
Ozek, M. M.
中科院分区：
文献类型：
--
作者：
Dincer, A.;Yener, U.;Ozek, M. M.
尽管与核因子-1相关的脑积水并不少见，但到目前为止，这些患者的MRI表现以及ETV在治疗与核因子-1相关的脑积水中的作用还没有得到彻底的研究。我们报告了54例核因子-1患者中7例与核因子-1相关的脑积水的磁共振成像表现。虽然梗阻的类型多种多样，包括导水管网状物、上皮髓粘连、导水管周围/顶盖错构瘤、小脑和桥脑被盖错构瘤、脑干胶质瘤或两者的组合，但在合并脑积水的NF-1患者中，错构瘤的存在是一致的。其中5例进行了8次ETV手术，随访时间长达53个月。所有接受ETV治疗的儿童在最近的检查中都没有分流。ETV可能是治疗与NF-1相关的脑积水的主要方法，无论梗阻的原因和程度如何。
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.