[Long-term prognosis of Peters anomaly]

Abstract

Background: Peters anomaly normally presents in early childhood. Common features are central corneal opacities and dysgeneses of the anterior eye segment. Early surgery is commonly warranted to prevent deep amblyopia or because of severe glaucoma. We herein present the clinical outcomes of all patients treated in the Eye Center of the Albert Ludwigs University of Freiburg since 2005. Emphasis is placed on the Peters subtype.

Methods: Data were collected retrospectively by means of chart review. Kaplan-Meier analyses were used to estimate visual prognosis, the indication for keratoplasty, and the incidence of retinal detachment.

Results: A total of 23 patients were identified. Subtype distribution was 40% type 1, 50% type 2, and 10% Peters plus syndrome. Ten patients were female (45%). Mean age at first presentation was 5 years; mean follow-up period censored in terms of eyeball preservation was 2 years (0 months-8 years). At mean follow-up, 40.5% of all patients had undergone at least one keratoplasty (up to six per eye); 43% had undergone glaucoma surgery (cylophotocoagulation, trabeculectomy, implants) at this time. Important complications were retinal detachment (31%) and phthisis bulbi (15%). After 4 years, visual acuity in the better eye was at most 0.05 in every second patient.

Conclusion: Prognosis of visual acuity in Peters anomaly is poor. It is generally not possible to restore visual function in the long run, i. e., reading-grade visual acuity is rarely achieved. Surgical interventions are associated with a high risk of severe complications. Therefore, the young patients should be connected to institutions for visually impaired persons at an early stage.

Keywords: Keratoplasty; Peters anomaly; Peters-Subtype; Visual acuity; Visual prognosis.