Abstract
To describe a patient with bilateral peripapillary astrocytic hamartomas with exudation of subretinal fluid into the macula and loss of vision without evidence of choroidal neovascularization. The patient rapidly responded to intravitreal bevacizumab injections resulting in reduced subretinal fluid and clinical improvement.
A 70-year-old female presented with worsening vision in her left eye due to subretinal fluid exudation from a peripapillary astrocytic hamartoma. The patient was treated with two doses of bevacizumab with rapid improvement in vision and resolution of subretinal fluid. Genetic testing was negative for common pathogenic variants for tuberous sclerosis and neurofibromatosis, which are highly associated with bilateral optic nerve and retinal astrocytic hamartomas.
Astrocytic hamartomas with exudation may be responsive to bevacizumab suggesting a dependence of these lesions on vascular endothelial growth factor (VEGF) independent of secondary choroidal neovascularization. Furthermore, this case describes a patient with bilateral astrocytic hamartomas without genetic or clinical confirmation of associated phakomatoses, such as tuberous sclerosis and neurofibromatosis.
•Bilateral retinal astrocytic hamartomas (RAH) may become symptomatic in adulthood.•FA and OCT are important diagnostic tests when evaluating symptomatic RAH.•RAH can generate subretinal fluid independent of choroidal neovascularization.•RAH-associated subretinal fluid may respond to anti-VEGF therapy.