A CASE OF CONGENITAL SCLEROCORNEA WITH INITIAL PK SUCCESS FOLLOWED BY REJECTION

Title A CASE OF CONGENITAL SCLEROCORNEA WITH INITIAL PK SUCCESS FOLLOWED BY REJECTION
Author, Co-Author Ellie Patounas, Nektaria Mandadakis, Jerome Sherman, Harriette Canellos
Topic
Year
2007
Day
Friday
Program Number
075311
Room
Affiliation
State University of New York, College of Optometry
Abstract BACKGROUND: Sclerocornea is an uncommon developmental abnormality of the anterior segment due to mesenchymal dysgenesis, resulting in an opacified cornea that blends into the sclera. Early keratoplasty should be considered, but the risk of complications and graft rejection is high.


CASE REPORT(S): An 18 year old Asian male presented to the SUNY University Optometric Center in 2002 with a diagnosis of bilateral congenital sclerocornea. There was no significant medical history, and the patient was not on any medications. Presenting visual acuities measured with the Designs for Vision chart were 4/600 OD, 4/400 OS. There was a high amplitude nystagmus and a 40^ constant alternating esotropia. Slit lamp examination revealed milky white, opaque corneas OU. Corneal diameter was measured to be 8.5 mm OD/OS, in contrast to axial lengths of 28.34 mm OD and 28.21 mm OS. Flash VEPs were normal in both eyes, and B-scan showed intact retinas. After discussing risks and benefits of surgery with the patient and his father, he underwent sequential penetrating keratoplasties in 2003, and was on various anti-inflammatory eyedrops OU until October 2006. The patient reported substantial subjective improvement in vision, contrast and color perception but VAs were only slightly better. The posterior pole was visualized and found to be normal within weeks after the PKs. Signs of graft rejection OU were noted in June 2004. Most recent examination in May 2007 revealed visual acuities of 6/300 OD and 6/600 OS. Slit lamp examination revealed corneal opacification of all layers, with epithelial staining and microcystic changes. The borders of the PK rings were noted OU. Repeat penetrating keratoplasty is being considered, with a course of oral cyclosporine prior to surgery to help decrease the risk of graft rejection.

CONCLUSIONS: Penetrating keratoplasty is a consideration in patients with congenital sclerocornea. An immunosuppressive drug such as cyclosporine, both systemically and topically, may be indicated to decrease the risk of graft rejection.
Affiliation of Co-Authors State University of New York, College of Optometry, State University of New York, College of Optometry, State University of New York, College of Optometry
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