Intraocular use of methotrexate (MTX) for the treatment of proliferative vitreoretinopathy (PVR) in congenital aniridia (CI) and a possible link to aniridia fibrosis syndrome (AFS) treatment

Abstract

Purpose: To present a case of aggressive proliferative vitreoretinopathy (PVR) managed with intraoperative and postoperative intravitreal methotrexate (MTX) in a patient with congenital aniridia (CI). Observations: A 41-year-old female with a history of CI, living-related conjunctival-kerato-limbal allograft transplantation, and multiple intraocular surgeries presents with tractional retinal detachment (TRD) and aggressive grade C PVR 52 days after a primary 23-gauge pars plana vitrectomy (PPV) with rhegmatogenous retinal detachment repair. She underwent 23-gauge PPV, TRD repair including membrane peeling of pre- and sub-retinal PVR, 5000 centistoke silicone oil exchange, endolaser, and MTX infusion. She received intravitreal 200 μg/0.05mL MTX every two weeks for a total of five injections before switching to monthly injections which have continued indefinitely. Five months after TRD repair, she had a small area of recurrent PVR inferiorly without associated retinal traction. She developed a small epithelial defect that resolved without complication. At 13 months, the patient remains at her visual acuity baseline of 20/125 and an attached retina without progression of PVR. Conclusions and importance: We report a favorable outcome in the management of aggressive PVR with intraoperative and postoperative intravitreal MTX in a patient with CI. Despite a history of limbal stem cell deficiency and receiving numerous MTX injections, keratopathy was minimal. Further research is required to study the safety and efficacy of MTX in the prophylaxis and treatment of aggressive fibrotic responses often seen in CI.

Keywords

• Proliferative vitreoretinopathy
• Retinal detachment
• Congenital aniridia
• Methotrexate
• Aniridia fibrosis syndrome