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 corpus callosum are rare. Clinical and developmental data concerning affected patients are limited, the pathologic basis for these cysts has not been established, and prognosis is uncertain. The purpose of our study was to describe the clinical and developmental findings for 11 children with giant interhemispheric cysts, dysgenesis of the corpus callosum, and ventricular dilatation. Eight of the children required ventriculoperitoneal shunting, eight had normal neurologic examinations, and seven had experienced seizures. Seizure control appeared to correlate with neurodevelopmental function. Cognitively, children ranged from the average to mildly/moderately retarded level, Language delays were frequent, 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 findings suggest the possibility of only mildly delayed neurodevelopmental outcome is good for children with this constellation of neurologic anomalies.