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The Dandy-Walker syndrome--a 10-year experience of its management and outcome
V Kalidasan1, T Carroll, D Allcutt
1Our Lady's Hospital for Sick Children, Crumlin, Dublin, Republic of Ireland.
Insights
Dandy-Walker syndrome (DWS) in children often leads to significant challenges, including high rates of mental retardation. Despite a generally poor prognosis, energetic treatment offers a reasonable outcome for about 35% of affected children.
Area of Science:
- Pediatric Neurosurgery
- Developmental Neurology
- Congenital Malformations
Background:
- Hydrocephalus in childhood has diverse etiologies, including Dandy-Walker syndrome (DWS).
- DWS is a less common cause of pediatric hydrocephalus, often associated with significant neurological deficits.
Purpose of the Study:
- To review the management and outcomes of pediatric DWS over a 10-year period.
- To analyze the impact of DWS on neurological development and survival in children.
Main Methods:
- Retrospective review of twelve children diagnosed with DWS.
- Analysis of diagnostic age, investigations, treatment modalities, associated conditions, and patient outcomes.
Main Results:
- Two of twelve patients died.
- A high incidence of mental retardation (65%) and associated problems were observed.
- Ventriculo-peritoneal shunting was the primary treatment, with two patients also receiving cysto-peritoneal shunts.
Conclusions:
- Dandy-Walker syndrome in children is associated with a high rate of morbidity, including significant cognitive impairment.
- Despite a generally dismal long-term outlook, approximately 35% of children experience a reasonable outcome, supporting aggressive management strategies.
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.