Circumscribed choroidal hemangioma

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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 in
the posterior pole of the eye. Serous retinal detachment
accounts for decreased vision in most patients. Diagnosis of
this tumor is challenging with many patients initially
misdiagnosed with choroidal melanoma or metastasis. Several
ancillary tests such as ultrasonography, fluorescein
angiography, indocyanine green angiography, and magnetic
resonance imaging help differentiate this tumor from other
simulating lesions. Asymptomatic lesions should be observed,
but visually threatening or visually impairing lesions require
treatment. Photodynamic therapy, laser photocoagulation, and
transpupillary thermotherapy may be used for primary
management of this tumor. Patients who fail to respond to
previous treatment or those with extensive serous retinal
detachment can be treated using radiotherapeutic modalities.
Long interval between onset of symptoms and treatment, poor
visual acuity at presentation, and presence of chronic retinal or
retinal pigment epithelial changes are associated with poor
long-term vision.
Curr Opin in Ophthalmol 2003, 14:142–149 © 2003
Lippincott Williams & Wilkins.
Background
Choroidal 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.
Diagnosis
Clinical 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 81years [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.), the
Macula Foundation, New York, NY (C.L.S.), and the Rosenthal Award of the
Macula Society (C.L.S.).
Correspondence to Carol L. Shields, MD, Ocular Oncology Service, Wills Eye
Hospital, 840 Walnut Street, Philadelphia, PA 19107, USA
Current Opinion in Ophthalmology2003, 14:142–149
Abbreviations
PDT photodynamic therapy
RPE retinal pigment epithelium
ISSN 1040–8738 © 2003 Lippincott Williams & Wilkins
142

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 with
associated subretinal fluid. (B) Wide-angle fundus photograph of circumscribed
choroidal hemangioma with minimal associated subretinal fluid and overlying
retinal 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, Demirci
H. Circumscribed choroidal hemangioma: clinical manifestations and
factors predictive of visual outcome in 200 consecutive cases.
Ophthalmology 2001; 108:2237–48.
Circumscribed choroidal hemangiomaMashayekhi and Shields 143

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 reflectivityof
circumscribed choroidal hemangioma. (B) On B-scan ultrasonography,
circumscribed choroidal hemangioma has a solid acoustic appearance similar to
the adjacent choroid.
Table 2. Characteristic features of circumscribed choroidal hemangioma on ancillary testing
Ultrasonography
A-scan: High internal reflectivity B-scan: Acoustically solid (similar to normal choroid)
Fluorescein angiography
Early: Mild, lacy hyperfluorescence Late: Intense, diffuse hyperfluorescence
Indocyanine green angiography
Early: Hyperfluorescence Late: Dye“washout”
MRI
Bright signal on both T1- and T2-weighted images
144Retina and vitreous disorders

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.
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 (C20/200) 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, diffuse
hyperfluorescence of choroidal hemangioma.
Circumscribed choroidal hemangiomaMashayekhi and Shields 145

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, Fuchset 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 1year.
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. Chaoet al.[26] have
reported 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 20
minutes after injection; note the surrounding annular hyperfluorescence.
146Retina and vitreous disorders

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,
Ritlandet 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 circumscribed
choroidal hemangioma shown in Figure 1A reveals the
presence 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 is
distinctly hyperintense compared with the vitreous. (B) On T2-weighted image,
the tumor is almost indistinguishable from the vitreous.
Circumscribed choroidal hemangiomaMashayekhi and Shields 147

