Retinal racemose hemangioma presenting with a chorioretinal anastomosis

Purpose: To report a case of neovascular glaucoma in an 8-year-old male, secondary to a racemose hemangioma without associated intracranial arteriovenous malformation, highlighting the challenges in management and novel findings on optical coherence tomography angiography (OCTA).

Observations: An 8-year-old male initially presented with pain, redness, and blurred vision in the right eye. The patient was diagnosed with secondary neovascular glaucoma due to a racemose hemangioma. Urgent interventions included intravitreal bevacizumab injection and tube shunt surgery for persistently high intraocular pressure. Pars plana vitrectomy and scatter laser photocoagulation were eventually performed to manage a tractional retinal detachment and peripheral ischemia, respectively. OCTA imaging revealed a racemose hemangioma with a unique chorioretinal anastomosis.

Conclusions and importance: We present a rare pediatric case of neovascular glaucoma secondary to a racemose hemangioma with significant peripheral ischemia and an unusual chorioretinal anastomosis. The discovery of a chorioretinal anastomosis on OCTA suggests a potentially severe variant of racemose hemangioma.