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This 22YO female with genetically confirmed von Hippel-Lindau (VHL) disease presented with 2 weeks of decreased vision in her left eye. Before presenting to us, she had received multiple intravitreal anti-VEGF injections and cryotherapy to the left eye elsewhere. At our institution, she was treated with 3 additional anti-VEGF injections, laser photocoagulation, and eventually vitrectomy. Both her brother and her paternal uncle had VHL. She underwent a surgical resection of a cerebellar tumor several years earlier. Vision was 20/20 in her OD, and 20/600 OS.

Optos color RG imaging shows a temporal peripheral yellow-orange retinal capillary hemangioma fed and drained by dilated, tortuous vessels that emanate from and exit the optic disc. Multiple additional smaller angiomas are present, including one superotemporally. Scattered subretinal lipid and chorioretinal scarring are noted.

Macular OCT scanning shows outer retinal and subretinal fluid with hyperreflective lipid exudates. OCT through a temporal macular angioma shows a hyperreflective dome-shaped lesion with posterior shadowing. The temporal peripheral angioma shows severe angiographic leakage.

Learning Points:
Retinal capillary hemangiomas appear clinically as orange-red lesions, most commonly peripherally, and are associated with prominent, dilated, tortuous paired feeding and draining vessels. These lesions can cause severe vision loss or blindness from exudative retinal detachment and thus usually require aggressive treatment. Treatment options include thermal laser, photodynamic therapy, cryotherapy, and anti-VEGF injections.

More recently, oral belzutifan, an inhibitor of hypoxia-inducible factor 2 alpha, has emerged as a highly effective treatment for these lesions. In LITESPARK-004, a Phase 2 trial, all 16 eyes improved without new lesions after a mean follow-up of 37 months (Wiley et al., Ophthalmology 2024;131;1324-1332).

Retinal capillary hemangioma is also a predominant feature of VHL, an autosomal dominant condition caused by mutations in the VHL tumor suppressor gene. Patients develop benign and malignant tumors, including central nervous system hemangioblastoma, pheochromocytoma, and renal cell carcinoma.