Proliferative ischemic retinopathy after arteriovenous malformation embolization in a child with hereditary hemorrhagic telangiectasia

To describe a case of hereditary hemorrhagic telangiectasia, presenting with multiple branch retinal artery occlusions, retinal ischemia, neovascularization, and vitreous hemorrhage after cerebral arteriovenous malformation embolization.

The authors report a 7-year-old patient with decreased vision in his left eye after embolization of a pineal arteriovenous malformation secondary to hereditary hemorrhagic telangiectasia. Ophthalmic evaluation, fundus photography, fluorescein angiography, spectral domain optical coherence tomography, electroretinogram, examination under anesthesia, and pars plana vitrectomy (PPV) were performed.

Fundus examination of the left eye revealed extensive posterior segment ischemia, vascular tortuosity, and vitreous hemorrhage. Fluorescein angiography was remarkable for partial obstruction of retinal arteries, midperipheral nonperfusion, and associated leakage from multiple areas of neovascularization. Spectral domain optical coherence tomography was normal. Electroretinogram demonstrated decreased b-wave amplitude. The patient was subsequently treated with 25-gauge pars plana vitrectomy, panretinal endophotocoagulation, and intravitreal bevacizumab. Five weeks after surgery, best-corrected visual acuity had improved to 20/40, and examination showed resolution of vitreous hemorrhage and neovascularization.

Retinal vascular abnormalities, posterior segment ischemia, and vitreous hemorrhage suggested a combination of retinal involvement of hereditary hemorrhagic telangiectasia complicated by nontarget embolization.