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Hemangioma ASRS 2020

A 30-year-old young male presented with a drop in vision in the right eye for 1 year (6/60). The fundus examination revealed choroidal hemangioma superotemporal to the macula. Choroidal hemangioma is an unusual benign vascular tumor of the choroid. It can be circumscribed solitary or diffuse tumor with the later having other systemic associations. Circumscribed choroidal hemangiomas (CCHs) are usually unilateral, unifocal hamartomatous vascular tumors affecting people in the second to the fourth decade. It appears as a round to oval, orangish-red mass in the posterior pole with a smooth homogenous surface mostly present in the macular and peripapillary area. The hyperopic shift is seen in sub-foveal tumors in contrast to para-foveal ones which are usually asymptomatic or present with metamorphopsia or photopsia and diminished vision secondary to exudative retinal detachment. B-scan shows a highly reflective tumor without any shadowing or acoustic solidity with a high anterior A scan spike. EDI-OCT here depicts a smooth gently sloping choroidal mass with compressed choriocapillaris and enlarged medium and large choroidal vessels. Over a period of time, structural abnormalities of the outer retina can be visualized. Ancillary testing using Fluorescein Angiography shows lacy hyperfluorescence during the early arterial phase followed by increased hyper-fluorescence due to progressive profuse leakage from pinpoint foci during the arterial and venous phase. Indocyanine green angiography shows a lacy diffuse fluorescent tumor in the early phase followed by hypo-fluorescent tumors due to dye wash out in the late phase. Intrinsic autofluorescence is also seen in CCHs from lipofuscin and fresh sub-retinal fluid. The tumor is relatively hyperintense with respect to vitreous in T1-weighted images in iso-intense in T2-weighted images of MRI. Asymptomatic cases need no treatment, while patients showing vision loss with the presence or absence of exudative retinal detachment can be treated with photodynamic therapy which is the preferred treatment due to site-specific action. Selective occlusion of choroidal neovascularization can be achieved while the neurosensory retinal layers and Bruch membrane are almost unaffected, leaving retinal function intact. Green or rarely red wavelength laser photocoagulation is used to create a chorioretinal adhesion and resolve the SRF. Other treatment modalities include Transpupilary thermotherapy, external beam irradiation, proton beam therapy, brachytherapy, and gamma knife.

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