Fowler Syndrome Presenting as a Dandy-Walker Malformation: A Second Case Report

被引：9

作者：

Al-Adnani, Mudher ^{[1
]}

Kiho, Liina ^{[1
]}

Scheimberg, Irene ^{[1
]}

机构：

[1] Royal London Hosp, Dept Histopathol, London E1 1BB, England

来源：

PEDIATRIC AND DEVELOPMENTAL PATHOLOGY | 2009年 / 12卷 / 01期

关键词：

Dandy-Walker cyst; Fowler syndrome; proliferative vasculopathy; HYDRANENCEPHALIC-HYDROCEPHALIC SYNDROME; PROLIFERATIVE VASCULOPATHY; 2; SIBLINGS; FEATURES; VARIANT;

D O I：

10.2350/07-09-0348.1

中图分类号：

R36 [病理学];

学科分类号：

100104 ;

摘要：

Fowler syndrome, also known as proliferative vasculopathy and hydrocephaly-hydranencephaly, is a lethal condition characterized by hydrocephalus associated with progressive destruction of central nervous system tissue as a result of an unusual and characteristic proliferative vasculopathy. The occurrence of Fowler syndrome in consanguineous families and recurrence in both sexes are suggestive of an autosomal recessive transmission. We present the second case of Fowler syndrome presenting as a Dandy-Walker malformation, in a consanguineous family.

引用

页码：68 / 72

页数：5

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