Abstract
Purpose:
To investigate the clinical course and outcome of penetrating keratoplasty in children with Peters anomaly
Methods:
This retrospective chart review study included 70 eyes from 48 patients diagnosed with Peters anomaly at the Hospital for Sick Children from 2000 to 2012. Clinical data was collected through the examination of patient records, clinical images and ultrasound.
Results:
Twenty-three patients (47.9%) had bilateral involvement, 20 (41.6%) had unilateral involvement and five (10.5%) had one eye with Peters anomaly and other anomalies in fellow eye (such as anophthalmos). Type I phenotype (no lenticular involvement) was found in 77% of eyes and the remainder had type II anomaly (lens involved). Penetrating keratoplasty (PKP) was performed in 44 eyes of 31 patients (63% of all eyes). The median age at first keratoplasty was 3.6 months. The majority of eyes received one graft, however 12% required two or more grafts (range 2-5). The rate of failure was 15% for the first graft and increased to greater than 60% for subsequent PKPs during an average follow up of 56 months. Type II Peters anomaly resulted in higher need for PKP (72% vs 58%) and graft failure requiring regrafting (30% vs 13%) than Peters anomaly type I. The graft was clear at last follow-up in 75% of all eyes (84% of type I and 64% of type II). One third of eyes developed glaucoma with mean age of onset of 19.7 months. More than 75% of eyes which had corneal transplant developed glaucoma after corneal transplant. Glaucoma incidence and age of onset were the same in both subtypes. Other treatments performed were optical iridectomy in 10%, rotational autograft in 3% and pharmacologic mydriasis in 3%.
Conclusions:
Children with Peters anomaly type II have a higher rate of PKP and graft rejections than those with a type I phenotype. Glaucoma was more likely to develop after PKP, suggesting that the etiology was either steroid-induced or related to chronic angle closure, further compromising any pre-existing goniodysgenesis.
Keywords: 479 cornea: clinical science