Circumscribed choroidal hemangioma is a benign vascular tumor that occurs as an orange-red mass in the posterior pole of the eye. It most commonly causes decreased vision through serous retinal detachment. Diagnosis can be challenging as it is often initially misdiagnosed as choroidal melanoma or metastasis. Imaging such as ultrasound, fluorescein angiography, and MRI help differentiate it from other lesions. Asymptomatic lesions may be observed, while visually threatening lesions require treatment such as photodynamic therapy, laser photocoagulation, or radiotherapy. Factors like delayed treatment and poor initial vision predict a worse visual outcome.

1. Circumscribed choroidal hemangioma
Arman Mashayekhi, MD, and Carol L. Shields, MD
Circumscribed choroidal hemangioma is an uncommon, Background
benign vascular tumor manifesting as an orange-red mass in Choroidal hemangioma is a benign, vascular, hamartoma-
the posterior pole of the eye. Serous retinal detachment tous tumor occurring in two distinct forms, circumscribed
accounts for decreased vision in most patients. Diagnosis of and diffuse, on the basis of the extent of choroidal in-
this tumor is challenging with many patients initially volvement. Circumscribed choroidal hemangioma is gen-
misdiagnosed with choroidal melanoma or metastasis. Several erally a solitary finding without systemic associations,
ancillary tests such as ultrasonography, fluorescein whereas diffuse choroidal hemangioma usually occurs in
angiography, indocyanine green angiography, and magnetic association with encephalofacial angiomatosis (Sturge-
resonance imaging help differentiate this tumor from other Weber syndrome) [1].
simulating lesions. Asymptomatic lesions should be observed,
but visually threatening or visually impairing lesions require The diagnosis and management of circumscribed choroi-
treatment. Photodynamic therapy, laser photocoagulation, and dal hemangioma continue to be a challenge for most
transpupillary thermotherapy may be used for primary clinicians. Many patients with choroidal hemangioma are
management of this tumor. Patients who fail to respond to referred to us because of suspected choroidal melanoma
previous treatment or those with extensive serous retinal or choroidal metastasis. Therefore, it is important to
detachment can be treated using radiotherapeutic modalities. achieve an accurate diagnosis of this tumor, reassure pa-
Long interval between onset of symptoms and treatment, poor tients of its benign nature, and provide proper therapy.
visual acuity at presentation, and presence of chronic retinal or
retinal pigment epithelial changes are associated with poor Diagnosis
long-term vision. Curr Opin in Ophthalmol 2003, 14:142–149 © 2003 Clinical manifestations
Lippincott Williams & Wilkins. 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,
Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, whereas during the same period, more than 10,000 pa-
Philadelphia, Pennsylvania, USA.
tients with choroidal melanoma were seen at that center.
Supported by the Eye Tumor Research Foundation, Philadelphia, PA (C.L.S.), the
Macula Foundation, New York, NY (C.L.S.), and the Rosenthal Award of the
Macula Society (C.L.S.). Although probably congenital, most patients do not de-
velop symptoms until they are in their fourth to sixth
Correspondence to Carol L. Shields, MD, Ocular Oncology Service, Wills Eye
Hospital, 840 Walnut Street, Philadelphia, PA 19107, USA decade of life. In the series reported from the Oncology
Service at Wills Eye Hospital, the mean age at presen-
Current Opinion in Ophthalmology 2003, 14:142–149
tation was 47 years with a range of 4 to 81 years [2••].
Abbreviations Most patients present with decreased visual acuity.
PDT photodynamic therapy Other less common symptoms include visual field de-
RPE retinal pigment epithelium
fect, metamorphopsia, flashes and floaters, and progres-
ISSN 1040–8738 © 2003 Lippincott Williams & Wilkins 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••].
