Clinical features, diagnosis, management, and prognosis of circumscribed choroidal hemangioma

Abstract

Because of its benign nature and rarity, circumscribed choroidal hemangioma (CCH) often receives limited attention, leading to a high rate of misdiagnosis and a lack of standardized treatment protocols. We provide a thorough clarification of the demographics, clinical features, diagnosis, management, and prognosis of CCH. We conducted a systematic search of the PubMed, EMBASE, and Ovid databases up to December, 2023, to identify relevant studies. The study included 106 studies encompassing 3,854 patients with CCH. The demographic profile revealed a male preponderance (62%, 95% CI 61%-64%) and a peak incidence in the working-age population (30-50 years, 48%, 95% CI 39%-57%), with the right eye being involved in 50% of cases (95% CI 48%-53%). Clinically, the most common symptom was decreased vision (90%, 95% CI 78%-99%), followed by blurred vision, visual field defect, and metamorphopsia. Fundus examination frequently revealed an orange-colored tumor (80%, 95% CI 58%-96%) located subfoveally (48%, 95% CI 42%-53%), often accompanied by subretinal fluid (SRF) (84%, 95% CI 78%-89%) and sometimes exudative retinal detachment (69%, 95% CI 51%-85%). The proportion of correctly diagnosed CCH upon first presentation was 13% (95% CI 3%-26%), with CCH commonly misdiagnosed as unspecified choroidal tumors, choroidal metastasis, and central serous chorioretinopathy. Photodynamic therapy (PDT) was the most widely researched treatment, taking up 18% (95% CI 8%-31%), followed by observation, transpupillary thermotherapy (TTT), and laser photocoagulation. When no treatment was applied, 33% of patients experienced visual acuity improvement, which increased to 76% (95% CI 58%-90%) with radiotherapy and 58% (95% CI 50%-67%) with PDT. In terms of tumor shrinkage, radiotherapy was most effective (100%), with PDT close behind (95% CI 96%-100%), and TTT at 63% (95% CI 45%-80%). PDT led to SRF resolution in 89% (95% CI 77%-97%) of patients and cystoid macular edema (CME) resolution in 73% (95% CI 38%-97%), while radiotherapy achieved the highest efficacy with 100% SRF resolution (95% CI: 99%-100%) and 100% CME resolution (95% CI: 83%-100%). Complication rates were highest with radiotherapy (14%, 95% CI 5%-25%) and PDT (9%, 95% CI 4%-16%). First-line treatment failure rates were highest for TTT (71%, 95% CI 44%-92%) and laser (70%, 95% CI 28%-99%), with radiotherapy showing the lowest rate (0%, 95% CI 0%-2%). Recurrence rates were highest for laser (68%, 95% CI 17%-100%) and TTT (62%, 95% CI 26%-93%), whereas radiotherapy had the lowest recurrence rate (0%, 95% CI 0%-1%). CCH predominantly affects the working-age male population, often leading to vision impairment and SRF. The diagnosis of CCH remains challenging, with low accuracy and frequent misdiagnoses. While PDT is the most widely researched treatment, radiotherapy offers superior outcomes in visual acuity, tumor shrinkage, and resolution of SRF and CME, though it carries higher complication rates. This study highlights the need for improved diagnostic accuracy and a balanced approach to treatment.

Keywords: Circumscribed choroidal hemangioma (CCH); clinical features; diagnosis; management; meta-analysis; prognosis.