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-Erfurthet 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 of extrafoveal hemangiomas.
Photodynamic therapy is an effective new modality and can be
used for both foveal and extrafoveal hemangiomas. The long-term results of this treatment modality are not yet known.
Radiotherapeutic methods should be considered for cases that
have failed previous treatment or are not good candidates for other treatment modalities because of subfoveal location or extensive subretinal 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 Cfinal)
Thickness, mm (initialCfinal) SRF (initialCfinal) Rx parameters
#Rx sessions
FU,
mo.
Barbazetto, et al
33
Case 1 Extrafoveal 20/40 C20/20 3.3 CFlat Yes CAbsorbed 100 J/cm
2
212
Case 2 Subfoveal 20/120 C20/50 4.6 CFlat Yes CAbsorbed One or more
overlapping spots
49
Madreperla
34
Case 1 Juxtafoveal 20/50 C20/25 2.4 C1.2 Yes CAbsorbed 50 J/cm
2
single spot
13
Case 2 Extrafoveal,
Juxtapapillary
20/70C20/20 2.0 CFlat Yes CAbsorbed 1 9
Case 3 Juxtafoveal 20/50 C20/40 2.8 CFlat Yes CAbsorbed 1 4
Robertson
35
Case 1 Extrafoveal,
Juxtapapillary
20/50C20/20 2.4 CFlat Yes CAbsorbed 50 J/cm
2
3to6 overlapping spots
214
Case 2 Extrafoveal,
Juxtapapillary
20/150C20/20 2.9 CFlat Yes CAbsorbed 1 13
Case 3 Subfoveal,
Juxtapapillary
3/200C20/200 3.9 CFlat Yes CAbsorbed 2 11
Sheidow, et al
36
Case 1 Subfoveal 20/400 C20/50 3.8 CFlat Yes CAbsorbed 50 J/cm
2
single spot
212
Schmidt-Erfurth, et al
37
15 cases* Juxtapapillary
12 cases
Subfoveal
3 cases
20/125C20/80* 3.8* CFlat Yes CAbsorbed 100 J/cm
2
single spot
1–412 –50
*The characteristics of individual cases were not described in this report. Figures represent mean values.
148Retina and vitreous disorders

with longer follow-up periods are necessary to answer
these questions.
Conclusions
Circumscribed 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
1Shields JA, Shields CL: Atlas of Intraocular Tumors. Philadelphia: Lippincott
Williams & Wilkins; 1999:170–179.

2Shields CL, Honavar SG, Shields JA, et al.: Circumscribed choroidal heman-
gioma: clinical manifestations and factors predictive of visual outcomein 200
consecutive cases. Ophthalmology 2001, 108:2237–2248.
The authors reviewed the clinical and ancillary testing characteristicsof 200 cases
of circumscribed choroidal hemangioma and determined factors predictive of poor
visual outcome.
3Witschel H, Font RL: Hemangioma of the choroid. A clinicopathologic study
of 71 cases and a review of the literature. Surv Ophthalmol 1976, 20:415–
431.
4Anand R, Augsburger JJ, Shields JA: Circumscribed choroidal hemangiomas.
Arch Ophthalmol 1989, 107:1338–1342.
5Shields CL, Shields JA, Gross NE, et al.: Survey of 520 uveal metastases.
Ophthalmology 1997, 104:1265–1276.
6Scott 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.
7Cheung D, Grey R, Renni I: Circumscribed choroidal haemangioma in a pa-
tient with Sturge-Weber syndrome [letter]. Eye 2000, 14:238–240.