142

2. Circumscribed choroidal hemangioma Mashayekhi and Shields 143
Figure 1. Clinical appearance of circumscribed choroidal the Oncology Service at Wills Eye Hospital, the clinical
hemangioma
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
(A) Wide-angle fundus photograph of circumscribed choroidal hemangioma with Table 1. Referral diagnosis in 200 consecutive patients with
associated subretinal fluid. (B) Wide-angle fundus photograph of circumscribed circumscribed choroidal hemangioma referred to the Oncology
choroidal hemangioma with minimal associated subretinal fluid and overlying Service, Wills Eye Hospital [2••]
retinal pigment epithelial alterations. Referral diagnosis Number (%)
Choroidal hemangioma 58 (29)
This benign tumor can cause visual impairment by vari- Choroidal melanoma 58 (29)
Choroidal metastasis 17 (9)
ous mechanisms, such as exudative retinal detachment, Retinal detachment 12 (6)
overlying photoreceptor degeneration, elevation or tilt- Central serous chorioretinopathy 9 (5)
ing of the macular region, cystoid macular edema, sub- Macular edema 5 (3)
Others 13 (9)
retinal fibrosis, RPE alterations, and retinoschisis [1,2••]. No diagnosis 28 (14)
Differential diagnosis Adapted from: Shields CL, Honavar SG, Shields JA, Cater J, Demirci
H. Circumscribed choroidal hemangioma: clinical manifestations and
The diagnosis of circumscribed choroidal hemangioma factors predictive of visual outcome in 200 consecutive cases.
can be challenging. Of the 200 patients in the series from Ophthalmology 2001; 108:2237–48.

3. 144 Retina and vitreous disorders
tumor—can appear orange, similar to choroidal heman- Table 2. Characteristic features of circumscribed choroidal
hemangioma on ancillary testing
gioma [1,5].
Ultrasonography
A-scan: High internal reflectivity
It is generally believed that circumscribed choroidal B-scan: Acoustically solid (similar to normal choroid)
hemangioma is not associated with Sturge-Weber syn- Fluorescein angiography
drome, but in the Wills Eye Hospital series, there were Early: Mild, lacy hyperfluorescence
Late: Intense, diffuse hyperfluorescence
four patients with typical circumscribed choroidal hem- Indocyanine green angiography
angioma who had a facial nevus flammeus and other Early: Hyperfluorescence
manifestations of Sturge-Weber syndrome [2••]. The Late: Dye “washout”
MRI
findings in the choroid may have been a limited form of Bright signal on both T1- and T2-weighted images
this syndrome because others have also observed this
association [6,7,8•]. Five additional patients showed sys-
temic mucosal or remote cutaneous hemangiomas, and sic vascular pulsations; however, similar ultrasonographic
one patient had neurofibromatosis [2••]. features may be seen with choroidal metastasis [1].
Natural course
Fluorescein angiography of choroidal hemangioma typi-
Most cases of circumscribed choroidal hemangioma are cally shows lacy hyperfluorescence in the prearterial or
stationary, but several authors have reported spontane- early arterial phase and diffuse, intense, late hyperfluo-
ous enlargement of this lesion [9,10]. Enlargement of rescence (Fig. 3). Fluorescein angiographic findings,
choroidal hemangioma is secondary to venous congestion
Figure 2. Ultrasonographic appearance of circumscribed
rather than cellular multiplication [10]. We are aware of choroidal hemangioma
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]. (A) A-scan ultrasound shows the characteristic high internal reflectivity of
These features would be unlikely with choroidal mela- circumscribed choroidal hemangioma. (B) On B-scan ultrasonography,
circumscribed choroidal hemangioma has a solid acoustic appearance similar to
noma because melanoma usually displays acoustic hol- the adjacent choroid.
lowness, low to medium internal reflectivity, and intrin-

4. Circumscribed choroidal hemangioma Mashayekhi and Shields 145
Figure 3. Fluorescein angiographic appearance of other retinal changes associated with circumscribed cho-
circumscribed choroidal hemangioma.
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.
Treatment
The 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].
(A) Lacy appearance of choroidal hemangioma in arterial phase. (B) Late, diffuse
Management of choroidal hemangioma is based on tu-
hyperfluorescence of choroidal hemangioma.
mor size, location, and related ocular symptoms. Asymp-
tomatic circumscribed choroidal hemangiomas only need
although characteristic, are not pathognomonic of this to be observed, and treatment is generally reserved for
tumor [2••]. Indocyanine green angiography demon- patients with vision-threatening or vision-impairing le-
strates an early well-defined area of intense hyperfluo- sions. In the series reported from the Oncology Service at
rescence, often followed by a characteristic “dye wash- Wills Eye Hospital, 43% of patients were treated with
out” in late frames (Fig. 4) [14,15]. These features would observation alone [2••]. These patients generally
be unlikely with choroidal melanoma or metastasis, for showed minimal findings with little or no subretinal fluid
which filling on fluorescein angiography and indocyanine or fluid-related visual disturbances, or, conversely, they
green angiography is slower and less intense. With mag- showed advanced findings with chronic macular edema
netic resonance imaging, choroidal hemangioma shows so great that treatment would have been of little visual
bright signal on T1- and T2-weighted images (Fig. 5) benefit. In addition, observation with refraction is advis-
unlike choroidal melanoma and metastasis, which gen- able for patients who have hyperopic amblyopia second-
erally show bright signal on T1-weighted and low signal ary to subfoveal tumors.
on T2-weighted images (Table 2) [16].
