Median cerebrofacial dysgenesis: the syndrome of median facial defects with hypotelorism.

Two patients with almost identical median facial defects are described. Both patients manifested a pure Pitressin responsive diabetes insipidus. These two patients are part of a graded series of median cerebrofacial malformations with orbital hypotelorism, which have been grouped under the heading of Median Cerebrofacial Dysgenesis. A revised classification of this group of anomalies is presented. The importance of distinguishing this group of patients from the group with the Median Cleft Face Syndrome with hypertelorism is stressed. Careful attention to face-brain relationships will help elucidate our understanding of the embryogenesis of the facial region, and extend the number of diagnostic facies which currently can be recognized.