Congenital opacities of the cornea.

CONGENITAL corneal opacities vary greatly in extent and location and are usually associated with other anomalies of the anterior segment. Although a variety of clinical terms, such as cornea plana, sclerocornea, Rieger's anomaly, Axenfeld's syndrome, has been used to describe these anomalies, recent evidence (Goldstein and Cogan, 1962) suggests that they are all closely related entities. Theodore (1944) believed that the anomalies were the result of an abnormal mesodermal development and supported the earlier views of Collins (1907) and Seefelder (1920), who attributed the corneal opacities to a defect in Descemet's membrane. Forsius (1961) has drawn attention to the familial occurrence of cornea plana which emphasizes the importance of genetic factors when considering the aetiology of these anomalies. However, many sporadic cases have been reported (Goldstein and Cogan, 1962), frequently in association with other systemic and neurological defects, and the possibility that some of the anomalies may be due to teratogenic agents should not be overlooked. A group of 7 patients has been examined in whom the principal clinical finding was dense congenital opacification of the cornea. The problems associated with their aetiology, diagnosis, and management will be illustrated by reference to the clinical histories and the pathological examination of three eyes.