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Circumscribed choroidal hemangiomaArman Mashayekhi, MD, and Carol L. Shields, MDCircumscribed choroidal hemangioma is an uncommon,Backgroundbenign vascular tumor manifesting as an orange-red mass inChoroidal hemangioma is a benign, vascular, hamartoma-the posterior pole of the eye. Serous retinal detachmenttous tumor occurring in two distinct forms, circumscribedaccounts for decreased vision in most patients. Diagnosis ofand diffuse, on the basis of the extent of choroidal in-this tumor is challenging with many patients initiallyvolvement. Circumscribed choroidal hemangioma is gen-misdiagnosed with choroidal melanoma or metastasis. Severalerally a solitary finding without systemic associations,ancillary tests such as ultrasonography, fluoresceinwhereas diffuse choroidal hemangioma usually occurs inangiography, indocyanine green angiography, and magneticassociation with encephalofacial angiomatosis (Sturge-resonance imaging help differentiate this tumor from otherWeber syndrome) .simulating lesions. Asymptomatic lesions should be observed,but visually threatening or visually impairing lesions requireThe diagnosis and management of circumscribed choroi-treatment. Photodynamic therapy, laser photocoagulation, anddal hemangioma continue to be a challenge for mosttranspupillary thermotherapy may be used for primaryclinicians. Many patients with choroidal hemangioma aremanagement of this tumor. Patients who fail to respond toreferred to us because of suspected choroidal melanomaprevious treatment or those with extensive serous retinalor choroidal metastasis. Therefore, it is important todetachment can be treated using radiotherapeutic modalities.achieve an accurate diagnosis of this tumor, reassure pa-Long interval between onset of symptoms and treatment, poortients of its benign nature, and provide proper therapy.visual acuity at presentation, and presence of chronic retinal orretinal pigment epithelial changes are associated with poorDiagnosislong-term vision. Curr Opin in Ophthalmol 2003, 14:142–149 © 2003Clinical manifestationsLippincott Williams & Wilkins.Circumscribed choroidal hemangioma is a relatively raretumor. Between 1974 and 2000, 200 patients with cir-cumscribed choroidal hemangioma were diagnosed andtreated 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.), theMacula Foundation, New York, NY (C.L.S.), and the Rosenthal Award of theAlthough probably congenital, most patients do not de-Macula Society (C.L.S.).velop symptoms until they are in their fourth to sixthCorrespondence to Carol L. Shields, MD, Ocular Oncology Service, Wills Eyedecade of life. In the series reported from the OncologyHospital, 840 Walnut Street, Philadelphia, PA 19107, USAService at Wills Eye Hospital, the mean age at presen-Current Opinion in Ophthalmology 2003, 14:142–149tation was 47 years with a range of 4 to 81years [2••].AbbreviationsMost patients present with decreased visual acuity.PDTphotodynamic therapyOther less common symptoms include visual field de-RPEretinal pigment epitheliumfect, metamorphopsia, flashes and floaters, and progres-ISSN 1040–8738 © 2003 Lippincott Williams & Wilkinssive hypermetropia [2••].Circumscribed choroidal hemangioma usually appears asa 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 pigmentepithelium (RPE), often surrounds the tumor. Almost allcases occur posterior to the equator, usually near andtemporal to the optic disc . Clumps of pigment, prob-ably secondary to hyperplasia of overlying RPE, are notuncommon [2••]. Retinal or subretinal exudation is not acommon feature of choroidal hemangiomas and was re-ported in only 7% of patients in one series . Mushroomappearance is exceedingly rare [2••].142Circumscribed choroidal hemangioma Mashayekhi and Shields143Figure 1. Clinical appearance of circumscribed choroidalthe Oncology Service at Wills Eye Hospital, the clinicalhemangiomadiagnosis of choroidal hemangioma was accurately sus-pected before referral for only 29% of the patients, and14% were referred without any specific diagnosis (Table1) [2••]. Several reasons may account for this situation:(1)The ophthalmoscopic appearance of choroidal hem-angioma may be almost indistinguishable from thenormal 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 ofretinal detachment, central serous chorioretinopa-thy, or macular edema.