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Adult-onset presentation of Dandy-Walker variant in siblings
1Department of Surgery, Northwestern University Medical School, Chicago, Illinois, USA.
Background:
The Dandy-Walker syndrome and Dandy-Walker variant usually present as isolated cases of hydrocephalus in pediatric patients.
Methods And Results:
THis paper consists of a case report of the adult onset of symptoms in two sisters having Dandy-Walker variant. Such an occurrence has never before been reported in the medical literature. Both patients presented with headaches and progressive neurologic deficit. On computed tomography (CT scan) of the head, both were found to have hydrocephalus, with hypoplasia of the inferior vermis. Both patients were treated successfully with ventriculoperitoneal shunting. A third sister, with a similar history, elected not to undergo CT scanning or surgical treatment.
Conclusions:
Variants of the Dandy-Walker syndrome may occasionally present clinically in the adult age group. Such an occurrence in siblings is consistent with an underlying genetic etiology.