Author: Meriem Ouederni (Tunisia)
Co-authors: Fehmy Nefaa, Olfa Beizig, Rym Maamouri, Zied Chelly, Monia Cheour
Purpose
Combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) is a rare benign tumor that can cause significant visual loss. It is considered to be of congenital origin. The purpose of our study was to analyze multimodal imaging features in two eyes with late onset CHRRPE.
Setting/Venue
Department of ophthalmology, Habib Thameur Hospital, Tunis (Tunisia)
Methods
Two eyes of 2 patients diagnosed with unilateral late onset CHRRPE were included. Complete ophthalmic examination and multimodal imaging including fundus images, fluorescein angiography (FA), Swept-source optical coherence tomography (SS-OCT), and swept-source OCT angiography (OCT-A) were performed.
Results
• Case1 A 71-year-old man presented with decreased vision in his left eye. BCVA was limited to 10/200. Anterior segment examination was unremarkable. Dilated fundus examination revealed a fibrotic mass with tortuous vasculature, located in the posterior pole. FA showed dilated telangiectatic capillaries with multiple punctate hyperfluorescent spots, and late leakage in the posterior pole. SS-OCT showed full thickness retinal folds with distortions and omega-shaped disorganization of the inner layers. The ellipsoid zone and the retinal pigment epithelium appeared intact. OCT-A angiograms showed abnormal vessels within the lesion, with vascular traction and multiple punctiform hypersignals in the deep capillary complex. • Case2 A 68-year-old female presented with progressive visual loss.BCVA was 20/60 in the right eye and 20/200 in the left eye, slit lamp examination showed bilateral cataract. Fundus examination of the left eye showed an elevated macular lesion, slightly pigmented, with vascular tortuosity and epiretinal membrane. FA revealed areas of pinpoint hyperfluorescence with mild late leakage. SS-OCT showed a multilayer epiretinal membrane, inner retinal disorganization limited by outer plexiform layer (omega sign), with preserved outer retinal layers. OCT-A noticed increased tortuosity of the superficial vessels, capillary rarefaction in both plexuses with multiple punctiform hypersignals in the deep capillary complex.
Conlusions
CHRRPE is a congenital tumor often revealed in childhood. In case of late diagnosis, multimodal imaging can help to differentiate simple CHRRPE from similar RPE lesions and complex epiretinal membranes. OCT and OCTA features allow non-invasive diagnosis and follow up.
Financial Disclosure
No finanacial disclosure
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