Abstract

Background : Congenital hereditary endothelial dystrophy(CHED) with hearing loss (Harboyan syndrome) is a rare disorder of cornea.It presents as bilateral corneal clouding with severe visual loss,nystagmus and deafness. Optical Keratoplasty is a definitive treatment which can produce a substantial visual gain.
Purpose:To report visual outcome in 3 siblings operated with optical keratoplasty.
Method:A Muslim family with consanguinity reported in which three out of six children were found to have diffuse bilateral corneal opacity with nystagmus with severe visual loss and deafness.In these three children,optical Keratoplasty was performed in both the eyes.One child had complicated cataract which was operated in same sitting.
Result: One eye of each child showed signs of graft rejection and the other eye of each child was relatively better with clear cornea & had substantial visual gain.
Conclusion:In children of CHED, Optical Keratoplasty carries a relatively good surgical prognosis.