Choroidal hemangioma is a rare, congenital vascular tumor of the choroid. Two main forms are described: the diffuse type, usually associated with Sturge-Weber syndrome, and the circumscribed type, which is sporadic and typically becomes symptomatic in adulthood (4^th to 6^th decade).^(1,2) Although benign, circumscribed choroidal hemangioma (CCH) may lead to progressive vision loss due to exudation, serous retinal detachment (SRD), and secondary degenerative changes of the retinal pigment epithelium (RPE) and photoreceptors.

Because of its clinical similarity with choroidal melanoma, metastasis, nevus, or osteoma, accurate diagnosis is crucial to avoid mismanagement.

Despite its benign nature, CCH presents significant diagnostic and therapeutic challenges, particularly in settings where access to first-line treatment modalities is limited.

Therefore, the objective of this case report was to highlight the importance of multimodal imaging in establishing an accurate diagnosis of CCH and to discuss the available therapeutic options. This report was approved by the Ethics Committee of the Tunis Military Hospital (CAAE number: 92/2025/CLPP).

A 41-year-old patient with no relevant medical or family history presented with progressive visual decline in the right eye. Best-corrected visual acuity (BCVA) was measured at 1/10 in the right eye and 20/20 in the left eye. Examination of the anterior segment was unremarkable in both eyes. Fundus examination revealed a circumscribed yellow-orange choroidal mass located along the inferotemporal vascular arcade, associated with an SRD extending to the macula (Figure 1A). Optical coherence tomography (OCT) demonstrated a dome-shaped choroidal mass with subretinal fluid, RPE alterations, cystoid macular edema and outer retinal damage involving the photoreceptors. (Figure 1B) Fluorescein angiography revealed early diffuse hyperfluorescence that persisted in late phases. Indocyanine green (ICG) angiography was not available. B-scan ultrasonography confirmed a homogeneous, echogenic dome-shaped choroidal mass with associated SRD. Orbital magnetic resonance imaging (MRI) revealed a lesion that was hyperintense on T1, isointense on T2, and exhibited intense homogeneous enhancement after gadolinium injection (Figure 2).

Since photodynamic therapy was not available, proton beam therapy was considered the most appropriate therapeutic alternative for our patient. Following treatment, the patient demonstrated structural improvement with a marked reduction in macular edema; however, visual acuity remained limited to 2/10, likely due to persistent outer retinal damage (Figure 3).

Circumscribed choroidal hemangioma is a rare, benign vascular tumor that typically becomes symptomatic in adulthood. Circumscribed choroidal hemangiomas are typically found at the posterior pole, most often in the superotemporal quadrant near the macula, and are characterized by sharply demarcated borders composed of compressed melanocytes and choroidal lamellae.^(3,4)

Histologically, CCH is characterized by a proliferation of thin-walled, blood-filled vascular channels within the choroid. Two principal morphologic patterns are recognized: cavernous, composed predominantly of large dilated vascular spaces separated by delicate stromal septa, and capillary, consisting of smaller, capillary-sized vessels embedded in a loosely organized connective tissue matrix. Most lesions exhibit a mixed pattern, demonstrating variable proportions of both vascular types. The endothelial lining is typically flat and inconspicuous, without evidence of cytologic atypia, mitotic activity, or significant perivascular smooth muscle proliferation, supporting the non-neoplastic nature of the lesion.⁽⁵⁾

Although non-malignant, it may lead to significant visual loss through chronic subretinal fluid, SRD, and secondary degenerative changes such as RPE hyperplasia, subretinal fibrosis, retinoschisis, and photoreceptor degeneration.⁽⁶⁾

On fundus examination, the lesion typically appears as a sharply demarcated, red-orange mass, sometimes bordered by subtle basal pigmentation. It is most commonly identified at the posterior pole, representing roughly two-thirds of reported cases.⁽⁷⁾ A predilection for the superotemporal juxtamacular region has been noted.⁽⁴⁾ CCHs are almost always solitary and affect only one eye; bilateral involvement remains uncommon, although a few exceptions have been described in the literature.^(8,9)

Differentiating CCH from other choroidal lesions is crucial, particularly from amelanotic choroidal melanoma, which usually presents as a pigmented dome- or mushroom-shaped mass with low-to-medium echogenicity and compression of the choriocapillaris on OCT.⁽¹⁰⁾ Other differential diagnoses include choroidal metastases, which are often multifocal and yellowish; choroidal nevus, generally flat with drusen on its surface; and choroidal osteoma, a calcified whitish lesion with high reflectivity and posterior acoustic shadowing on ultrasound.^(4,11)

Management depends on the presence of symptoms and lesion characteristics. Observation is recommended for asymptomatic cases, while treatment is indicated in patients with visual decline or persistent subretinal fluid.⁽¹⁾

Photodynamic therapy with verteporfin is considered the first-line treatment. Photodynamic therapy may induce regression or atrophy of the vascular channels within the hemangioma while sparing the neurosensory retina, leading to reduced vascular leakage and subsequent improvement or stabilization of vision.⁽¹²⁾

Transpupillary thermotherapy may be used as an alternative but is primarily indicated for extrafoveal, post-equatorial circumscribed choroidal hemangiomas characterized by mild subretinal fluid, with a basal diameter under 10 mm and a thickness below 4 mm.⁽¹³⁾

Transpupillary thermotherapy can result in complete or partial tumor regression in approximately half of the treated cases, although it carries an increased risk of fibrosis and choroidal atrophy.⁽¹⁴⁾

Radiotherapy, particularly proton beam therapy, represents a valuable and minimally invasive treatment option for larger tumors (> 3 mm) or extensive SRDs, offering good functional outcomes in a single session.⁽¹⁵⁾

Adjuvant therapies such as intravitreal anti-vascular endothelial growth factor (VEGF) or dexamethasone implants may reduce macular edema by reducing vascular wall permeability and enhancing the efficacy of photodynamic therapy.⁽⁷⁾ Enucleation is reserved for painful blind eyes with neovascular glaucoma.

Circumscribed choroidal hemangioma is a rare but clinically significant benign vascular tumor whose macular complications may lead to severe and irreversible visual impairment. Accurate diagnosis is essential, as its clinical and imaging characteristics may mimic other amelanotic choroidal tumors with major therapeutic implications. Ultimately, early recognition and appropriate selection of the therapeutic modality are key to optimizing visual outcomes in patients with CCH.