Orange pigment overlying a choroidal tumor
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A 55-year-old lady presented for an opinion regarding decreased visual acuity in her right eye (OD) for 6 months. On examination, best-corrected visual acuity (BCVA) was 20/100 OD and 20/20 in her left eye (OS). The fundus examination revealed a 5 × 5 mm subretinal lightly pigmented lesion under the fovea and extending inferiorly. Overlying orange pigment and subretinal fluid were clinically visualized and later confirmed by fundus photography and fundus autofluorescence (FAF).

Fundus photography OD showed the presence of orange pigment over the lesion, with moderate overlying hyper autofluorescence and marked surrounding hyper autofluorescence on FAF. Optical coherence tomography (OCT) evidenced a dome-shaped mass with the intralesional expanded vascular pattern within the choroid and overlying subretinal fluid. Ocular ultrasound (US) showed an echo dense, dome-shaped choroidal mass measuring 2.67 mm in thickness. Additional workup with indocyanine green (ICG) angiography showed early hypercyanescence of the mass in the pre-arterial phase and diffuse late staining of the lesion. A diagnosis of circumscribed choroidal hemangioma (CCH) was confirmed and PDT was advised.

Choroidal hemangioma is a benign vascular tumor of the uveal tract and can be circumscribed or diffuse. More than 90% of reported cases are found in patients in the fifth decade of life, with blurred vision being the most common symptom. The clinical characteristics of CCHs include subretinal fluid (72-81%), retinal pigment epithelial hyperplasia (33%) or metaplasia (20%), and macular edema (17-24%). The orange pigment can occur over this tumor but often is not clinically visible as it blends into the orange color of the mass. A large series on pseudomelanomas revealed that CCH is the fifth most common lesion misdiagnosed as choroidal melanoma.

Treatment options for CCHs include observation (if asymptomatic and without subretinal fluid), photodynamic therapy (PDT), plaque brachytherapy, and lens-sparing external beam radiotherapy (LS-EBRT). PDT is strongly preferred over radiation methods as this avoids long-term radiation complications. An analysis of 458 patients with choroidal hemangioma in the pre-PDT era (vs. PDT era) revealed the final visual outcome at 20/400 (pre-PDT) vs 20/63 (PDT) over a 51-year follow-up.