Initially xenon arc photocoagulation and later argon laser
Optical coherence tomography is a useful method for photocoagulation were the most important treatment
detection of minimal amounts of subretinal fluid and modalities [2••,4,19]. More recently, transpupillary

5. 146 Retina and vitreous disorders
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 20
minutes after injection; note the surrounding annular hyperfluorescence.
thermotherapy [17,20–23], plaque radiotherapy [24–26], angiomas. Recently, Fuchs et al. [17] have reported their
external beam radiotherapy [2••,18,25,27], proton beam results of treatment of 10 patients with circumscribed
radiotherapy [28–30], and photodynamic therapy (PDT) choroidal hemangioma using transpupillary thermo-
[33,34••,35–37] have been introduced (Table 3). therapy. Subretinal fluid persisted in three eyes because
the hemangioma could not be treated completely due to
Laser photocoagulation has been used extensively for proximity to the fovea. Visual acuity improved in 4 pa-
management of circumscribed choroidal hemangiomas tients who had symptoms for less than 12 months, while
[2••,4,19]. While success rates as high as 100% have visual acuity was unchanged in patients who had symp-
been reported [4], others have reported recurrent sub- toms for more than 1 year.
retinal fluid in more than 50% of treated patients [19]. In
the series reported from Wills Eye Hospital, argon laser Plaque radiotherapy has been reported to offer excellent
photocoagulation was successful in completely resolving results with 100% success in resolution of subretinal fluid
subretinal fluid in 62% of patients [2••]. In general, if [24–26]. The disadvantages of plaque radiotherapy are
the subretinal fluid does not respond appropriately to that it requires two operative procedures for insertion
one or two sessions of surface and delimiting argon laser and removal of the plaque with several days of hospital-
photocoagulation, other treatment modalities should ization and may theoretically cause radiation-induced
be employed. complications, such as cataract, retinopathy, and papil-
lopathy. This modality should be considered for patients
In several small series, transpupillary thermotherapy has who have failed to respond to previous treatment (eg,
been reported to cause resolution of choroidal hemangi- laser photocoagulation) or who are not good candidates
oma-related subretinal fluid, both as a primary treatment for other treatment modalities because of subfoveal lo-
and following failed prior laser photocoagulation [17,20– cation or extensive subretinal fluid. Chao et al. [26] have
23]. This method can occasionally be visually destructive reported a case of circumscribed choroidal hemangioma
and cause further visual field and acuity loss; therefore, it in the macular region with total secondary retinal detach-
should not be used for management of subfoveal hem- ment and iris neovascularization that was successfully

6. Circumscribed choroidal hemangioma Mashayekhi and Shields 147
Figure 5. Magnetic resonance imaging of circumscribed Ritland et al. [27] published the results of nine eyes
choroidal hemangioma.
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
(A) On T1-weighted image with gadolinium enhancement, the hemangioma is
published on circumscribed choroidal hemangioma
distinctly hyperintense compared with the vitreous. (B) On T2-weighted image,
the tumor is almost indistinguishable from the vitreous. 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-
managed with iodine-125 plaque radiotherapy. Com- retinal fluid and complete flattening of hemangioma. Vi-
plete resolution of subretinal fluid and iris neovascular- sual acuity improved in all but 2 of the 24 treated eyes.
ization was achieved after treatment, thus preventing neo- None of the patients developed retinal damage, retinal
vascular glaucoma and avoiding the need for enucleation. nonperfusion, or visual fields defects. Following treat-
ment, some investigators noted RPE alterations at the
Other radiotherapeutic methods, such as external beam site of the original tumor [37••]. Persistent, focal choroi-
radiotherapy [2••,18,25,27] and proton beam radio- dal ischemia and atrophy were reported after treatment
therapy [28–30], have been reported to successfully man- of prominent lesions with three or more sessions of PDT
age circumscribed choroidal hemangiomas. Recently, [37••]. There was no recurrence of tumor or subretinal
Figure 6. Optical coherence tomography of circumscribed choroidal hemangioma
Optical coherence tomography of the circumscribed
choroidal hemangioma shown in Figure 1A reveals the
presence of subretinal fluid adjacent to the foveola.