(3)The funduscopic appearance of circumscribed cho-roidal hemangioma may simulate other benign ormalignant conditions of the fundus. In the seriesfrom 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 differentiatechoroidal hemangiomas from simulating lesions. Choroi-dal hemangiomas have a distinctive orange-red colorsimilar to the surrounding choroid, whereas amelanoticmelanomas are a more yellow-tan color, often with subtleintrinsic pigment and visible overlying drusen. Clinicallyevident drusen are rare overlying choroidal hemangiomaand were detected in only 2% of patients in one series[2••]. In contrast to choroidal melanomas, choroidalhemangiomas almost never attain a mushroom-shapedappearance [2••]. Choroidal metastasis appears as acreamy yellow plateau or elevated mass and in contrast tochoroidal hemangioma, which is almost always solitaryand unilateral, may commonly be multifocal or bilateral.However, three specific choroidal metastases—fromrenal cell carcinoma, thyroid carcinoma, and carcinoid(A) Wide-angle fundus photograph of circumscribed choroidal hemangioma withTable 1. Referral diagnosis in 200 consecutive patients withassociated subretinal fluid. (B) Wide-angle fundus photograph of circumscribedcircumscribed choroidal hemangioma referred to the Oncologychoroidal hemangioma with minimal associated subretinal fluid and overlyingService, Wills Eye Hospital [2••]retinal pigment epithelial alterations.Referral diagnosisNumber (%)Choroidal hemangioma58 (29)This benign tumor can cause visual impairment by vari-Choroidal melanoma58 (29)Choroidal metastasis17 (9)ous mechanisms, such as exudative retinal detachment,Retinal detachment12 (6)overlying photoreceptor degeneration, elevation or tilt-Central serous chorioretinopathy9 (5)ing of the macular region, cystoid macular edema, sub-Macular edema5 (3)Others13 (9)retinal fibrosis, RPE alterations, and retinoschisis [1,2••].No diagnosis28 (14)Differential diagnosisAdapted from: Shields CL, Honavar SG, Shields JA, Cater J, DemirciH. Circumscribed choroidal hemangioma: clinical manifestations andThe diagnosis of circumscribed choroidal hemangiomafactors predictive of visual outcome in 200 consecutive cases.can be challenging. Of the 200 patients in the series fromOphthalmology 2001; 108:2237–48.144Retina and vitreous disorderstumor—can appear orange, similar to choroidal heman-Table 2. Characteristic features of circumscribed choroidalgioma [1,5].hemangioma on ancillary testingUltrasonographyIt is generally believed that circumscribed choroidalA-scan: High internal reflectivityB-scan: Acoustically solid (similar to normal choroid)hemangioma is not associated with Sturge-Weber syn-Fluorescein angiographydrome, but in the Wills Eye Hospital series, there wereEarly: Mild, lacy hyperfluorescencefour patients with typical circumscribed choroidal hem-Late: Intense, diffuse hyperfluorescenceIndocyanine green angiographyangioma who had a facial nevus flammeus and otherEarly: Hyperfluorescencemanifestations of Sturge-Weber syndrome [2••]. TheLate: Dye “washout”findings in the choroid may have been a limited form ofMRIBright signal on both T1- and T2-weighted imagesthis syndrome because others have also observed thisassociation [6,7,8•]. Five additional patients showed sys-temic mucosal or remote cutaneous hemangiomas, andsic vascular pulsations; however, similar ultrasonographicone patient had neurofibromatosis [2••].features may be seen with choroidal metastasis .Fluorescein angiography of choroidal hemangioma typi-Natural coursecally shows lacy hyperfluorescence in the prearterial orMost cases of circumscribed choroidal hemangioma areearly arterial phase and diffuse, intense, late hyperfluo-stationary, but several authors have reported spontane-rescence (Fig. 3). Fluorescein angiographic findings,ous enlargement of this lesion [9,10]. Enlargement ofchoroidal hemangioma is secondary to venous congestionFigure 2. Ultrasonographic appearance of circumscribedrather than cellular multiplication . We are aware ofchoroidal hemangiomathree reports of circumscribed choroidal hemangiomaspresenting with exudative retinal detachment and de-creased vision during the second or third trimesters ofpregnancy [8•,11,12]. Spontaneous reabsorption of sub-retinal fluid can occur in some patients following deliv-ery [8•,11].PathologyIn 1976, Witschel and Font  described the clinicopath-ologic features of 45 eyes with