OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IMAGING OF A CONGENITAL SIMPLE HAMARTOMA OF THE RETINAL PIGMENT EPITHELIUM.

ABSTRACT

PURPOSE: To report the case of a small pigmented tumor in the macular region investigated using optical coherence tomography angiography (OCT-A) in the context of a multimodal approach. METHODS: Case report. RESULTS: A 24-year-old man was referred for evaluation of an asymptomatic unilateral round comma-shaped pigmented tumor located in the macular region of his right eye. Spectral-domain OCT revealed an irregular hyperreflective mass within the retina, with a thickened hyaloid membrane and abrupt margins, and a complete posterior shadowing effect. On fluorescein angiography, early hyperfluorescence without leakage in the middle and late phases and a subtle halo of hyperfluorescence in the late phase were observed. OCT-A revealed the presence of intrinsic vascularization at the level of the inner capillary plexus, with signal intensity present at the level of the outer capillary plexus. In the superficial scans, the retinal vessels were oriented radially to the lesion and were in continuity with the vascular network within the tumor mass. No choroidal vessels could be identified. CONCLUSION: We report the case of a congenital simple hamartoma of the retinal pigment epithelium investigated using OCT-A as part of multimodal imaging. OCT-A provided a new insight in the assessment of the lesion, differential diagnosis, and follow-up of the patient. This imaging modality could be an important non-invasive tool in the management of congenital hamartomas of the retinal pigment epithelium.