Sphenoethmoidal encephalomeningocele and midline anomalies of face and brain

An autopsy case of sphenoethmoidal encephalomeningocele associated with anophthalmia, agenesis of the corpus callosum, cleft palate and nasal septum defect is presented. A small colloid cyst and a cyst of unknown nature were found in the third ventricle. Based on a review of the literature, a peculiar association of sphenoethmoidal encephalomeningocele with callosal defect and midline facial anomalies seems not to be fortuitous and we proposed to call it "Sakoda complex" as a distinctive disease entity. Pathogenetic mechanism and significance of separation of this syndrome are discussed from a embryological standpoint. Anophthalmia and the cyst of unknown nature are interpreted as midline anomalies that may be attributed to the same pathogenetic cause.