Introduction

Choroidal hemangiomas are benign vascular hamartomas which occur in two forms: circumscribed and diffuse. The circumscribed form is typically an isolated finding without systemic associations, while the diffuse form generally occurs in association with Sturge–Weber syndrome (discussed separately in Chapter 132, Phakomatoses). There have been rare reports of circumscribed choroidal hemangioma in patients with Sturge–Weber syndrome.^1,2 The first histologically confirmed case of choroidal hemangioma was published by Leber in 1868.³ The incidence of the disease is difficult to estimate since most circumscribed choroidal hemangiomas only come to medical attention if patients become symptomatic or if they are discovered incidentally during routine examination; however, the disease is felt to be relatively rare, with five cases discovered upon histological examination of 4500 enucleated eyes.⁴ More than 90% of reported cases have been in Caucasian patients (though there have been published cases in black, Hispanic, and Asian patients), with a relatively even distribution between males and females.^5,6 The mean age at diagnosis in the two largest case series ranged from 38.7 years to 47 years, considerably older than the mean age at diagnosis for diffuse choroidal hemangioma, which is typically in the first decade of life.^5,6

Clinical features

Circumscribed choroidal hemangiomas are generally orange-red elevated masses, which are found posterior to the equator (Figs 153.1–153.4). The color of the tumor has also been variably described as “salmon-colored”,⁷ “yellow-white”,⁸ and “grayish-pink”.⁶ Choroidal elevation may be difficult to discern on color photographs, and may be more apparent on clinical examination. Pigmentary changes have also been reported on the surface of the tumor or as a ring of pigmentation around the edge of the tumor.⁶ There may be accumulation of lipofuscin pigment (orange pigment) over the lesion, though this is often difficult to distinguish except with fluorescein angiography.⁹ In the largest published series of 200 patients by Shields et al., 67% of tumors were in the macula, 34% were between the macula and the equator, and no tumors were anterior to the equator.⁵ In the second-largest series of 45 patients with circumscribed choroidal hemangioma reported by Witschel and Font, all of the circumscribed tumors were located posterior to the equator.⁶ In the Shields series, the mean tumor diameter was 6.7 mm, and the mean tumor thickness was 3.1 mm.⁵ Lesions are usually solitary and unilateral, though bilateral choroidal hemangiomas have been reported.⁷ Overlying subretinal fluid or serous retinal detachment is a common finding, present in 47% of patients in the Witschel series,⁶ and 81% of the patients in the Shields series (Fig. 153.3).⁵ Cystoid macular edema is also a common finding, present in up to 17% of patients, and exudates, epiretinal membrane, and retinal hemorrhages have also been reported.⁵ Exudates are uncommon in melanocytic lesions. Choroidal neovascularization is rare but has been reported in four patients.^5,10 Retinal neovascularization has also been reported in three eyes with circumscribed choroidal hemangioma.¹¹ Patients with total retinal detachments can also develop neovascularization of the iris and angle and neovascular glaucoma.⁵

Fig. 153.1 (A) An orange-red choroidal hemangioma with overlying changes in the retinal pigment epithelium at the superior aspect of the optic disc. The visual acuity in this eye was reduced to 20/50 (6/15) owing to subretinal fluid that extended from the tumor to the fovea. (B–D) Intravenous fluorescein angiograms showing fluorescence in this mass before filling of the retinal arteries. There is leakage of dye over the tumor.

Fig. 153.2 (A) Circumscribed choroidal hemangioma 3.2 mm in height. (B) Indocyanine green angiography taken during induced intraocular hypertension showing that the filling of the hemangioma originates from two short posterior ciliary arteries. The arrow indicates what is probably an arterial anastomosis inside the tumor. (C) ICGA during normal intraocular pressure showing complete filling of the tumor and a sector of reduced choroidal perfusion (arrows). (D) The late ICGA photograph showing clearing of dye from the tumor and diffusion into the choroid and subretinal space (“washout” phenomenon).

(Reproduced with permission from Piccolino FC, Borgia L, Zinicola E. Indocyanine green angiography of circumscribed choroidal hemangiomas. Retina 1996;16:19–28.)

Fig. 153.3 (A) Orange-red choroidal hemangioma with overlying retinal pigment epithelium changes and accompanying serous retinal detachment. (B) Spectral domain optical coherence tomography (SD-OCT) showing choroidal elevation and subretinal fluid. (C) FA showing characteristic early hyperfluorescence in arterial phase. (D) Color fundus photography after photodynamic therapy. (E) SD-OCT after photodynamic therapy showing resolution of subretinal fluid and tumor regression.

