title: [A case of Klippel-Trenaunay-Weber syndrome accompanied by congenital hydrocephalus and micropolygyria].
abstract: We report a patient with the Klippel-Trenaunay-Weber syndrome accompanied by congenital hydrocephalus, which was slowly progressive and an Ommaya's reservoir was set up. However, the hydrocephalus remained stable even when the shunt was removed due to infection. Generalized tonic clonic convulsions had appeared from six months after birth and were treated with valproic acid. The electroencephalogram showed hypsarrhythmia. He died at eight months of age. Autopsy revealed extensive micropolygyria of the bilateral cerebral hemispheres and hydrocephalus. To our knowledge, it is rare for the Klippel-Trenaunay-Weber syndrome to be accompanied by congenital hydrocephalus, and there has been no previous report of its occurrence with micropolygyria.
authors.affiliation: Department of Pediatrics, Saitama Medical School.
authors.forename: H
authors.initials: H
authors.lastname: Shime
authors.forename: R
authors.initials: R
authors.lastname: Araki
authors.forename: H
authors.initials: H
authors.lastname: Koide
authors.forename: T
authors.initials: T
authors.lastname: Miyaji
authors.forename: K
authors.initials: K
authors.lastname: Shioda
mesh_terms: D001921:Brain / Q000002:abnormalities* / Q000473:pathology; D004569:Electroencephalography; D004829:Epilepsy, Generalized / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D006849:Hydrocephalus / Q000150:complications* / Q000473:pathology; D007231:Infant, Newborn; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications* / Q000473:pathology; D008297:Male; D014635:Valproic Acid / Q000627:therapeutic use
chemical_list: D014635:Valproic Acid