ASSOCIATION OF ANOPHTHALMIA AND ESOPHAGEAL ATRESIA

We report on a boy and a girl with bilateral anophthalmia and proximal esophageal atresia, In addition to vestigial optic nerves and chiasma , MRI studies showed other central nervous system abnormalities; one h ad ectopic tissue in the hypothalamic region, and the other had genera lized ventriculomegaly associated with atrophy, Two other cases, both males, have been reported with anophthalmia and esophageal atresia as their only malformations, These 4 cases are reviewed in light of recen t advances in the understanding of ocular embryogenesis and of the mid brain as a developmental field, Concurrence of these defects appears t o be nonrandom. (C) 1995 Wiley-Liss, Inc.