This case is either a combined hamartoma of the retina and RPE or an unusual macular pucker centered over the optic nerve. There is contracted fibrotic tissue overlying the optic nerve
We don’t know why a pigmented polyp-like RPE detachment developed, which was not present when we initially saw him about 5 years earlier.
Learning Points:
Combined hamartomas are classically described as benign congenital lesions composed of glial cells, vascular tissue, and pigmented retinal pigment epithelial cells. However, more recent data indicate that these lesions primarily arise from the inner retinal layer.
