Circumscribed choroidal hemangioma

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Page 1: Circumscribed choroidal hemangioma

Circumscribed choroidal hemangioma

Arman Mashayekhi, MD, and Carol L. Shields, MD

Circumscribed choroidal hemangioma is an uncommon,benign vascular tumor manifesting as an orange-red mass inthe posterior pole of the eye. Serous retinal detachmentaccounts for decreased vision in most patients. Diagnosis ofthis tumor is challenging with many patients initiallymisdiagnosed with choroidal melanoma or metastasis. Severalancillary tests such as ultrasonography, fluoresceinangiography, indocyanine green angiography, and magneticresonance imaging help differentiate this tumor from othersimulating lesions. Asymptomatic lesions should be observed,but visually threatening or visually impairing lesions requiretreatment. Photodynamic therapy, laser photocoagulation, andtranspupillary thermotherapy may be used for primarymanagement of this tumor. Patients who fail to respond toprevious treatment or those with extensive serous retinaldetachment can be treated using radiotherapeutic modalities.Long interval between onset of symptoms and treatment, poorvisual acuity at presentation, and presence of chronic retinal orretinal pigment epithelial changes are associated with poorlong-term vision. Curr Opin in Ophthalmol 2003, 14:142–149 © 2003

Lippincott Williams & Wilkins.

BackgroundChoroidal hemangioma is a benign, vascular, hamartoma-

tous tumor occurring in two distinct forms, circumscribed

and diffuse, on the basis of the extent of choroidal in-

volvement. Circumscribed choroidal hemangioma is gen-

erally a solitary finding without systemic associations,

whereas diffuse choroidal hemangioma usually occurs in

association with encephalofacial angiomatosis (Sturge-

Weber syndrome) [1].

The diagnosis and management of circumscribed choroi-

dal hemangioma continue to be a challenge for most

clinicians. Many patients with choroidal hemangioma are

referred to us because of suspected choroidal melanoma

or choroidal metastasis. Therefore, it is important to

achieve an accurate diagnosis of this tumor, reassure pa-

tients of its benign nature, and provide proper therapy.

DiagnosisClinical manifestations

Circumscribed choroidal hemangioma is a relatively rare

tumor. Between 1974 and 2000, 200 patients with cir-

cumscribed choroidal hemangioma were diagnosed and

treated by the Oncology Service at Wills Eye Hospital,

whereas during the same period, more than 10,000 pa-

tients with choroidal melanoma were seen at that center.

Although probably congenital, most patients do not de-

velop symptoms until they are in their fourth to sixth

decade of life. In the series reported from the Oncology

Service at Wills Eye Hospital, the mean age at presen-

tation was 47 years with a range of 4 to 81 years [2••].

Most patients present with decreased visual acuity.

Other less common symptoms include visual field de-

fect, metamorphopsia, flashes and floaters, and progres-

sive hypermetropia [2••].

Circumscribed choroidal hemangioma usually appears as

a discrete, round, orange-red tumor, similar in appear-

ance to the adjacent surrounding choroid (Fig. 1). A pig-

mented rim, possibly secondary to compression of adja-

cent choroid or elevation of the retinal pigment

epithelium (RPE), often surrounds the tumor. Almost all

cases occur posterior to the equator, usually near and

temporal to the optic disc [3]. Clumps of pigment, prob-

ably secondary to hyperplasia of overlying RPE, are not

uncommon [2••]. Retinal or subretinal exudation is not a

common feature of choroidal hemangiomas and was re-

ported in only 7% of patients in one series [4]. Mushroom

appearance is exceedingly rare [2••].

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University,Philadelphia, Pennsylvania, USA.

Supported by the Eye Tumor Research Foundation, Philadelphia, PA (C.L.S.), theMacula Foundation, New York, NY (C.L.S.), and the Rosenthal Award of theMacula Society (C.L.S.).

Correspondence to Carol L. Shields, MD, Ocular Oncology Service, Wills EyeHospital, 840 Walnut Street, Philadelphia, PA 19107, USA

Current Opinion in Ophthalmology 2003, 14:142–149

Abbreviations

PDT photodynamic therapyRPE retinal pigment epithelium

ISSN 1040–8738 © 2003 Lippincott Williams & Wilkins

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This benign tumor can cause visual impairment by vari-

ous mechanisms, such as exudative retinal detachment,

overlying photoreceptor degeneration, elevation or tilt-

ing of the macular region, cystoid macular edema, sub-

retinal fibrosis, RPE alterations, and retinoschisis [1,2••].

