CLINICAL AND DEVELOPMENTAL FINDINGS IN CHILDREN WITH GIANT INTERHEMISPHERIC CYSTS AND DYSGENESIS OF THE CORPUS-CALLOSUM

Giant interhemispheric cysts in association with dysgenesis of the cor pus callosum are rare. Clinical and developmental data concerning affe cted patients are limited, the pathologic basis for these cysts has no t been established, and prognosis is uncertain. The purpose of our stu dy was to describe the clinical and developmental findings for 11 chil dren with giant interhemispheric cysts, dysgenesis of the corpus callo sum, and ventricular dilatation. Eight of the children required ventri culoperitoneal shunting, eight had normal neurologic examinations, and seven had experienced seizures. Seizure control appeared to correlate with neurodevelopmental function. Cognitively, children ranged from t he average to mildly/moderately retarded level, Language delays were f requent, particularly in children with predominantly left hemispheric cysts. Adaptive behavior was mildly delayed with weakness observed in motor skills, Despite the striking neuroimaging abnormalities, our fin dings suggest the possibility of only mildly delayed neurodevelopmenta l outcome is good for children with this constellation of neurologic a nomalies.