Branch retinal vein occlusion and peripheral nonperfusion associated with astrocytic hamartoma of the optic nerve head in a patient with tuberous sclerosis.

Purpose: To report a case of branch retinal vein occlusion (BRVO) causing retinal neovascularization (NV) and vitreous hemorrhage associated with astrocytic hamartoma (AH) involving the optic nerve head in a patient with tuberous sclerosis complex (TSC). We review the natural history of the clinical presentation and posit its etiology.

Methods: Electronic health records were reviewed for patient history and demographics and multimodal ophthalmic imaging including fundus photography, fluorescein angiography, and optical coherence tomography (OCT).

Results: Neovascularization emanating superiorly and inferiorly from a large AH of the optic nerve head was identified as the cause of a longstanding vitreous hemorrhage. Retinal nonperfusion was also found in superior peripheral quadrants, most notably distal to the superior NV. OCT was useful in visualizing small retinal AH in the fellow eye that was otherwise undetected on prior dilated fundus examinations. The patient was treated with intravitreal anti-VEGF and superior sectoral PRP in the involved eye.

Conclusion: BRVO and associated NV may represent rare vision-threatening sequela of AH in TSC and manifested in this case with diffuse peripheral retinal nonperfusion secondary to BRVO-induced ischemia. OCT is a valuable resource in identifying and monitoring AH of the retina.