circumscribed choroidalhemangioma that came to enucleation and were evalu-ated at the Armed Forces Institute of Pathology. Theydescribed solitary hemangioma as a circumscribed tumorwith a sharply demarcated margin, causing compressionof 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 fibroustransformation or ossification. Almost all lesions showeddegenerative changes of the overlying retina, includingloss of photoreceptors, cystoid degeneration, gliosis, andinvasion by RPE cells.Ancillary studiesChoroidal hemangioma shows characteristic features onultrasonography, fluorescein angiography, indocyaninegreen angiography, and magnetic resonance imaging(Table 2). With ultrasonography, the hemangioma isacoustically solid on B-scan, and the echogenic characteris 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 ofThese features would be unlikely with choroidal mela-circumscribed choroidal hemangioma. (B) On B-scan ultrasonography,noma because melanoma usually displays acoustic hol-circumscribed choroidal hemangioma has a solid acoustic appearance similar tothe adjacent choroid.lowness, low to medium internal reflectivity, and intrin-Circumscribed choroidal hemangioma Mashayekhi and Shields145Figure 3. Fluorescein angiographic appearance ofother retinal changes associated with circumscribed cho-circumscribed choroidal hemangioma.roidal hemangioma (Fig. 6). Marked attenuation of thelight signal occurs after passing through the neurosensoryretina and RPE, limiting the usefulness of this techniquefor direct evaluation of choroidal tumors.TreatmentThe goal for management of choroidal hemangioma ispreservation or improvement of visual acuity by stimu-lating absorption of subretinal fluid and resolution ofmacular edema before irreversible retinal or RPE alter-ations have occurred. Re-treatment of residual extrafo-veal tumors without subretinal fluid is not necessary. It isnot yet clear whether incomplete flattening of hemangi-omas predisposes to later recurrence of subretinal fluid.A secondary goal, in more advanced cases, is preventionof neovascular glaucoma from long-standing, extensive,secondary retinal detachment .Long delay between onset of symptoms and onset oftreatment may be associated with a worse visual outcome[2••,17,18]. In the Wills Eye Hospital series, poor finalvisual acuity ( 20/200) was more common in patientstreated after 6 months of symptoms (72%) comparedwith those treated within 6 months of symptoms (42%)[2••]. In the same study, initial visual acuity was foundto be a good predictor of visual outcome [2••], and pa-tients with poor visual acuity at presentation, especiallyif 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 beenidentified 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 withparafoveal tumors, and in 38% of patients with an extra-foveal tumor .(A) Lacy appearance of choroidal hemangioma in arterial phase. (B) Late, diffuseManagement of choroidal hemangioma is based on tu-hyperfluorescence of choroidal hemangioma.mor size, location, and related ocular symptoms. Asymp-tomatic circumscribed choroidal hemangiomas only needalthough characteristic, are not pathognomonic of thisto be observed, and treatment is generally reserved fortumor [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 atrescence, often followed by a characteristic “dye wash-Wills Eye Hospital, 43% of patients were treated without” in late frames (Fig. 4) [14,15]. These features wouldobservation alone [2••]. These patients generallybe unlikely with choroidal melanoma or metastasis, forshowed minimal findings with little or no subretinal fluidwhich filling on fluorescein angiography and indocyanineor fluid-related visual disturbances, or, conversely, theygreen angiography is slower and less intense. With mag-showed advanced findings with chronic macular edemanetic resonance imaging, choroidal hemangioma showsso great that treatment would have been of little visualbright 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 signalary to subfoveal tumors.on T2-weighted images (Table 2) .Initially xenon arc photocoagulation and later argon laserOptical coherence tomography is a useful method forphotocoagulation were the most important treatmentdetection of minimal amounts of subretinal fluid andmodalities [2••,4,19]. More recently, transpupillary146Retina and vitreous disordersFigure 4. Indocyanine green angiographic appearance of circumscribed choroidal hemangioma.