Circumscribed choroidal hemangioma
Arman Mashayekhi, MD, and Carol L. Shields, MD

Circumscribed choroidal hemangioma is ...
Circumscribed choroidal hemangioma Mashayekhi and Shields 143

Figure 1. Clinical appearance of circumscribed choroidal   ...
144 Retina and vitreous disorders

tumor—can appear orange, similar to choroidal heman-              Table 2. Characterist...
Circumscribed choroidal hemangioma Mashayekhi and Shields 145

Figure 3. Fluorescein angiographic appearance of           ...
146 Retina and vitreous disorders

Figure 4. Indocyanine green angiographic appearance of circumscribed choroidal hemangio...
Circumscribed choroidal hemangioma Mashayekhi and Shields 147

Figure 5. Magnetic resonance imaging of circumscribed      ...
148 Retina and vitreous disorders

Table 3. Points regarding treatment of circumscribed                          after tre...
Circumscribed choroidal hemangioma Mashayekhi and Shields 149

with longer follow-up periods are necessary to answer      ...
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Circumscribed choroidal hemangioma


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Circumscribed choroidal hemangioma

  1. 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. 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. 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. 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. 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. 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. 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. 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. 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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. 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