8Cohen 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 hemangioma
during and following delivery is described.
9Medlock RD, Augsburger JJ, Wilkinson CP, et al.: Enlargement of circum-
scribed choroidal hemangiomas. Retina 1991, 11:385–388.
10Shields JA, Stephens RF, Eagle RC Jr, et al.: Progressive enlargement of a
circumscribed choroidal hemangioma. A clinicopathologic correlation.Arch
Ophthalmol 1992, 110:1276–1278.
11Pitta C, Bergen R, Littwin S: Spontaneous regression of a choroidal heman-
gioma following pregnancy. Ann Ophthalmol 1979, 11:772–774.
12Shields CL: Diagnostic and therapeutic challenges. Retina 1999, 19:69–74.
13Verbeek 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.
14Shields CL, Shields JA, De Potter P: Patterns of indocyanine green videoan-
giography of choroidal tumors. Br J Ophthalmol 1995, 79:237–245.
15Arevalo JF, Shields CL, Shields JA, et al.: Circumscribed choroidal hemangi-
oma: characteristic features with indocyanine green videoangiography.Oph-
thalmology 2000, 107:344–350.
16Stroszczynski C, Hosten N, Bornfeld N, et al.: Choroidal hemangioma: MR
findings and differentiation from uveal melanoma. AJNR Am J Neuroradiol
1998, 19:1441–1447.
17Fuchs AV, Mueller AJ, Grueterich M, et al.: Transpupillary thermotherapy
(TTT) in circumscribed choroidal hemangioma. Graefes Arch Clin Exp Oph-
thalmol 2002, 240:7–11.
18Schilling H, Sauerwein W, Lommatzsch A, et al.: Long-term results after low
dose ocular irradiation for choroidal haemangiomas. Br J Ophthalmol 1997,
81:267–273.
19Sanborn GE, Augsburger JJ, Shields JA: Treatment of circumscribed choroi-
dal hemangiomas. Ophthalmology 1982, 89:1374–1380.
20Othmane IS, Shields CL, Shields JA, et al.: Circumscribed choroidal heman-
gioma managed by transpupillary thermotherapy. Arch Ophthalmol 1999,
117:136–137.
21Rapizzi E, Grizzard WS, Capone A Jr: Transpupillary thermotherapy in the
management of circumscribed choroidal hemangioma. Am J Ophthalmol
1999, 127:481–482.
22Garcia-Arumi J, Ramsay LS, Guraya BC: Transpupillary thermotherapy for
circumscribed choroidal hemangiomas. Ophthalmology 2000, 107:351–
356.
23Mosci C, Polizzi A, Zingirian M: Transpupillary thermotherapy for circum-
scribed choroidal hemangiomas: first choice in therapy. Eur J Ophthalmol
2001, 11:316–318.
24Zografos L, Bercher L, Chamot L, et al.: Cobalt-60 treatment of choroidal
hemangiomas. Am J Ophthalmol 1996, 121:190–199.
25Madreperla SA, Hungerford JL, Plowman PN, et al.: Choroidal hemangiomas:
visual and anatomic results of treatment by photocoagulation or radiation
therapy. Ophthalmology 1997, 104:1773–1778.
26Chao AN, Shields CL, Shields JA, et al.: Plaque radiotherapy for choroidal
hemangioma with total retinal detachment and iris neovascularization. Retina
2001, 21:682–684.
27Ritland JS, Eide N, Tausjo J: External beam irradiation therapy for choroidal
haemangiomas. Visual and anatomic results after a dose of 20 to 25 Gy. Acta
Ophthalmol Scand 2001, 79:184–186.
28Hannouche D, Frau E, Desjardins L, et al.: Efficacy of proton therapy in cir-
cumscribed choroidal hemangiomas associated with serious retinal detach-
ment. Ophthalmology 1997, 104:1780–1784.
29Lee V, Hungerford JL: Proton beam therapy for posterior pole circumscribed
choroidal haemangioma. Eye 1998, 12:925–928.
30Zografos L, Egger E, Bercher L, et al.: Proton beam irradiation of choroidal
hemangiomas. Am J Ophthalmol 1998, 126:261–268.
31Schmidt-Erfurth U, Bauman W, Gragoudas E, et al.: Photodynamic therapy of
experimental choroidal melanoma using lipoprotein-delivered benzoporphy-
rin. Ophthalmology 1994, 101:89–99.
32Schmidt-Erfurth U, Hasan T, Gragoudas E, et al.: Vascular targeting in pho-
todynamic occlusion of subretinal vessels. Ophthalmology 1994, 101:1953–
1961.
33Barbazetto I, Schmidt-Erfurth: Photodynamic therapy of choroidal hemangi-
oma: two case reports. Graefes Arch Clin Exp Ophthalmol 2000, 238:214–
221.

34Madreperla SA: Choroidal hemangioma treated with photodynamic therapy
using verteporfin. Arch Ophthalmol 2001, 119:1606–1610.
Photodynamic therapy was successful in eliminating subretinal fluid andimproving
vision in three patients with circumscribed choroidal hemangioma.

35Robertson 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 detachment
resolved with improvement of visual acuity. Nerve fiber bundle defects were not
identified.
36Sheidow TG, Harbour JW: Photodynamic therapy for circumscribed choroi-
dal hemangioma. Can J Ophthalmol 2002, 37:314–317.

37Schmidt-Erfurth UM, Michels S, Kusserow C, et al.: Photodynamic therapy for
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).
38Schmidt-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.
Circumscribed choroidal hemangiomaMashayekhi and Shields 149