title: Fowler syndrome presenting as a Dandy-Walker malformation: a second case report.
abstract: 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.
authors.affiliation: Department of Histopathology, The Royal London Hospital, England, United Kingdom. madnani8@hotmail.com
authors.forename: Mudher
authors.initials: M
authors.lastname: Al-Adnani
authors.forename: Liina
authors.initials: L
authors.lastname: Kiho
authors.forename: Irene
authors.initials: I
authors.lastname: Scheimberg
mesh_terms: D000015:Abnormalities, Multiple / Q000473:pathology*; D000328:Adult; D001808:Blood Vessels / Q000473:pathology; D003616:Dandy-Walker Syndrome / Q000473:pathology*; D005260:Female; D006801:Humans; D006832:Hydranencephaly / Q000473:pathology; D006849:Hydrocephalus / Q000473:pathology; D008297:Male; D011247:Pregnancy