Differential diagnosis

The diagnosis of circumscribed choroidal hemangioma

can be challenging. Of the 200 patients in the series from

the Oncology Service at Wills Eye Hospital, the clinical

diagnosis of choroidal hemangioma was accurately sus-

pected before referral for only 29% of the patients, and

14% were referred without any specific diagnosis (Table

1) [2••]. Several reasons may account for this situation:

(1) The ophthalmoscopic appearance of choroidal hem-

angioma may be almost indistinguishable from the

normal adjacent choroid. Failure to detect the tu-

mor may account for referral diagnoses such as ret-

robulbar optic neuritis, high hypermetropia, or age-

related macular degeneration.

(2) The presence of an overlying exudative retinal de-

tachment can obscure the underlying choroidal de-

tails and may make the tumor even more obscure.

This situation can lead to an erroneous diagnosis of

retinal detachment, central serous chorioretinopa-

thy, or macular edema.

(3) The funduscopic appearance of circumscribed cho-

roidal hemangioma may simulate other benign or

malignant conditions of the fundus. In the series

from Wills Eye Hospital, 38% of patients were re-

ferred with the diagnosis of an intraocular malig-

nancy, most commonly amelanotic choroidal mela-

noma or choroidal metastasis (Table 1) [2••].

Several ophthalmoscopic features may help differentiate

choroidal hemangiomas from simulating lesions. Choroi-

dal hemangiomas have a distinctive orange-red color

similar to the surrounding choroid, whereas amelanotic

melanomas are a more yellow-tan color, often with subtle

intrinsic pigment and visible overlying drusen. Clinically

evident drusen are rare overlying choroidal hemangioma

and were detected in only 2% of patients in one series

[2••]. In contrast to choroidal melanomas, choroidal

hemangiomas almost never attain a mushroom-shaped

appearance [2••]. Choroidal metastasis appears as a

creamy yellow plateau or elevated mass and in contrast to

choroidal hemangioma, which is almost always solitary

and unilateral, may commonly be multifocal or bilateral.

However, three specific choroidal metastases—from

renal cell carcinoma, thyroid carcinoma, and carcinoid

Figure 1. Clinical appearance of circumscribed choroidal

hemangioma

(A) Wide-angle fundus photograph of circumscribed choroidal hemangioma withassociated subretinal fluid. (B) Wide-angle fundus photograph of circumscribedchoroidal hemangioma with minimal associated subretinal fluid and overlyingretinal pigment epithelial alterations.

Table 1. Referral diagnosis in 200 consecutive patients with

circumscribed choroidal hemangioma referred to the Oncology

Service, Wills Eye Hospital [2••]

Referral diagnosis Number (%)

Choroidal hemangioma 58 (29)Choroidal melanoma 58 (29)Choroidal metastasis 17 (9)Retinal detachment 12 (6)Central serous chorioretinopathy 9 (5)Macular edema 5 (3)Others 13 (9)No diagnosis 28 (14)

Adapted from: Shields CL, Honavar SG, Shields JA, Cater J, DemirciH. Circumscribed choroidal hemangioma: clinical manifestations andfactors predictive of visual outcome in 200 consecutive cases.Ophthalmology 2001; 108:2237–48.

Circumscribed choroidal hemangioma Mashayekhi and Shields 143

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tumor—can appear orange, similar to choroidal heman-

gioma [1,5].

It is generally believed that circumscribed choroidal

hemangioma is not associated with Sturge-Weber syn-

drome, but in the Wills Eye Hospital series, there were

four patients with typical circumscribed choroidal hem-

angioma who had a facial nevus flammeus and other

manifestations of Sturge-Weber syndrome [2••]. The

findings in the choroid may have been a limited form of

this syndrome because others have also observed this

association [6,7,8•]. Five additional patients showed sys-

temic mucosal or remote cutaneous hemangiomas, and

one patient had neurofibromatosis [2••].

Natural course

Most cases of circumscribed choroidal hemangioma are

stationary, but several authors have reported spontane-

ous enlargement of this lesion [9,10]. Enlargement of

choroidal hemangioma is secondary to venous congestion

rather than cellular multiplication [10]. We are aware of

three reports of circumscribed choroidal hemangiomas

presenting with exudative retinal detachment and de-

creased vision during the second or third trimesters of

pregnancy [8•,11,12]. Spontaneous reabsorption of sub-

retinal fluid can occur in some patients following deliv-

ery [8•,11].

