August 1984

Septo-optic Dysplasia and Median Cleft Face Syndrome

Author Affiliations

IWK Hospital for Children and Dalhousie University Halifax, Nova Scotia, Canada

Am J Dis Child. 1984;138(8):795-796. doi:10.1001/archpedi.1984.02140460085032

Sir.—I would like to add some comments on the case reported by Stewart et al1 in AJDC. Although I greatly appreciate the interesting comment of the authors on the intricacy of multiple syndromes described by DeMorsier as "median cranioencephalic dysrhaphia," I have to disagree with their statement that the case reported fulfills the diagnostic criteria of septooptic dysplasia described by DeMorsier. Indeed, the photographic documentation provided by the authors does not show an optic nerve hypoplasia. The optic nerve presented (left eye) shows findings more in keeping with optic nerve coloboma.2 If the glial tissue of the central portion of that nerve would appear to be elevated on stereoscopic viewing, then a morning glory syndrome diagnosis would be more adequate. While both these entities have been described with the absence of corpus callosum and other midcranial and facial anomalies,3,4 the case reported by Stewart et al

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