7. 148 Retina and vitreous disorders
Table 3. Points regarding treatment of circumscribed after treatment, visual acuity increased to 20/200 [35•].
choroidal hemangioma
One of the patients reported by Barbazetto [33] also had
• Asymptomatic tumors need only periodic observation. a subfoveal hemangioma with visual acuity of 20/120 im-
• Laser photocoagulation, despite initial good response, may be
associated with a considerable recurrence rate.
proving to 20/50 following treatment. Although indi-
• Both laser photocoagulation and transpupillary thermotherapy can vidual visual acuity results have not been provided,
be visually destructive and should be limited to treatment of Schmidt-Erfurth et al. [37••] did not detect any residual
extrafoveal hemangiomas.
• Photodynamic therapy is an effective new modality and can be
field defects in three eyes with subfoveal hemangiomas,
used for both foveal and extrafoveal hemangiomas. The long-term “other than the scotoma related to the extrafoveal area
results of this treatment modality are not yet known. showing choroidal atrophy because of overtreatment.”
• Radiotherapeutic methods should be considered for cases that
have failed previous treatment or are not good candidates for other
Obviously, it is important to avoid overtreatment in PDT
treatment modalities because of subfoveal location or extensive of subfoveal hemangiomas. We have treated 10 patients
subretinal fluid. with circumscribed choroidal hemangioma with single-
spot, single-session PDT thus far, and subretinal fluid
fluid during follow-up periods of 3 to 50 months. Al- has resolved and vision improved in each patient. In
though there has been no report of optic nerve damage in Table 4, it becomes evident that patients with subfoveal
treated eyes with juxtapapillary choroidal hemangiomas, hemangiomas generally presented with lower visual acu-
it is important to avoid directing the laser beam at the ities and ended with lower visual acuities compared with
optic disc because optic nerve ischemia has been reported those with extrafoveal tumors.
after PDT of papillary capillary hemangiomas [38].
Despite initial good results, many questions are still un-
Photodynamic therapy has been successfully employed answered regarding the role of PDT in management of
for management of subfoveal choroidal hemangiomas circumscribed choroidal hemangiomas. These include
[33,35•,36,37••]. Sheidow and Harbour treated a patient criteria for case selection (which lesions and when to
with subfoveal choroidal hemangioma and visual acuity treat), physical parameters of treatment (power, duration,
of 20/400. Following treatment, visual acuity improved to spot size, and number of spots used in one session), num-
20/50 with minimal pigment epithelial changes over the ber of sessions, interval between sessions (treatment in-
tumor [36]. One of the patients reported by Robertson tervals), endpoint of treatment (resolution of leakage and
had a subfoveal choroidal hemangioma with cystoid subretinal fluid; complete flattening of tumor), and long-
macular edema and visual acuity of 3/200. Two months term recurrence rate and complications. Larger studies
Table 4. Clinical features, treatment parameters, and outcome to treatment of 24 published cases of
circumscribed choroidal hemangioma treated by PDT
Thickness, mm # Rx FU,
Author Location VA (initial final) (initial final) SRF (initial final) Rx parameters sessions mo.
Barbazetto, et al33
Case 1 Extrafoveal 20/40 20/20 3.3 Flat Yes Absorbed 100 J/cm2 2 12
Case 2 Subfoveal 20/120 20/50 4.6 Flat Yes Absorbed One or more 4 9
overlapping
spots
Madreperla34
Case 1 Juxtafoveal 20/50 20/25 2.4 1.2 Yes Absorbed 50 J/cm2 1 3
single spot
Case 2 Extrafoveal, 20/70 20/20 2.0 Flat Yes Absorbed 1 9
Juxtapapillary
Case 3 Juxtafoveal 20/50 20/40 2.8 Flat Yes Absorbed 1 4
Robertson35
Case 1 Extrafoveal, 20/50 20/20 2.4 Flat Yes Absorbed 50 J/cm2 2 14
Juxtapapillary 3 to 6
overlapping
spots
Case 2 Extrafoveal, 20/150 20/20 2.9 Flat Yes Absorbed 1 13
Juxtapapillary
Case 3 Subfoveal, 3/200 20/200 3.9 Flat Yes Absorbed 2 11
Juxtapapillary
Sheidow, et al36
Case 1 Subfoveal 20/400 20/50 3.8 Flat Yes Absorbed 50 J/cm2 2 12
single spot
Schmidt-Erfurth, et al37
15 cases* Juxtapapillary 20/125 20/80* 3.8* Flat Yes Absorbed 100 J/cm2 1–4 12–50
12 cases single spot
Subfoveal
3 cases
*The characteristics of individual cases were not described in this report. Figures represent mean values.

