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Eur J Pediatr Surg 1995; 5: 16-18
DOI: 10.1055/s-2008-1066255
Original article

© Georg Thieme Verlag KG Stuttgart · New York

The Dandy-Walker Syndrome - A 10-Year Experience of its Management and Outcome

V.  Kalidasan , T.  Carroll , D.  Allcutt , R. J. Fitzgerald
  • Our Lady's Hospital for Sick Children, Crumlin, Dublin 12, The Children's Hopital, Temple Street, Dublin, Republic of Ireland
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Publication Date:
25 March 2008 (online)

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Abstract

Hydrocephalus 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.

Key words

Hydrocephalus - Dandy-Walker syndrome - Posterior cranial fossa cyst - Mental retardation