Dandy walker syndrome- True is -

Ans. is 'd' i.e., All are true o The Dandy-Walker malformation consists of a cystic expansion of the 4th ventricle in the posterior fossa and midline cerebellar hypoplasia, which results from a developmental failure of the roof of the 4th ventricle during embryogenesiso Approximately 90% of patients have hydrocephalus, and a significant number of children have associated anomalies, including agenesis of the posterior cerebellar vermis and corpus callosum. Infants present with a rapid increase in head size and a prominent occiput.o Transillumination of the skull may be positive. Most children have evidence of long-tract signs, cerebellar ataxia, and delayed motor and cognitive milestones, probably due to the associated structural anomalies.o The Dandy-Walker malformation is managed by shunting the cystic cavity (and on occasion the ventricles as well) in the presence of hydrocephalus.