Penetrating Keratoplasty (PKP) for Anterior Segment Dysgenesis (Peter's Anomaly)
Contributed by Matt Ward, MD and Kenneth Goins, MD
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This is an unfortunate case of a 2 year old boy with bilateral corneal opacification and lenticular adhesion due to anterior segment dysgenesis (Type II Peter's anomaly) and light perception vision OU. His parents were eager to find any avenue to enable some visual function. Peter's anomaly is a congenital corneal whitening that is descriptive of a spectrum of developmental disorder of the anterior segment--60% are bilateral. The disorder has been linked to mutations in the following genes: PAX6, PITX2, FOXC1, CYP1B1, MAF and MYOC.
Unfortunately, any case of pediatric keratoplasty is fraught with peril and has a low chance of success. Success is even less likely in the setting of keratolenticular adhesion due to the robust healing response in children and subsequent proliferative vitreoretinopathy and tractional retinal detachment. It is essential to assess for keratolenticular adhesion preoperatively using high frequency ultrasound biomicroscopy (UBM).
It was difficult to get a definitive view of the lens with UBM in this case and we could not be certain whether the lens was touching the cornea or not. The risks were discussed at length with the family and the decision was made to proceed. Unfortunately, there was near immediate vitreous loss requiring anterior vitrectomy. The child unfortunately did not gain vision as hoped and the postoperative course was complicated by retinal detachment.