Large, Spontaneous Macular Hole with Posterior Pole Detachment in a Patient with Best Vitelliform Macular Dystrophy

PURPOSE: To describe the visual and anatomical outcomes in a patient with a full-thickness macular hole and Best vitelliform macular dystrophy. METHODS: The authors present a case of a large spontaneous macular hole with associated posterior pole detachment in a patient with a history of Best vitelliform macular dystrophy including clinical course and surgical outcome. PATIENT: The patient presented with a history of blurred central vision. He was known to have Best vitelliform macular dystrophy. Examination revealed BCVA ⁶/₃₆ (0.78 logMAR) and a full-thickness macular hole (1,102 µm) with a shallow posterior pole detachment extending to the vascular arcades. He underwent phacovitrectomy with silicone oil tamponade. Internal limiting membrane (ILM) peel was prohibited due to a very adherent posterior hyaloid membrane (PHM). RESULTS: The patient developed type 2 closure. He had oil removal in 14 months combined with PHM and ILM peel. Two months postoperatively, he had further reduction of the foveal defect and the retina remained flat. Final BCVA was ⁶/₂₄ (0.60 logMAR). CONCLUSION: Macular holes with Best disease are rare and are thought to be due to rupture of a cyst in the vitelliform stage or atrophy in later stages. This case outlines that closure of the macular hole, flattening of the detachment, and improvement in visual acuity is possible with vitrectomy and ILM peeling.