Diffuse Corneal Clouding in Siblings With Fetal Alcohol Syndrome Deepak P. Edward, M.D., Junping Li, M.D., Soichi Sawaguchi, M.D., Joel Sugar, M.D., Beatrice Y. J. T. Yue, Ph.D., and Mark O. M. Tso, M.D. Three of four siblings born to parents with a history of heavy alcohol abuse had bilateral diffusely cloudy corneas at birth. These three siblings, who had mild systemic features of fetal alcohol syndrome, underwent corneal transplantations, and their specimens were examined by light and electron microscopy. Histologically, the alterations in Bowman's layer ranged from thickening to total loss. There were varying degrees of corneal stromal edema. The unique pathologic feature in the corneas was the anomaly of the anterior band- ed zone of Descemet's membrane, which was either absent, poorly formed, or thinned in the central and peripheral cornea. The corneal endothelium was attenuated or multilayered. The diffuse clouding and the range of histo- logic abnormalities in the corneas might be related to the maternal alcohol abuse. CORNEAL CLOUDING at birth may result from a variety of disorders, including endothelial and stromal dystrophies, glaucoma, anterior cham- Accepted for publication Dec. 7, 1992. From the Georgiana Dvorak Theobald Ophthalmic Pathology Laboratory, Department of Ophthalmology and Visual Sciences, Lions of Illinois Eye Research Institute, UIC Eye Center, University of Illinois at Chica- go College of Medicine, Chicago, Illinois. This study was supported in part by core grant EYOI792 from the National Eye Institute, Bethesda, Maryland; gifts from the Lions of Illinois Foundation, Maywood, Illinois; an unrestricted research grant from Research to Prevent Blindness, Inc., New York, New York (UIC Eye Center); gifts from the Clifford Sawyer Estate; and a grant from the McGraw Foundation, Arlington Heights, Illinois (Dr. Tso). Reprint requests to Mark O. M. Tso, M.D., Georgiana Dvorak Theobald Ophthalmic Pathology Laboratory, Department of Ophthalmology and Visual Sciences, Lions of Illinois Eye Research Institute, UIC Eye Center, University of Illinois at Chicago College of Medicine, 1855 W. Taylor s-, Chicago, IL 60612. ber cleavage syndromes, and metabolic diseas- es.! Herein, we describe the unique clinicopatho- logic findings in three siblings with bilateral congenital corneal clouding among four chil- dren born to parents with a history of heavy alcohol abuse. Patients and Methods The mother of the four siblings gave a history of heavy alcohol abuse (-1.4 to 1.8 1of alcohol daily) through all four pregnancies. There was no family history of similar disease. The family pedigree is shown in Figure 1. Patient III-I The patient had bilateral cloudy corneas at birth. Preoperative examination disclosed that the patient responded to light in both eyes. Bilateral, diffuse ground-glass haze with nor- mal corneal diameters and an intraocular pres- sure of 24 mm Hg were noted (Fig. 2, left). Systemic examination disclosed a flat nasal bridge, elongated philtrum, thinner vermilion border to the upper lip, developmental delay, mental retardation, and marked growth retar- dation. The patient underwent corneal trans- plantations for the right eye at age 3 months and for the left eye at age 61J2 months. The grafts remained clear postoperatively (Fig. 2, right). At the last examination, the patient was able to follow light and maintain fixation with both eyes. Patient 111-2 The patient had cloudy corneas in both eyes at birth. The preoperative examination dis- closed that the patient responded to light and had bilateral nystagmus. Additionally, bilateral ground-glass corneal haze, especially in the 484 ©AMERICAN JOURNAL OF OPHTHALMOLOGY 115:484-493, APRIL, 1993