[Sphenoethmoidal meningoencephalocele associated with agenesis of corpus callosum and median cleft lip and palate--report of two cases (author's transl)].
Two cases of sphenoethmoidal meningoencephalocele are reported. Patients are a 1 year 7 month old boy and a 3 year old boy. Although sphenoethmoidal meningoencephalocele is reasonably classified as a type of basal meningoencephalocele, the authors could not find out any reports on cases designated as such. The reason must be in difficulty to differentiate the sphenoethmoidal type from the transsphenoidal type. Authors could differentiate them using CT scan. Two other cases were found in cases reported as transsphenoidal type. Further, it is assumed that these four cases including our two cases were accompanied with agenesis of corpus callosum and median cleft lip and palate. Despite the fact that the respective anomalies are rare, they are completely the same, which signifies the possibility that 1) sphenoethmoidal meningoencephalocele, 2) agenesis of corpus callosum and 3) median cleft lip and palate are a single unit of congenital anomalies. It probably should be considered that the cause for these malformations were present even before the formation of the lips which takes place earliest, that is, sixth week of gestation.
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging*; D061085:Agenesis of Corpus Callosum; D002675:Child, Preschool; D002971:Cleft Lip / Q000150:complications*; D002972:Cleft Palate / Q000150:complications*; D004677:Encephalocele / Q000150:complications* / Q000000981:diagnostic imaging; D005005:Ethmoid Sinus; D006801:Humans; D007223:Infant; D008297:Male; D008588:Meningocele / Q000150:complications* / Q000000981:diagnostic imaging; D011859:Radiography; D013101:Sphenoid Sinus