TOPLESS OPTIC DISKS IN CHILDREN OF MOTHERS WITH TYPE-I DIABETES-MELLITUS

PURPOSE: To determine the prevalence of and risk factors for superior segmental optic nerve hypoplasia in offspring of mothers with type I d iabetes mellitus. METHODS: Thirty four subjects aged between 4 and 37 years, the children of 23 mothers who had type I diabetes mellitus at the time of pregnancy, aged between 4 and 37 years, were recruited fro m one institution and prospectively examined in search of an optic dis k-related anomaly. RESULTS: Of the 34 subjects, three females (8.8%) s howed classic ophthalmoscopic and peri metric features of superior seg mental optic nerve hypoplasia, bilateral in two patients and unilatera l in one, Pregnancies leading to affected children showed a tendency t o be shorter, birth weight to be lower, and control of maternal diabet es mellitus to be poorer compared with pregnancies resulting in unaffe cted children. No variable unique to the affected subjects as opposed to the unaffected majority could be identified. CONCLUSIONS: We found a superior segmental optic nerve hypoplasia, described as a ''topless disk,'' in three of 34 subjects (8.8%) at risk for this condition. Top less disk thus seems to be more common than was previously thought, po ssibly having been missed because of its subtle signs and only mild im pairment of visual performance in affected individuals. Female sex, sh ort gestation time, low birth weight, and poor maternal diabetes contr ol may represent additional risk factors for the development of a topl ess disk. Its pathogenesis remains obscure, but the responsible pathog enic event may occur in the perinatal period. (C) 1998 by Elsevier Sci ence Inc. All rights reserved.