Combined Hamartoma of the Retina and Retinal Pigment Epithelium: A Literature Review and Case Series.

We present a case series of two young patients referred to our Ophthalmology Department with a progressive decrease in visual acuity of their left eye, where multimodal imaging findings led us to the diagnosis of combined hamartoma of the retina and retinal pigment epithelium (CHR-RPE). Based on a literature review, we discuss the different multimodal imaging strategies, differential diagnosis, and management of CHR-RPE, a rare benign congenital lesion that can mimic malignant intraocular tumors. CHR-RPE has a challenging differential diagnosis with entities such as choroidal melanoma, nevus, retinoblastoma, and astrocytoma. A correct diagnosis approach is essential to avoid unnecessary invasive procedures. Multimodal imaging plays a crucial role: Optical coherence tomography (OCT) reveals epiretinal membranes (ERM) and retinal disorganization, and fluorescein angiography (FA) demonstrates vascular tortuosity and leakage in advanced disease. Management is usually conservative with strict follow-up. Surgical intervention, including vitrectomy and membrane peeling, remains controversial due to the risk of damaging dysplastic retina. Reported outcomes are variable, although selected cases showed visual improvement, particularly when combined with adjunctive enzymatic therapy.