Multimodal imaging of congenital hypertrophy of retinal pigment epithelium (chrpe) lesions at different presentations.

Abstract

Purpose: To describe the clinical presentation, fundus autofluorescence (FAF), optical coherence tomography (OCT) and OCT angiography (OCT-A) features of all three types of CHRPE: a solitary type, grouped configuration bear tracks and multifocal type with the systemic association. Methods: A comprehensive eye examination included measurement of presenting and best-corrected vision, applanation intraocular pressure (IOP), slit-lamp biomicroscopy and dilated fundus examination. The retinal imaging included fundus photography, FAF, OCT, and OCT-A. Results: The study included retinal imaging of 3 varieties of CHRPE seen in 3 patients on routine eye examination. In all patients vision was 20/20, N6 in both eyes, and IOP was 14-18 mmHg. Fundus showed bilateral, multifocal, flat, pigmented retinal lesions (Case 1), solitary, the well-circumscribed lesion (Case 2) and multiple small, flat, pigmented lesions (Case 3). FAF in all 3 variants showed hypoautofluorescence corresponding to the area of the lesion with isolated areas of is fluorescence in areas of lacunae. OCT in CFase 1 and 2 showed increased reflectance of RPE layer with outer retinal layer abnormalities whereas in Case 3 showed normal RPE layer. OCT (Case 1) also revealed thickening of RPE associated with the intraretinal extension. OCTA of outer retinal layers in Case 1 and 2 showed vascular attenuation over the CHRPE lesion with hyporeflective areas whereas in Case 3 it was normal. Conclusion: A careful clinical examination with multimodal imaging helps to differentiate a benign variant of CHRPE from its counterpart associated with systemic features.