Ocular Cicatricial Pemphigoid

An elderly patient with severe ocular cicatricial pemphigoid (OCP) and immunosuppressed with mycophenolate mofetil has previously lost the right eye due to corneal perforation and choroidal hemorrhage resulting in evisceration. After 5 years of quiescence, his left eye ocular surface decompensated secondary to severe limbal stem cell deficiency with nonhealing epithelial defects requiring complete tarsorrhaphy. He undergoes implantation of a Boston keratoprosthesis type 2 with a novel technique of eyelid closure to decrease risk of device extrusion. The patient remained with a full tarsorrhaphy for 6 weeks at which point a central window to the optical cylinder was opened. At 3.5 months postoperatively, the patient has successful healing around the cylinder with no extrusion or skin retraction. His visual acuity improved from light perception to 20/30 at near in the left eye, and now is fully functional.

Presentation Date: 04/13/2023

Issue Date: 04/21/2023