8. Circumscribed choroidal hemangioma Mashayekhi and Shields 149
with longer follow-up periods are necessary to answer 17 Fuchs AV, Mueller AJ, Grueterich M, et al.: Transpupillary thermotherapy
(TTT) in circumscribed choroidal hemangioma. Graefes Arch Clin Exp Oph-
these questions. thalmol 2002, 240:7–11.
18 Schilling H, Sauerwein W, Lommatzsch A, et al.: Long-term results after low
Conclusions dose ocular irradiation for choroidal haemangiomas. Br J Ophthalmol 1997,
81:267–273.
Circumscribed choroidal hemangioma is a benign vascu-
lar tumor capable of causing decreased vision secondary 19 Sanborn GE, Augsburger JJ, Shields JA: Treatment of circumscribed choroi-
dal hemangiomas. Ophthalmology 1982, 89:1374–1380.
to exudative retinal detachment. Despite its rather char-
20 Othmane IS, Shields CL, Shields JA, et al.: Circumscribed choroidal heman-
acteristic ophthalmoscopic appearance, many patients gioma managed by transpupillary thermotherapy. Arch Ophthalmol 1999,
are initially misdiagnosed with other choroidal tumors. 117:136–137.
Asymptomatic lesions should be observed, but visually 21 Rapizzi E, Grizzard WS, Capone A Jr: Transpupillary thermotherapy in the
threatening or visually impairing lesions require treat- management of circumscribed choroidal hemangioma. Am J Ophthalmol
1999, 127:481–482.
ment. PDT has recently emerged as an effective and
22 Garcia-Arumi J, Ramsay LS, Guraya BC: Transpupillary thermotherapy for
promising new treatment modality for extrafoveal and circumscribed choroidal hemangiomas. Ophthalmology 2000, 107:351–
subfoveal tumors. 356.
23 Mosci C, Polizzi A, Zingirian M: Transpupillary thermotherapy for circum-
scribed choroidal hemangiomas: first choice in therapy. Eur J Ophthalmol
References and recommended reading 2001, 11:316–318.
Papers of particular interest, published within the annual period of review,
have been highlighted as: 24 Zografos L, Bercher L, Chamot L, et al.: Cobalt-60 treatment of choroidal
• Of special interest hemangiomas. Am J Ophthalmol 1996, 121:190–199.
•• Of outstanding interest 25 Madreperla SA, Hungerford JL, Plowman PN, et al.: Choroidal hemangiomas:
1 Shields JA, Shields CL: Atlas of Intraocular Tumors. Philadelphia: Lippincott visual and anatomic results of treatment by photocoagulation or radiation
Williams & Wilkins; 1999:170–179. therapy. Ophthalmology 1997, 104:1773–1778.
2 Shields CL, Honavar SG, Shields JA, et al.: Circumscribed choroidal heman- 26 Chao AN, Shields CL, Shields JA, et al.: Plaque radiotherapy for choroidal
•• gioma: clinical manifestations and factors predictive of visual outcome in 200 hemangioma with total retinal detachment and iris neovascularization. Retina
consecutive cases. Ophthalmology 2001, 108:2237–2248. 2001, 21:682–684.
The authors reviewed the clinical and ancillary testing characteristics of 200 cases
of circumscribed choroidal hemangioma and determined factors predictive of poor 27 Ritland JS, Eide N, Tausjo J: External beam irradiation therapy for choroidal
visual outcome. haemangiomas. Visual and anatomic results after a dose of 20 to 25 Gy. Acta
Ophthalmol Scand 2001, 79:184–186.
3 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– 28 Hannouche D, Frau E, Desjardins L, et al.: Efficacy of proton therapy in cir-
431. cumscribed choroidal hemangiomas associated with serious retinal detach-
ment. Ophthalmology 1997, 104:1780–1784.
4 Anand R, Augsburger JJ, Shields JA: Circumscribed choroidal hemangiomas.
Arch Ophthalmol 1989, 107:1338–1342. 29 Lee V, Hungerford JL: Proton beam therapy for posterior pole circumscribed
choroidal haemangioma. Eye 1998, 12:925–928.
5 Shields CL, Shields JA, Gross NE, et al.: Survey of 520 uveal metastases.
Ophthalmology 1997, 104:1265–1276. 30 Zografos L, Egger E, Bercher L, et al.: Proton beam irradiation of choroidal
hemangiomas. Am J Ophthalmol 1998, 126:261–268.
