Hemangiomas of the choroid occur in circumscribed and diffuse forms.
A circumscribed choroidal hemangioma typically occurs sporadically (without any systemic association) (Fig 18-1). This dome-shaped, often inconspicuous vascular hamartoma is generally located posterior to the equator, often in the macular area (see Fig 18-1A, B). Initially, it may be difficult to distinguish the tumor from the surrounding fundus because it blends with the adjacent normal choroid. Eventually, degenerative changes occur in the overlying retinal pigment epithelium (RPE), making the tumor more visible on clinical examination and fundus photography. These tumors also cause cystoid degeneration in the overlying outer retinal layers. In some cases, the tumors cause a secondary exudative retinal detachment that often extends into the foveal region, resulting in blurred vision and metamorphopsia.
Figure 18-1 Circumscribed choroidal hemangioma. A, Dome-shaped tumor (inferior edge outlined by arrows) is similar in color to that of the surrounding fundus. B, Wide-angle fundus photograph in which the eye is illuminated transsclerally better highlights the reddish color of the hemangioma. C, A-scan ultrasonographic image shows characteristic high internal reflectivity (arrow).D, Optical coherence tomography (OCT) shows a dome-shaped lesion with very low signal intensity (asterisk). ILM = internal limiting membrane; RPE = retinal pigment epithelium. E, Early fluorescein angiogram (arterial phase) shows intratumoral choroidal vessels. F, In the late phase of the angiogram, the tumor is hyperfluorescent. G, H, Indocyanine green angiography demonstrates early hyperfluorescence, which is due to the choroidal location; the fluorescence increases in intensity during the angiogram. Compare parts E–H with part A (same lesion).
(Parts A, B, and E–H courtesy of Tero Kivelä, MD; part D courtesy of Robert H. Rosa Jr, MD.)
Circumscribed choroidal hemangioma may be difficult to diagnose because it can resemble other choroidal lesions, including
Diffuse choroidal hemangioma generally occurs in patients with Sturge-Weber syndrome (encephalofacial angiomatosis). In rare cases, it may occur in patients with congenital ocular melanocytosis (phakomatosis pigmentovascularis). Diffuse choroidal hemangioma produces diffuse homogenous reddish-orange coloration of the fundus, resulting in an ophthalmoscopic pattern referred to as “tomato ketchup” fundus (Fig 18-2). Secondary glaucoma and exudative retinal detachment develop in eyes with this lesion. For more information on Sturge-Weber syndrome, see BCSC Section 6, Pediatric Ophthalmology and Strabismus.
Ancillary diagnostic studies are helpful in evaluating both types of choroidal hemangiomas (circumscribed and diffuse). A-scan ultrasonography (echography) shows a high-amplitude initial echo and high-amplitude broad internal echoes (high internal reflectivity; see Fig 18-1C). B-scan ultrasonography reveals localized or diffuse choroidal thickening with prominent internal reflections but without choroidal excavation or acoustic shadowing. Optical coherence tomography (OCT) of circumscribed lesions typically shows minimal internal signal (see Fig 18-1D), a smooth surface, and tapered borders. Fluorescein angiography (FA) reveals hyperfluorescence of large choroidal vessels in the early choroidal filling phase; the fluorescence increases throughout the angiogram, with late staining of the tumor and late leakage or pooling in the cystoid spaces of the overlying retina (see Fig 18-1E, F). Indocyanine green (ICG) angiography, the preferred modality for imaging choroidal vascular lesions, demonstrates early hyperfluorescence because of the intrinsic vascularity of these lesions. This hyperfluorescence peaks at around 3–4 minutes, and there is a classic “washout” of the dye in later frames, leaving a persistently hyperfluorescent rim (see Fig 18-1G, H). If this pattern is not seen, infiltrative lesions should be considered. Infrared imaging has recently been described as a method for identifying circumscribed choroidal hemangiomas, which appear dark in these images as compared with the surrounding tissue. Often, this method provides the clearest visualization of the lesion, allowing clinicians to determine the size and borders of circumscribed choroidal hemangiomas.
Figure 18-2 Diffuse choroidal hemangioma, clinical photographs. The saturated “tomato ketchup” red-orange color of the affected left fundus (A) contrasts markedly with the color of the unaffected right fundus (B) of the same patient.
Asymptomatic choroidal hemangiomas require no treatment. The most common indication for treatment is exudative retinal detachment with subretinal fluid tracking toward the fovea and subsequent cystoid macular edema. Symptomatic circumscribed choroidal hemangiomas traditionally were managed with laser photocoagulation, which created chorioretinal adhesions. However, photocoagulation was often unsuccessful and created scars. Recurrent detachments were common.
The current treatment of choice for symptomatic circumscribed choroidal hemangioma is photodynamic therapy (PDT; see sidebar). Most choroidal hemangiomas respond to PDT, with resolution of the subretinal fluid and partial regression of the lesion, often with associated improvement in vision. However, cystoid macular edema may persist, particularly if chronic, and any degenerative changes in the overlying RPE may limit visual recovery; PDT may need to be repeated. See Chapter 9 in BCSC Section 12, Retina and Vitreous, for further discussion of choroidal hemangioma.
Low-dose radiation (via brachytherapy or external radiotherapy methods such as charged-particle, stereotactic, or external beam) has been successfully used to treat choroidal hemangiomas, including those unresponsive to PDT. All these methods can be used to treat patients with circumscribed choroidal hemangioma; for diffuse choroidal hemangioma, because of the diffuse nature of the tumor, external beam radiotherapy techniques are used rather than plaque brachytherapy. Complications from radiation include dry eye, cataract, and radiation retinopathy. These complications, as well as the exudative retinal detachment, may limit visual recovery. In addition, fibrotic changes can occur over the lesion, affecting vision.
To date, there is little published evidence to support the use of vascular endothelial growth factor (VEGF) inhibitors to treat choroidal hemangioma.
Boixadera A, García-Arumí J, Martínez-Castillo V, et al. Prospective clinical trial evaluating the efficacy of photodynamic therapy for symptomatic circumscribed choroidal hemangioma. Ophthalmology. 2009;116(1):100–105.
Papastefanou VP, Plowman PN, Reich E, et al. Analysis of long-term outcomes of radiotherapy and verteporfin photodynamic therapy for circumscribed choroidal hemangioma. Ophthalmol Retina. 2018;2(8):842–857.
Excerpted from BCSC 2020-2021 series: Section 4 - Ophthalmic Pathology and Intraocular Tumors. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.