Peripheral combined hamartoma of the retina and retinal pigment epithelium with remote peripapillary choroidal neovascular membrane

Purpose: To describe the first reported case of combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) associated with a remote choroidal neovascular membrane (CNVM). Observations: A 19-month-old girl with a normal prenatal and infantile history presented with esotropia of the left eye. Fundus examination demonstrated a large, elevated, charcoal-colored lesion in the nasal equatorial retina. There was dragging of the nasal retinal vessels and a retinal fold, presumed to have resulted from nasal traction from the lesion. There was also subretinal hemorrhage in the peripapillary macula. Multimodal imaging demonstrated a peripapillary choroidal neovascular membrane (CNVM) underlying the retinal fold. There was no leakage within the tumor or secondary retinal neovascularization. Examination of the fellow eye was unremarkable. The patient was diagnosed with peripheral CHRRPE with associated peripapillary CNVM. She was treated with serial intravitreal bevacizumab to the affected eye which resulted in a reduction in leakage from the CNVM and resolution of the subretinal hemorrhage. The CHRRPE remained stable on follow-up. Conclusions: Peripheral CHRRPE can rarely be associated with a remote CNVM.