MACULAR EXUDATION ASSOCIATED WITH A RACEMOSE HEMANGIOMA, VASOPROLIFERATIVE TUMOR, AND COATS-LIKE VASCULOPATHY

The purpose of this study was to describe the clinical course and multimodal chorioretinal imaging of an eye that had a concurrent racemose hemangioma, retinal vasoproliferative tumor, and Coats-like vasculopathy.

Comprehensive ophthalmic examinations and multimodal imaging studies were performed initially, during sessions of laser photocoagulation and aflibercept therapy for the patient's macular exudation and vasoproliferative tumor, and at subsequent follow-up clinical visits for a period of 2 years after therapy.

A 14-year-old boy presented with 20/400 visual acuity in his right eye. Examination revealed macular exudation associated with a group 3 racemose hemangioma, a vasoproliferative tumor nasal to the optic disc, and a sclerosed retinal vein with collateral vessels in the temporal periphery from a prior branch retinal vein occlusion. Additional findings in the right eye included peripheral retinal telangiectasis, aneurysms, and extensive capillary nonperfusion consistent with a Coats-like vasculopathy. The left eye was normal. Systemic testing revealed no abnormalities. Macular exudation resolved and the vasoproliferative tumor regressed after laser photocoagulation and three monthly intravitreal aflibercept injections. The best-corrected visual acuity in the right eye was 20/40 2 years after treatment.

The unusual combination of uncommon retinal vascular abnormalities in the patient's right eye showed marked improvement after laser photocoagulation and anti-VEGF therapy. Racemose hemangioma exudation and abnormal retinal hemodynamics may have contributed to development of the vasoproliferative tumor and Coats-like vasculopathy. This case demonstrates that targeted treatment of exudation and vasoproliferation can provide significant visual improvement despite a patient's complex combination of retinal vascular abnormalities.