title: The Dandy-Walker syndrome--a 10-year experience of its management and outcome.
abstract: Hydroephalus of childhood may be associated with intracranial haemorrhage, infection, aqueductal stenosis, Arnold-Chiari malformation (spina bifida), tumours or more uncommonly with the Dandy-Walker syndrome (DWS). We present our experience with the management of this condition over a 10-year period. Twelve children with a definite diagnosis of DWS are reviewed with regard to the age at diagnosis, investigations, treatment, associated problems and overall outcome. Two patients died; there was a high incidence of mental retardation (65%) and associated problems. All but one child had ventriculo-peritoneal shunting and two had cysto-peritoneal shunts in addition to their V-P shunts. There were no familial cases; one child had a chromosomal abnormality. Although the long-term outcome is dismal in most cases, about 35% of the children have a reasonable outcome and hence the condition warrants energetic treatment.
authors.affiliation: Our Lady's Hospital for Sick Children, Crumlin, Dublin, Republic of Ireland.
authors.forename: V
authors.initials: V
authors.lastname: Kalidasan
authors.forename: T
authors.initials: T
authors.lastname: Carroll
authors.forename: D
authors.initials: D
authors.lastname: Allcutt
authors.forename: R J
authors.initials: RJ
authors.lastname: Fitzgerald
mesh_terms: D002648:Child; D002675:Child, Preschool; D003616:Dandy-Walker Syndrome / Q000235:genetics / Q000401:mortality / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008607:Intellectual Disability / Q000209:etiology; D007621:Karyotyping; D008297:Male; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D015996:Survival Rate; D016896:Treatment Outcome; D017287:Ventriculoperitoneal Shunt