Congenital corneal opacities in a cornea referral practice

Purpose: To address the primary causes of the congenital corneal opacities seen on the Cornea Service at Wills Eye Hospital (Philadelphia, PA), the associated systemic anomalies and their management. Methods: Retrospective review of the medical records of all patients with the diagnosis of congenital corneal opacities seen at the Cornea Service from January 1, 1992 to June 30, 2003. Children 12 years old or younger at the first visit to our department were included in the study. We classified the location and extent of corneal pathology. We divided the management into medical and surgical. Results: Seventy-two eyes of 47 patients were included in the study. The mean follow-up time from the first to the last visit was 33.1 months. The most common primary cause of congenital corneal abnormalities was Peters anomaly (40.3%), followed by sclerocornea (18.1%), dermoid (15.3%), congenital glaucoma (6.9%), microphthalmia (4.2%), birth trauma, and metabolic disease (2.8%). Seven eyes (9.7%) were classified as idiopathic. Ten patients had systemic abnormalities associated with their ocular condition. The management was medical in 38 eyes (52.7%). Twenty-four eyes (32.4%) underwent only 1 penetrating keratoplasty (PK). Only 1 eye received a regraft during the follow-up period. Eight grafts failed during the follow-up period. Conclusion: The importance of this study is to share our experience with this rare entity, congenital corneal opacities, describing their clinical presentation and their management.