Pathology

In 1976, Witschel and Font [3] described the clinicopath-

ologic features of 45 eyes with circumscribed choroidal

hemangioma that came to enucleation and were evalu-

ated at the Armed Forces Institute of Pathology. They

described solitary hemangioma as a circumscribed tumor

with a sharply demarcated margin, causing compression

of adjacent choroidal melanocytes and lamellae. No pro-

liferation of endothelial cells was observed in these tu-

mors. Alterations of overlying RPE were common, rang-

ing from atrophy and local proliferation to severe fibrous

transformation or ossification. Almost all lesions showed

degenerative changes of the overlying retina, including

loss of photoreceptors, cystoid degeneration, gliosis, and

invasion by RPE cells.

Ancillary studies

Choroidal hemangioma shows characteristic features on

ultrasonography, fluorescein angiography, indocyanine

green angiography, and magnetic resonance imaging

(Table 2). With ultrasonography, the hemangioma is

acoustically solid on B-scan, and the echogenic character

is generally similar to that of the surrounding choroid

(Fig. 2A). On A-scan ultrasonography, high internal re-

flectivity is characteristic (Fig. 2B). Intrinsic vascular pul-

sation is generally not a feature of this tumor [2••,13].

These features would be unlikely with choroidal mela-

noma because melanoma usually displays acoustic hol-

lowness, low to medium internal reflectivity, and intrin-

sic vascular pulsations; however, similar ultrasonographic

features may be seen with choroidal metastasis [1].

Fluorescein angiography of choroidal hemangioma typi-

cally shows lacy hyperfluorescence in the prearterial or

early arterial phase and diffuse, intense, late hyperfluo-

rescence (Fig. 3). Fluorescein angiographic findings,

Figure 2. Ultrasonographic appearance of circumscribed

choroidal hemangioma

(A) A-scan ultrasound shows the characteristic high internal reflectivity ofcircumscribed choroidal hemangioma. (B) On B-scan ultrasonography,circumscribed choroidal hemangioma has a solid acoustic appearance similar tothe adjacent choroid.

Table 2. Characteristic features of circumscribed choroidal

hemangioma on ancillary testing

UltrasonographyA-scan: High internal reflectivityB-scan: Acoustically solid (similar to normal choroid)

Fluorescein angiographyEarly: Mild, lacy hyperfluorescenceLate: Intense, diffuse hyperfluorescence

Indocyanine green angiographyEarly: HyperfluorescenceLate: Dye “washout”

MRIBright signal on both T1- and T2-weighted images

144 Retina and vitreous disorders

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although characteristic, are not pathognomonic of this

tumor [2••]. Indocyanine green angiography demon-

strates an early well-defined area of intense hyperfluo-

rescence, often followed by a characteristic “dye wash-

out” in late frames (Fig. 4) [14,15]. These features would

be unlikely with choroidal melanoma or metastasis, for

which filling on fluorescein angiography and indocyanine

green angiography is slower and less intense. With mag-

netic resonance imaging, choroidal hemangioma shows

bright signal on T1- and T2-weighted images (Fig. 5)

unlike choroidal melanoma and metastasis, which gen-

erally show bright signal on T1-weighted and low signal

on T2-weighted images (Table 2) [16].

Optical coherence tomography is a useful method for

detection of minimal amounts of subretinal fluid and

other retinal changes associated with circumscribed cho-

roidal hemangioma (Fig. 6). Marked attenuation of the

light signal occurs after passing through the neurosensory

retina and RPE, limiting the usefulness of this technique

for direct evaluation of choroidal tumors.

TreatmentThe goal for management of choroidal hemangioma is

preservation or improvement of visual acuity by stimu-

lating absorption of subretinal fluid and resolution of

macular edema before irreversible retinal or RPE alter-

ations have occurred. Re-treatment of residual extrafo-

veal tumors without subretinal fluid is not necessary. It is

not yet clear whether incomplete flattening of hemangi-

omas predisposes to later recurrence of subretinal fluid.

A secondary goal, in more advanced cases, is prevention

of neovascular glaucoma from long-standing, extensive,

secondary retinal detachment [11].

