PENETRATING KERATOPLASTY IN XERODERMA-PIGMENTOSUM - CASE-REPORTS AND REVIEW OF THE LITERATURE

Xeroderma pigmentosum is an uncommon inherited disorder characterized by hypersensitivity to ultraviolet radiation, with defective repair of DNA damage caused by short-wavelength radiation. Corneal complication s of this disorder may require penetrating keratoplasty for visual reh abilitation. Surgery is rarely undertaken in these eyes due to multipl e associated problems involving the ocular surface and the lids. We re port three cases of successful penetrating keratoplasty in xeroderma p igmentosum and review nine cases reported earlier. Successful grafts w ere achieved in all 12 eyes initially. Persistent superficial punctate keratopathy was observed in one eye and graft rejection episodes in f ive eyes. However, graft failure occurred due to an untreated rejectio n episode in only one eye. Another eye was treated by exenteration for recurrent ocular malignancies. This report highlights the encouraging results of penetrating keratoplasty in carefully selected patients of xeroderma pigmentosum with corneal pathology.