Surgical intervention for corneal involvement in xeroderma pigmentosa
Xeroderma pigmentosum (XP) is a rare autosomal recessive genetic disorder characterized by solar injury to exposed skin and ocular tissues due to an enzymatic defect in the ability to repair DNA damage caused by ultraviolet (UV) light . The cornea in XP may show dryness, haze, exposure keratitis, vascularization, and opacification .
Most patients present to the cornea clinic with a significantly scarred cornea which requires corneal transplantation for visual rehabilitation. In such cases, corneal surgeons are faced with the dilemma of performing penetrating keratoplasty (PK) versus lamellar keratoplasty (LK). Anterior LK is preferred over PK for management of corneal opacity as it eliminates the risk of endothelial rejection. The few reports on the outcomes of PK in XP are encouraging, but the feasibility and results of anterior LK in XP patients is not available in the literature.
According to the only reported in vivo study of corneal endothelium in XP, the patients were found...
KeywordsXeroderma Pigmentosum Corneal Endothelium Corneal Edema Penetrate Keratoplasty Deep Anterior Lamellar Keratoplasty
The authors would like to thank Hyderabad Eye Research Foundation and Hyderabad Eye Institute
Conflict of interest
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