Long delay between onset of symptoms and onset of

treatment may be associated with a worse visual outcome

[2••,17,18]. In the Wills Eye Hospital series, poor final

visual acuity (�20/200) was more common in patients

treated after 6 months of symptoms (72%) compared

with those treated within 6 months of symptoms (42%)

[2••]. In the same study, initial visual acuity was found

to be a good predictor of visual outcome [2••], and pa-

tients with poor visual acuity at presentation, especially

if associated with chronic retinal and RPE changes,

should be warned of long-term poor visual acuity. Loca-

tion of the hemangioma relative to fovea has also been

identified as an important predictor of final visual acuity.

In a smaller series reported from Wills Eye Hospital,

poor visual acuity of 20/200 was obtained in 69% of pa-

tients with subfoveal hemangioma, in 47% of those with

parafoveal tumors, and in 38% of patients with an extra-

foveal tumor [4].

Management of choroidal hemangioma is based on tu-

mor size, location, and related ocular symptoms. Asymp-

tomatic circumscribed choroidal hemangiomas only need

to be observed, and treatment is generally reserved for

patients with vision-threatening or vision-impairing le-

sions. In the series reported from the Oncology Service at

Wills Eye Hospital, 43% of patients were treated with

observation alone [2••]. These patients generally

showed minimal findings with little or no subretinal fluid

or fluid-related visual disturbances, or, conversely, they

showed advanced findings with chronic macular edema

so great that treatment would have been of little visual

benefit. In addition, observation with refraction is advis-

able for patients who have hyperopic amblyopia second-

ary to subfoveal tumors.

Initially xenon arc photocoagulation and later argon laser

photocoagulation were the most important treatment

modalities [2••,4,19]. More recently, transpupillary

Figure 3. Fluorescein angiographic appearance of

circumscribed choroidal hemangioma.

(A) Lacy appearance of choroidal hemangioma in arterial phase. (B) Late, diffusehyperfluorescence of choroidal hemangioma.

Circumscribed choroidal hemangioma Mashayekhi and Shields 145

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thermotherapy [17,20–23], plaque radiotherapy [24–26],

external beam radiotherapy [2••,18,25,27], proton beam

radiotherapy [28–30], and photodynamic therapy (PDT)

[33,34••,35–37] have been introduced (Table 3).

Laser photocoagulation has been used extensively for

management of circumscribed choroidal hemangiomas

[2••,4,19]. While success rates as high as 100% have

been reported [4], others have reported recurrent sub-

retinal fluid in more than 50% of treated patients [19]. In

the series reported from Wills Eye Hospital, argon laser

photocoagulation was successful in completely resolving

subretinal fluid in 62% of patients [2••]. In general, if

the subretinal fluid does not respond appropriately to

one or two sessions of surface and delimiting argon laser

photocoagulation, other treatment modalities should

be employed.

In several small series, transpupillary thermotherapy has

been reported to cause resolution of choroidal hemangi-

oma-related subretinal fluid, both as a primary treatment

and following failed prior laser photocoagulation [17,20–

23]. This method can occasionally be visually destructive

and cause further visual field and acuity loss; therefore, it

should not be used for management of subfoveal hem-

angiomas. Recently, Fuchs et al. [17] have reported their

results of treatment of 10 patients with circumscribed

choroidal hemangioma using transpupillary thermo-

therapy. Subretinal fluid persisted in three eyes because

the hemangioma could not be treated completely due to

proximity to the fovea. Visual acuity improved in 4 pa-

tients who had symptoms for less than 12 months, while

visual acuity was unchanged in patients who had symp-

toms for more than 1 year.

Plaque radiotherapy has been reported to offer excellent

results with 100% success in resolution of subretinal fluid

[24–26]. The disadvantages of plaque radiotherapy are

that it requires two operative procedures for insertion

and removal of the plaque with several days of hospital-

ization and may theoretically cause radiation-induced

complications, such as cataract, retinopathy, and papil-

lopathy. This modality should be considered for patients

who have failed to respond to previous treatment (eg,laser photocoagulation) or who are not good candidates

for other treatment modalities because of subfoveal lo-

cation or extensive subretinal fluid. Chao et al. [26] havereported a case of circumscribed choroidal hemangioma

in the macular region with total secondary retinal detach-

ment and iris neovascularization that was successfully

Figure 4. Indocyanine green angiographic appearance of circumscribed choroidal hemangioma.

(A) Reticular hyperfluorescence is visible 20 seconds after injection. (B) Diffuse,intense hyperfluorescence 1 minute after injection. (C) Washout of dye 20minutes after injection; note the surrounding annular hyperfluorescence.

