IVTA can substantially improve vision in some patients, but most patients have stable visual acuity compared with baseline at 1 year despite repeated injections.
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.
The development of secondary choroidal neovascularization in Best's disease is rare in the pediatric population. A retrospective review of pediatric patients with choroidal neovascularization secondary to Best's disease was performed. The patients' courses and treatments were recorded. Three patients with choroidal neovascularization were identified. All had decreased vision and were treated in an individual manner. Vision improved after treatment in all patients. Treatment may hasten resolution of choroidal neovascularization and improve vision.
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