6 Scott IU, Alexandrakis G, Cordahi GJ, et al.: Diffuse and circumscribed cho-
roidal hemangiomas in a patient with Sturge-Weber syndrome. Arch Ophthal- 31 Schmidt-Erfurth U, Bauman W, Gragoudas E, et al.: Photodynamic therapy of
mol 1999, 117:406–407. experimental choroidal melanoma using lipoprotein-delivered benzoporphy-
rin. Ophthalmology 1994, 101:89–99.
7 Cheung D, Grey R, Renni I: Circumscribed choroidal haemangioma in a pa-
tient with Sturge-Weber syndrome [letter]. Eye 2000, 14:238–240. 32 Schmidt-Erfurth U, Hasan T, Gragoudas E, et al.: Vascular targeting in pho-
todynamic occlusion of subretinal vessels. Ophthalmology 1994, 101:1953–
8 Cohen VM, Rundle PA, Rennie IG: Choroidal hemangiomas with exudative
1961.
• retinal detachments during pregnancy. Arch Ophthalmol 2002, 120:862–
864. 33 Barbazetto I, Schmidt-Erfurth: Photodynamic therapy of choroidal hemangi-
The clinical course of three patients with circumscribed choroidal hemangioma oma: two case reports. Graefes Arch Clin Exp Ophthalmol 2000, 238:214–
during and following delivery is described. 221.
9 Medlock RD, Augsburger JJ, Wilkinson CP, et al.: Enlargement of circum- 34 Madreperla SA: Choroidal hemangioma treated with photodynamic therapy
scribed choroidal hemangiomas. Retina 1991, 11:385–388. using verteporfin. Arch Ophthalmol 2001, 119:1606–1610.
•
10 Shields JA, Stephens RF, Eagle RC Jr, et al.: Progressive enlargement of a Photodynamic therapy was successful in eliminating subretinal fluid and improving
circumscribed choroidal hemangioma. A clinicopathologic correlation. Arch vision in three patients with circumscribed choroidal hemangioma.
Ophthalmol 1992, 110:1276–1278.
35 Robertson DM: Photodynamic therapy for choroidal hemangioma associated
11 Pitta C, Bergen R, Littwin S: Spontaneous regression of a choroidal heman- • with serous retinal detachment. Arch Ophthalmol 2002, 120:1155–1161.
gioma following pregnancy. Ann Ophthalmol 1979, 11:772–774. Two patients with extrafoveal and one patient with subfoveal circumscribed cho-
roidal hemangioma were treated with photodynamic therapy. Serous detachment
12 Shields CL: Diagnostic and therapeutic challenges. Retina 1999, 19:69–74. resolved with improvement of visual acuity. Nerve fiber bundle defects were not
13 Verbeek AM, Koutentakis P, Deutman AF: Circumscribed choroidal heman- identified.
gioma diagnosed by ultrasonography. A retrospective analysis of 40 cases. 36 Sheidow TG, Harbour JW: Photodynamic therapy for circumscribed choroi-
Int Ophthalmol 1995–96, 19:185–189. dal hemangioma. Can J Ophthalmol 2002, 37:314–317.
14 Shields CL, Shields JA, De Potter P: Patterns of indocyanine green videoan-
37 Schmidt-Erfurth UM, Michels S, Kusserow C, et al.: Photodynamic therapy for
giography of choroidal tumors. Br J Ophthalmol 1995, 79:237–245.
•• symptomatic choroidal hemangioma: visual and anatomic results. Ophthal-
15 Arevalo JF, Shields CL, Shields JA, et al.: Circumscribed choroidal hemangi- mology 2002, 109:2284–2294.
oma: characteristic features with indocyanine green videoangiography. Oph- Documents the outcome of photodynamic therapy of 15 patients with circum-
thalmology 2000, 107:344–350. scribed choroidal hemangioma over a mean interval of 19 months (12–50 months).
16 Stroszczynski C, Hosten N, Bornfeld N, et al.: Choroidal hemangioma: MR 38 Schmidt-Erfurth UM, Kusserow C, Barbazetto IA, et al.: Benefits and compli-
findings and differentiation from uveal melanoma. AJNR Am J Neuroradiol cations of photodynamic therapy of papillary capillary hemangiomas. Oph-
1998, 19:1441–1447. thalmology 2002, 109:1256–1266.