Dandy-Walker Syndrome: Experience at the Hospital for Sick Children, Toronto

Authors:
  • HHHarold J. Hoffman
    Harold J. Hoffman
  • BHBruce Hendrick
    Bruce Hendrick
  • RHRobin P. Humphreys
    Robin P. Humphreys
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Abstract
Thirty-five patients with the Dandy-Walker syndrome (DWS) treated over the years 1964-1987 at the Hospital for Sick Children, Toronto, were reviewed. Thirty-three patients (94%) had hydrocephalus at the time of diagnosis. The primary association of aqueduct stenosis was excluded by radiological investigation and clinical course in all patients. Associated central nervous system (CNS) anomalies were present in 10 patients. Seven had occipital encephalocele and in 1 of these there was an associated Klippel-Feil syndrome. Four patients had agenesis of the corpus callosum and 1 patient had polyporencephaly. The treatment of these patients has changed over the years in our institution so that during an earlier period, 3 patients were treated by resection of the cyst membranes. We then went through a period of shunting the lateral ventricle so that 21 patients were treated with either a lateral ventricle to peritoneal or lateral ventricle to atrial shunt. More recently, patients with the DWS have been treated with a cyst-peritoneal shunt and 10 patients were thus treated. Only 1 patient was treated with a simultaneous lateral ventricle and cyst-peritoneal shunt. Nine of the twenty-one patients (43%) with a primary lateral ventricle to peritoneum or lateral ventricle to atrial shunt developed a secondary aqueduct stenosis and an isolated fourth ventricle which required additional cyst-peritoneal shunting. Only 1 of the 10 patients treated with a primary cyst-peritoneal shunt required an additional lateral ventricle peritoneal shunt. Two patients initially treated with a lateral ventricle peritoneal shunt and with a primary cyst-peritoneal shunt subsequently had a percutaneous stereotactic third ventriculostomy. Both of these patients remained shunt-independent but 1 died in a traffic accident. Primary cyst-peritoneal shunting seems to be the treatment of choice for the DWS.