146 Retina and vitreous disorders

Page 6: Circumscribed choroidal hemangioma

managed with iodine-125 plaque radiotherapy. Com-

plete resolution of subretinal fluid and iris neovascular-

ization was achieved after treatment, thus preventing neo-

vascular glaucoma and avoiding the need for enucleation.

Other radiotherapeutic methods, such as external beam

radiotherapy [2••,18,25,27] and proton beam radio-

therapy [28–30], have been reported to successfully man-

age circumscribed choroidal hemangiomas. Recently,

Ritland et al. [27] published the results of nine eyes

treated with fractionated external beam irradiation. All

eyes responded favorably with regression in tumor thick-

ness, resolution of subretinal fluid, and improvement of

visual acuity. No radiation side effects were noted during

the follow-up period (range, 0.4–8.8 years). Similar good

results have been obtained with proton beam radio-

therapy, providing resolution of subretinal fluid in 67 to

100% of patients [28–30]. It has been claimed that proton

beam-induced papillopathy and maculopathy can be

avoided if a low dose of 18 Gy is delivered [29,30].

Photodynamic therapy is the most recent modality used

for the management of circumscribed choroidal heman-

giomas. PDT using benzoporphyrin-MA (verteporfin)

has been previously shown to cause immediate disinte-

gration of endothelial membranes and vascular thrombo-

sis, leading to complete necrosis of experimental choroi-

dal melanoma in a rabbit model [31]. In contrast to laser

photocoagulation and transpupillary thermotherapy, the

success of PDT does not depend on a thermal effect,

allowing a selective occlusion of vascular lesions with

minimal damage to the adjacent retina [32].

During the past 2 years, 5 small case series have been

published on circumscribed choroidal hemangioma

managed with photodynamic therapy (Table 4)

[33,34•,35•,36, 37••]. All treated patients have shown an

excellent response to PDT, with rapid resorption of sub-

retinal fluid and complete flattening of hemangioma. Vi-

sual acuity improved in all but 2 of the 24 treated eyes.

None of the patients developed retinal damage, retinal

nonperfusion, or visual fields defects. Following treat-

ment, some investigators noted RPE alterations at the

site of the original tumor [37••]. Persistent, focal choroi-

dal ischemia and atrophy were reported after treatment

of prominent lesions with three or more sessions of PDT

[37••]. There was no recurrence of tumor or subretinal

Figure 6. Optical coherence tomography of circumscribed choroidal hemangioma

Optical coherence tomography of the circumscribedchoroidal hemangioma shown in Figure 1A reveals thepresence of subretinal fluid adjacent to the foveola.

Figure 5. Magnetic resonance imaging of circumscribed

choroidal hemangioma.

(A) On T1-weighted image with gadolinium enhancement, the hemangioma isdistinctly hyperintense compared with the vitreous. (B) On T2-weighted image,the tumor is almost indistinguishable from the vitreous.

Circumscribed choroidal hemangioma Mashayekhi and Shields 147

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fluid during follow-up periods of 3 to 50 months. Al-

though there has been no report of optic nerve damage in

treated eyes with juxtapapillary choroidal hemangiomas,

it is important to avoid directing the laser beam at the

optic disc because optic nerve ischemia has been reported

after PDT of papillary capillary hemangiomas [38].

Photodynamic therapy has been successfully employed

for management of subfoveal choroidal hemangiomas

[33,35•,36,37••]. Sheidow and Harbour treated a patient

with subfoveal choroidal hemangioma and visual acuity

of 20/400. Following treatment, visual acuity improved to

20/50 with minimal pigment epithelial changes over the

tumor [36]. One of the patients reported by Robertson

had a subfoveal choroidal hemangioma with cystoid

macular edema and visual acuity of 3/200. Two months

after treatment, visual acuity increased to 20/200 [35•].

One of the patients reported by Barbazetto [33] also had

a subfoveal hemangioma with visual acuity of 20/120 im-

proving to 20/50 following treatment. Although indi-

vidual visual acuity results have not been provided,

Schmidt-Erfurth et al. [37••] did not detect any residual

field defects in three eyes with subfoveal hemangiomas,

“other than the scotoma related to the extrafoveal area

showing choroidal atrophy because of overtreatment.”

