Optical Coherence Tomography Angiography Imaging of a Congenital Simple Hamartoma of the Retinal Pigment Epithelium

Abstract

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.

Retinal sensitivity and photoreceptor arrangement changes secondary to congenital simple hamartoma of retinal pigment epithelium

“…In agreement to Gass [3] description of intrinsic vasculature as an element to characterize CSHRPE, the present report also demonstrated an exclusive vascular circuit distinguishing from other OCTA reports [6, 7], as well as an evident patent feeder vessel (Figs. 2, 3) as published by Shields and colleagues [1] in 100% of their cases.…”

“…Recently, Arjmand et al [6] and Zola et al [7] reported a case imaged with optical coherence tomography angiography (OCT-A) for noninvasive assessment of the intrinsic retinal microvasculature. Similar to them, we explored OCT-A of this mass and found better microvasculature detail than with fluorescein angiography (FA).…”

Macular Edema in a Peculiar Case of Pigmentary Maculopathy

“…3 Several studies have described hypoautofluorescence of the lesions on FAF images and hypofluorescence in early-phase fluorescein angiography (FA) with/ without discrete hyperfluorescence around the lesion in the late phase of the angiogram. 3,[5][6][7] Fluorescein angiography may reveal feeder vessels related to the lesion. Similar to other studies, [5][6][7][8][9] the current case had intrinsic flow to the pigmented lesions at the SCP, seen bilaterally on OCTA and more evident in the left eye (Figures 6 and 7).…”

Congenital simple hamartoma of the retinal pigment epithelium with depigmentation at the margin in an Indian female

“…The fluorescein angiogram shows hypofluorescence of the black lesion and hyperfluorescence at the depigmentation within the lesion [ 7 ] or at the margin with or without a hyperfluorescent halo [ 7 ]. Depigmentation within the lesion may correspond to the intrinsic vasculature of the hamartoma [ 8 ].…”