(A) Reticular hyperfluorescence is visible 20 seconds after injection. (B) Diffuse,intense hyperfluorescence 1 minute after injection. (C) Washout of dye 20minutes after injection; note the surrounding annular hyperfluorescence.thermotherapy [17,20–23], plaque radiotherapy [24–26],angiomas. Recently, Fuchs et al.  have reported theirexternal beam radiotherapy [2••,18,25,27], proton beamresults of treatment of 10 patients with circumscribedradiotherapy [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 becausethe hemangioma could not be treated completely due toLaser photocoagulation has been used extensively forproximity to the fovea. Visual acuity improved in 4 pa-management of circumscribed choroidal hemangiomastients who had symptoms for less than 12 months, while[2••,4,19]. While success rates as high as 100% havevisual acuity was unchanged in patients who had symp-been reported , others have reported recurrent sub-toms for more than 1year.retinal fluid in more than 50% of treated patients . Inthe series reported from Wills Eye Hospital, argon laserPlaque radiotherapy has been reported to offer excellentphotocoagulation was successful in completely resolvingresults with 100% success in resolution of subretinal fluidsubretinal fluid in 62% of patients [2••]. In general, if[24–26]. The disadvantages of plaque radiotherapy arethe subretinal fluid does not respond appropriately tothat it requires two operative procedures for insertionone or two sessions of surface and delimiting argon laserand removal of the plaque with several days of hospital-photocoagulation, other treatment modalities shouldization and may theoretically cause radiation-inducedbe employed.complications, such as cataract, retinopathy, and papil-lopathy. This modality should be considered for patientsIn several small series, transpupillary thermotherapy haswho have failed to respond to previous treatment (eg,been reported to cause resolution of choroidal hemangi-laser photocoagulation) or who are not good candidatesoma-related subretinal fluid, both as a primary treatmentfor other treatment modalities because of subfoveal lo-and following failed prior laser photocoagulation [17,20–cation or extensive subretinal fluid. Chao et al.  have23]. This method can occasionally be visually destructivereported a case of circumscribed choroidal hemangiomaand cause further visual field and acuity loss; therefore, itin the macular region with total secondary retinal detach-should not be used for management of subfoveal hem-ment and iris neovascularization that was successfullyCircumscribed choroidal hemangioma Mashayekhi and Shields147Figure 5. Magnetic resonance imaging of circumscribedRitland et al.  published the results of nine eyeschoroidal hemangioma.treated with fractionated external beam irradiation. Alleyes responded favorably with regression in tumor thick-ness, resolution of subretinal fluid, and improvement ofvisual acuity. No radiation side effects were noted duringthe follow-up period (range, 0.4–8.8 years). Similar goodresults have been obtained with proton beam radio-therapy, providing resolution of subretinal fluid in 67 to100% of patients [28–30]. It has been claimed that protonbeam-induced papillopathy and maculopathy can beavoided if a low dose of 18 Gy is delivered [29,30].Photodynamic therapy is the most recent modality usedfor 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 . In contrast to laserphotocoagulation and transpupillary thermotherapy, thesuccess of PDT does not depend on a thermal effect,allowing a selective occlusion of vascular lesions withminimal damage to the adjacent retina .During the past 2 years, 5 small case series have been(A) On T1-weighted image with gadolinium enhancement, the hemangioma ispublished on circumscribed choroidal hemangiomadistinctly hyperintense compared with the vitreous. (B) On T2-weighted image,managed with photodynamic therapy (Table 4)the tumor is almost indistinguishable from the vitreous.