Obviously, it is important to avoid overtreatment in PDT

of subfoveal hemangiomas. We have treated 10 patients

with circumscribed choroidal hemangioma with single-

spot, single-session PDT thus far, and subretinal fluid

has resolved and vision improved in each patient. In

Table 4, it becomes evident that patients with subfoveal

hemangiomas generally presented with lower visual acu-

ities and ended with lower visual acuities compared with

those with extrafoveal tumors.

Despite initial good results, many questions are still un-

answered regarding the role of PDT in management of

circumscribed choroidal hemangiomas. These include

criteria for case selection (which lesions and when to

treat), physical parameters of treatment (power, duration,

spot size, and number of spots used in one session), num-

ber of sessions, interval between sessions (treatment in-

tervals), endpoint of treatment (resolution of leakage and

subretinal fluid; complete flattening of tumor), and long-

term recurrence rate and complications. Larger studies

Table 3. Points regarding treatment of circumscribed

choroidal hemangioma

• Asymptomatic tumors need only periodic observation.• Laser photocoagulation, despite initial good response, may be

associated with a considerable recurrence rate.• Both laser photocoagulation and transpupillary thermotherapy can

be visually destructive and should be limited to treatment ofextrafoveal hemangiomas.

• Photodynamic therapy is an effective new modality and can beused for both foveal and extrafoveal hemangiomas. The long-termresults of this treatment modality are not yet known.

• Radiotherapeutic methods should be considered for cases thathave failed previous treatment or are not good candidates for othertreatment modalities because of subfoveal location or extensivesubretinal fluid.

Table 4. Clinical features, treatment parameters, and outcome to treatment of 24 published cases of

circumscribed choroidal hemangioma treated by PDT

Author Location VA (initial � final)Thickness, mm(initial � final) SRF (initial � final) Rx parameters

# Rxsessions

FU,mo.

Barbazetto, et al33

Case 1 Extrafoveal 20/40 � 20/20 3.3 � Flat Yes � Absorbed 100 J/cm2 2 12Case 2 Subfoveal 20/120 � 20/50 4.6 � Flat Yes � Absorbed One or more

overlappingspots

4 9

Madreperla34

Case 1 Juxtafoveal 20/50 � 20/25 2.4 � 1.2 Yes � Absorbed 50 J/cm2

single spot1 3

Case 2 Extrafoveal,Juxtapapillary

20/70 � 20/20 2.0 � Flat Yes � Absorbed 1 9

Case 3 Juxtafoveal 20/50 � 20/40 2.8 � Flat Yes � Absorbed 1 4Robertson35

Case 1 Extrafoveal,Juxtapapillary

20/50 � 20/20 2.4 � Flat Yes � Absorbed 50 J/cm2

3 to 6overlappingspots

2 14

Case 2 Extrafoveal,Juxtapapillary

20/150 � 20/20 2.9 � Flat Yes � Absorbed 1 13

Case 3 Subfoveal,Juxtapapillary

3/200 �20/200 3.9 � Flat Yes � Absorbed 2 11

Sheidow, et al36

Case 1 Subfoveal 20/400 � 20/50 3.8 � Flat Yes � Absorbed 50 J/cm2

single spot2 12

Schmidt-Erfurth, et al37

15 cases* Juxtapapillary12 cases

Subfoveal3 cases

20/125 � 20/80* 3.8* � Flat Yes � Absorbed 100 J/cm2

single spot1–4 12–50

*The characteristics of individual cases were not described in this report. Figures represent mean values.

148 Retina and vitreous disorders

Page 8: Circumscribed choroidal hemangioma

with longer follow-up periods are necessary to answer

these questions.

ConclusionsCircumscribed choroidal hemangioma is a benign vascu-

lar tumor capable of causing decreased vision secondary

to exudative retinal detachment. Despite its rather char-

acteristic ophthalmoscopic appearance, many patients

are initially misdiagnosed with other choroidal tumors.

Asymptomatic lesions should be observed, but visually

threatening or visually impairing lesions require treat-

ment. PDT has recently emerged as an effective and

promising new treatment modality for extrafoveal and

subfoveal tumors.

References and recommended reading

Papers of particular interest, published within the annual period of review,have been highlighted as:• Of special interest•• Of outstanding interest

1 Shields JA, Shields CL: Atlas of Intraocular Tumors. Philadelphia: LippincottWilliams & Wilkins; 1999:170–179.

••2 Shields CL, Honavar SG, Shields JA, et al.: Circumscribed choroidal heman-

gioma: clinical manifestations and factors predictive of visual outcome in 200consecutive cases. Ophthalmology 2001, 108:2237–2248.

