Multimodal imaging characteristics of combined hamartoma of the retina and retinal pigment epithelium

Abstract

Objective To observe the multimodal imaging characteristics of combined hamatoma of the retina and retinal pigment epithelium (CHRRPE). Methods A retrospective case study. From January 2013 to December 2017, 6 CHRRPE patients (6 eyes) diagnosed in Department of Ophthalmology, Sun Yat-sen Memorial Hospital were included in the study. There were 4 males and 2 females, with the mean age of 12.0±8.10 years. There were 5 eyes with BCVA ≤0.1, 1 eye with BCVA>1.0. Corneal fluoroscopy showed 1 eye with an external oblique 15°, and the remaining eye had no abnormalities in the anterior segment. All eyes underwent fundus color photography, FAF, FFA, ICGA, OCT and color Doppler flow imaging (CDFI). The multimodal imaging characteristics were observed. Results All the affected eyes CHRRPE were located in the posterior pole and showed mild elevation. Most of the retinal neuroepithelial layers had different degrees of hyperplasia, vascular tortuosity and retinal folds. Of the 6 eyes, 4 eyes (66.7%) involving the macula and optic disc, only 2 eyes (33.3%) involving the macula. OCT showed that the structure of the neuroepithelial layer was unclear and the signal intensity was uneven; it involved 2 eyes of the whole retina (33.3%) and only 4 eyes of the neuroepithelial layer (66.7%). FFA and ICGA showed that the choroidal background fluorescence of the early lesions was weakened, and the lesions showed slightly weak fluorescence; the late telangiectasia fluorescein was obviously leaked, and the lesions were stained with fluorescence. FAF mainly appears as weak autofluorescence with a small amount of strong autofluorescence. CDFI has no characteristic performance. Conclusions CHRRPE is mainly a membrane-like hyperplasia without angiogenesis, involving the retinal neuroepithelial layer, and may also involve the entire retina. OCT is dominated by strong reflection; AF, FFA and ICGA are mainly weak fluorescence. Key words: Retinal diseases; Hamartoma/diagnosis; Multimodal imaging characteristics