Keratoplasty in newborns with Peters' anomaly

Abstract

Severe Peters' anomaly with dense corneal opacities leads to blindness of the affected eye unless perforating keratoplasty is attempted. The optimal timing of this procedure has yet to be established. We performed keratoplasty early after birth in an attempt to optimally treat amblyopia. In eight eyes of five newborns with severe Peters' anomaly a first keratoplasty was performed at an average age of 54 days. A first control was done under general anesthesia 3 weeks thereafter, with subsequent controls being carried out according to the clinical course. Immunosuppressive therapy mostly consisted of topical steroid eye drops only. In two rekeratoplasty cases, systemic cyclosporin A was given in addition. Apart from the eight primary keratoplasties, three repeat keratoplasties, two lentectomies, and numerous glaucoma operations had to be performed. The average follow-up period was 46 months. As compared with the excellent results reported for penetrating keratoplasty in adults, the results obtained in this special group of newborns remain very poor. The observation of four eyes with a clear or partially clear graft and useful ambulatory vision might suggest a success rate of 50%. However, especially secondary glaucoma seems to be the limiting prognostic factor in the long run. At present, two of the four eyes continue to show uncontrolled intraocular pressure despite multiple surgical interventions, and their prognosis is poor. The performance of perforating keratoplasty in patients with Peters' anomaly early after birth is associated with a multitude of problems, especially glaucoma, and currently grafts can rarely be kept clear for an extended period. We would therefore conclude that it might be wise to postpone surgery until the patient is about 1 year old, in the hope that the overall chance for graft survival might be better at that point, even though persistent amblyopia might be quite severe and limit the functional success.