VITREORETINAL ABNORMALITIES DURING MACULAR HOLE REPAIR IN ALPORT SYNDROME.

Abstract

Purpose: The purpose of the study was to describe abnormal vitreoretinal findings during macular hole repair in Alport syndrome.

Methods: A case report of preoperative, intraoperative, and postoperative findings related to macular hole surgery in a patient with Alport syndrome was discussed.

Results: A 50-year-old woman with Alport syndrome was found to have bilateral full-thickness macular holes. Surgery was recommended for her left eye given recent onset of vision loss and smaller hole size (313 μ m). Intraoperatively, the vitreous was found to have fine fibrillar strands and to be abnormally adherent to the retinal surface. There was little to no internal limiting membrane present. Vitrectomy was performed with posterior cortical hyaloid peeling and 15% C3F8 gas tamponade. Two months after surgery, the macular hole was successfully closed.

Conclusion: Abnormal vitreous composition and adherence to the retinal surface may contribute to macular hole formation in patients with Alport syndrome. Standard surgical approaches including internal limiting membrane peeling may not be feasible, although thorough removal of the posterior cortical hyaloid may be sufficient to achieve macular hole closure.