Fig. 153.4 (A) A red-orange choroidal hemangioma superior to the optic disc with a serous detachment into the macula. The tumor thickness is 2.8 mm. (B) Intravenous fluorescein angiography of this tumor shows patchy hyperfluorescence at the time of arterial flow. (C) Late patchy staining of the tumor with some dye leakage into the subretinal space. (D) Ultrasonography of this tumor demonstrates a solid dome-shaped lesion on B-scan and high internal reflectivity with A-scan echography. (E) Sketch of this tumor with subretinal fluid extending into the macula. (F) Overlapping circles show the distribution of the laser applications following infusion of verteporfin. (G) Appearance of this tumor 3 months following photodynamic therapy. The tumor is now flat with some rarefaction of the retinal pigment epithelium and subtle evidence of subretinal fibrosis. (H) Ultrasonographic view of the tumor 3 months after treatment shows no measurable tumor height.

(Reproduced with permission from Robertson DM. Photodynamic therapy for choroidal hemangioma associated with serous retinal detachment. Arch Ophthalmol 2002;120:1155–61. © 2002 American Medical Association. All rights reserved.)

These lesions usually remain stationary in size, though there have been reports of gradual progressive enlargement.^12,13 In five cases, there was only slight enlargement with a mean of 1.6 × 1.5 mm in basal diameter and 0.9 mm in thickness over an average of 52 months of follow-up.¹² In another case with significant enlargement, the eye was enucleated due to concern for choroidal melanoma and pathologic examination revealed that the increase in size was most likely due to vascular congestion of the tumor vessels, and possibly an increase in the caliber and number of tumor vessels.¹³ There has also been one report of “blackening” of a circumscribed choroidal hemangioma from red-orange to dark gray after tantalum clip placement in preparation for external proton beam irradiation.¹⁴ The hemangioma subsequently returned to its original red-orange color 2 weeks after surgery (prior to proton beam irradiation), leading the authors to hypothesize that the transient color change may have been due to extravascular hemorrhage from surgical manipulation.¹⁴ Bosch and Helbig also reported blackening of a choroidal hemangioma after photodynamic therapy.¹⁵

The most commonly reported symptom is blurred vision in up to 81% of patients, though patients also note visual field deficits, metamorphopsia, and floaters.⁵ Presenting visual acuity ranged from 20/20–20/40 in 24% of eyes to 20/400 or worse in 34% of eyes in the Shields series, and 60% of eyes were “blind” in the Witschel series.^5,6

Differential diagnosis

The differential diagnosis of circumscribed choroidal hemangioma includes choroidal nevus, amelanotic choroidal melanoma, choroidal metastasis, choroidal osteoma, and central serous chorioretinopathy. On clinical examination, choroidal hemangiomas have a characteristic orange-red color, unlike choroidal metastases which tend to be creamy-yellow and amelanotic melanomas which tend to be more yellow-tan.⁵ Ancillary testing is very helpful to distinguish these conditions. Ultrasonography shows high internal reflectivity due to the vascular component of the lesion, in contrast to choroidal melanomas which display low to medium internal reflectivity. Circumscribed choroidal hemangiomas also have a very distinct appearance on indocyanine green angiography with rapid filling and “washout” phenomenon, unlike choroidal melanoma and metastasis which have slower and less intense filling.⁵ On MRI, circumscribed choroidal hemangiomas show bright signal on both T1- and T2-weighted images, whereas choroidal melanomas and metastases show bright signal on T1-weighted images but low signal on T2-weighted images.⁵

Ancillary studies

Pathology

Choroidal hemangiomas are hamartomas, benign vascular tumors which are composed of tissue elements normally found in a given location. Hemangiomas of the choroid are classified histopathologically according to the prevailing type or types of vessels within the tumor: capillary, cavernous, or mixed.⁶ The capillary type is composed of small vessels separated by loose connective tissue (Fig. 153.5), whereas the cavernous type is composed of larger vessels separated by relatively little connective tissue (Fig. 153.6). The mixed type shows both capillary and cavernous features. Choroidal hemangiomas are notable for the lack of cellular proliferation of the elements of the vessel walls, supporting the idea that these tumors are nonproliferative lesions.⁶

Fig. 153.5 Capillary type of choroidal hemangioma showing uniformly small vessels separated by a loose, edematous, intravascular stroma. The retina (upper right) is detached and cut obliquely through the plane of the nerve fiver (H&E; ×50).

(Reproduced with permission from AFIP and Witschel H, Font RL. Hemangioma of the choroid. A clinicopathologic study of 71 cases and a review of the literature. Surv Ophthalmol 1976;20:415–31.)

Fig. 153.6 Cavernous type of choroidal hemangioma with early cystoid changes of the outer plexiform and inner nuclear layers. The dilated, blood-filled spaces are lined with a flat layer of endothelial cells with minimal intervascular stroma. Proliferation of retinal pigment epithelial cells is present (H&E; ×50).

(Reproduced with permission from AFIP and Witschel H, Font, RL. Hemangioma of the choroid. A clinicopathologic study of 71 cases and a review of the literature. Surv Ophthalmol 1976;20:415–31.)

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