The Dandy-Walker syndrome--a 10-year experience of its management and outcome.
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.
Kalidasan
V
V
Our Lady's Hospital for Sick Children, Crumlin, Dublin, Republic of Ireland.
Carroll
T
T
Allcutt
D
D
Fitzgerald
R J
RJ
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