The authors reviewed the clinical and ancillary testing characteristics of 200 casesof circumscribed choroidal hemangioma and determined factors predictive of poorvisual outcome.

3 Witschel H, Font RL: Hemangioma of the choroid. A clinicopathologic studyof 71 cases and a review of the literature. Surv Ophthalmol 1976, 20:415–431.

4 Anand R, Augsburger JJ, Shields JA: Circumscribed choroidal hemangiomas.Arch Ophthalmol 1989, 107:1338–1342.

5 Shields CL, Shields JA, Gross NE, et al.: Survey of 520 uveal metastases.Ophthalmology 1997, 104:1265–1276.

6 Scott IU, Alexandrakis G, Cordahi GJ, et al.: Diffuse and circumscribed cho-roidal hemangiomas in a patient with Sturge-Weber syndrome. Arch Ophthal-mol 1999, 117:406–407.

7 Cheung D, Grey R, Renni I: Circumscribed choroidal haemangioma in a pa-tient with Sturge-Weber syndrome [letter]. Eye 2000, 14:238–240.

•8 Cohen VM, Rundle PA, Rennie IG: Choroidal hemangiomas with exudative

retinal detachments during pregnancy. Arch Ophthalmol 2002, 120:862–864.

The clinical course of three patients with circumscribed choroidal hemangiomaduring and following delivery is described.

9 Medlock RD, Augsburger JJ, Wilkinson CP, et al.: Enlargement of circum-scribed choroidal hemangiomas. Retina 1991, 11:385–388.

10 Shields JA, Stephens RF, Eagle RC Jr, et al.: Progressive enlargement of acircumscribed choroidal hemangioma. A clinicopathologic correlation. ArchOphthalmol 1992, 110:1276–1278.

11 Pitta C, Bergen R, Littwin S: Spontaneous regression of a choroidal heman-gioma following pregnancy. Ann Ophthalmol 1979, 11:772–774.

12 Shields CL: Diagnostic and therapeutic challenges. Retina 1999, 19:69–74.

13 Verbeek AM, Koutentakis P, Deutman AF: Circumscribed choroidal heman-gioma diagnosed by ultrasonography. A retrospective analysis of 40 cases.Int Ophthalmol 1995–96, 19:185–189.

14 Shields CL, Shields JA, De Potter P: Patterns of indocyanine green videoan-giography of choroidal tumors. Br J Ophthalmol 1995, 79:237–245.

15 Arevalo JF, Shields CL, Shields JA, et al.: Circumscribed choroidal hemangi-oma: characteristic features with indocyanine green videoangiography. Oph-thalmology 2000, 107:344–350.

16 Stroszczynski C, Hosten N, Bornfeld N, et al.: Choroidal hemangioma: MRfindings and differentiation from uveal melanoma. AJNR Am J Neuroradiol1998, 19:1441–1447.

17 Fuchs AV, Mueller AJ, Grueterich M, et al.: Transpupillary thermotherapy(TTT) in circumscribed choroidal hemangioma. Graefes Arch Clin Exp Oph-thalmol 2002, 240:7–11.

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•34 Madreperla SA: Choroidal hemangioma treated with photodynamic therapy

using verteporfin. Arch Ophthalmol 2001, 119:1606–1610.Photodynamic therapy was successful in eliminating subretinal fluid and improvingvision in three patients with circumscribed choroidal hemangioma.

•35 Robertson DM: Photodynamic therapy for choroidal hemangioma associated

with serous retinal detachment. Arch Ophthalmol 2002, 120:1155–1161.Two patients with extrafoveal and one patient with subfoveal circumscribed cho-roidal hemangioma were treated with photodynamic therapy. Serous detachmentresolved with improvement of visual acuity. Nerve fiber bundle defects were notidentified.

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symptomatic choroidal hemangioma: visual and anatomic results. Ophthal-mology 2002, 109:2284–2294.

Documents the outcome of photodynamic therapy of 15 patients with circum-scribed choroidal hemangioma over a mean interval of 19 months (12–50 months).

38 Schmidt-Erfurth UM, Kusserow C, Barbazetto IA, et al.: Benefits and compli-cations of photodynamic therapy of papillary capillary hemangiomas. Oph-thalmology 2002, 109:1256–1266.

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