Congenital hypertrophy of the retinal pigment epithelium associated with acquired retinoschisis and microcystic degeneration: The importance of multimodal imaging.

We present an illustrated clinical case with multimodal representation of a congenital hypertrophy of the depigmented retinal pigment epithelium (CHRPE) associated with acquired retinoschisis and microcystic degeneration (MD) in a 64-year-old hypermetropic, asymptomatic woman. Retinography showed a whitish lesion with pigmented edges, slightly raised, accompanied by a lower area with a microcystic pattern. The complementary tests carried out allowed to determine a well-defined diagnosis of exclusion. B-mode ultrasound showed a smooth, cupuliform and cystic elevation characteristic of acquired retinoschisis, and fluorescein angiography could rule out the presence of a vascular tumor. The hypoautofluorescence of the lesion determined the depigmentation of the CHRPE, while the slight perilesional hyperautofluorescence corresponded to the associated retinoschisis and DM. This is the first case described in the literature of an acquired retinoschisis associated with CHRPE.