Purpose: To report the outcome of tractional retinal detachment with retinal capillary hemangiomas after pars plana vitrectomy (PPV) and relaxing retinectomy in genotypically confirmed von Hippel-Lindau disease (VHL). Methods: Interventional case report of a 28-year-old patient with multiple and large retinal capillary hemangiomas underwent PPV combined with relaxing retinectomy. Results: Over the next several weeks, the retina recovered to reattachment and remained this way for 2 years. In excised angiomas, the finding of a missense Arg167Trp mutation and immunohistochemistry staining confirmed the diagnosis of VHL. Conclusion: Patients with tractional retinal detachment with hemangiomas secondary to VHL may benefit from PPV with relaxing retinectomy.
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