Summary

A 6-year-old girl, a case of xeroderma pigmentosum (XP), presented with a visual acuity (VA) of 20/20 and 20/400 in the right and left eye, respectively. For a diagnosis of vascularised corneal scar, penetrating keratoplasty was performed in the left eye twice and ultimately graft failed following multiple episodes of rejection. Type I keratoprosthesis was performed in the left eye. She continues to maintain VA of 20/40 in the left eye for more than a year. Similarly, a 24-year-old man, a case of XP, presented with VA of finger counting at 1 metre in both eyes. Ocular examination showed bilateral vascularised corneal scar and conjunctivalisation. Type I keratoprosthesis was performed as primary procedure in the left eye. He maintains a VA of 20/30 for more than a year. Type I keratoprosthesis could be a primary procedure for visual rehabilitation in patients with XP with severe ocular surface disease.