[33,34•,35•,36, 37••]. All treated patients have shown anexcellent 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, retinalvascular glaucoma and avoiding the need for enucleation.nonperfusion, or visual fields defects. Following treat-ment, some investigators noted RPE alterations at theOther radiotherapeutic methods, such as external beamsite of the original tumor [37••]. Persistent, focal choroi-radiotherapy [2••,18,25,27] and proton beam radio-dal ischemia and atrophy were reported after treatmenttherapy [28–30], have been reported to successfully man-of prominent lesions with three or more sessions of PDTage circumscribed choroidal hemangiomas. Recently,[37••]. There was no recurrence of tumor or subretinalFigure 6. Optical coherence tomography of circumscribed choroidal hemangiomaOptical coherence tomography of the circumscribedchoroidal hemangioma shown in Figure 1A reveals thepresence of subretinal fluid adjacent to the foveola.148Retina and vitreous disordersTable 3. Points regarding treatment of circumscribedafter treatment, visual acuity increased to 20/200 [35•].choroidal hemangiomaOne of the patients reported by Barbazetto  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 beproving to 20/50 following treatment. Although indi-associated with a considerable recurrence rate.• Both laser photocoagulation and transpupillary thermotherapy canvidual visual acuity results have not been provided,be visually destructive and should be limited to treatment ofSchmidt-Erfurth et al. [37••] did not detect any residualextrafoveal hemangiomas.field defects in three eyes with subfoveal hemangiomas,• Photodynamic therapy is an effective new modality and can beused for both foveal and extrafoveal hemangiomas. The long-term“other than the scotoma related to the extrafoveal arearesults of this treatment modality are not yet known.showing choroidal atrophy because of overtreatment.”• Radiotherapeutic methods should be considered for cases thatObviously, it is important to avoid overtreatment in PDThave failed previous treatment or are not good candidates for othertreatment modalities because of subfoveal location or extensiveof subfoveal hemangiomas. We have treated 10 patientssubretinal fluid.with circumscribed choroidal hemangioma with single-spot, single-session PDT thus far, and subretinal fluidfluid during follow-up periods of 3 to 50 months. Al-has resolved and vision improved in each patient. Inthough there has been no report of optic nerve damage inTable 4, it becomes evident that patients with subfovealtreated eyes with juxtapapillary choroidal hemangiomas,hemangiomas generally presented with lower visual acu-it is important to avoid directing the laser beam at theities and ended with lower visual acuities compared withoptic disc because optic nerve ischemia has been reportedthose with extrafoveal tumors.after PDT of papillary capillary hemangiomas .Despite initial good results, many questions are still un-Photodynamic therapy has been successfully employedanswered regarding the role of PDT in management offor management of subfoveal choroidal hemangiomascircumscribed choroidal hemangiomas. These include[33,35•,36,37••]. Sheidow and Harbour treated a patientcriteria for case selection (which lesions and when towith subfoveal choroidal hemangioma and visual acuitytreat), physical parameters of treatment (power, duration,of 20/400. Following treatment, visual acuity improved tospot size, and number of spots used in one session), num-20/50 with minimal pigment epithelial changes over theber of sessions, interval between sessions (treatment in-tumor . One of the patients reported by Robertsontervals), endpoint of treatment (resolution of leakage andhad a subfoveal choroidal hemangioma with cystoidsubretinal fluid; complete flattening of tumor), and long-macular edema and visual acuity of 3/200. Two monthsterm recurrence rate and complications. Larger studiesTable 4. Clinical features, treatment parameters, and outcome to treatment of 24 published cases ofcircumscribed choroidal hemangioma treated by PDTThickness, mm# RxFU,AuthorLocationVA (initialfinal)(initialfinal)SRF (initialfinal)Rx parameterssessionsmo.Barbazetto, et al33Case 1Extrafoveal20/4020/203.3FlatYesAbsorbed100 J/cm2212Case 2Subfoveal20/12020/504.6FlatYesAbsorbedOne or more49overlappingspotsMadreperla34Case 1Juxtafoveal20/5020/252.41.2YesAbsorbed50 J/cm213single spotCase 2Extrafoveal,20/7020/202.0FlatYesAbsorbed19JuxtapapillaryCase 3Juxtafoveal20/5020/402.8FlatYesAbsorbed14Robertson35Case 1Extrafoveal,20/5020/202.4FlatYesAbsorbed50 J/cm2214Juxtapapillary3 to 6overlappingspotsCase 2Extrafoveal,20/15020/202.9FlatYesAbsorbed113JuxtapapillaryCase 3Subfoveal,3/20020/2003.9FlatYesAbsorbed211JuxtapapillarySheidow, et al36Case 1Subfoveal20/40020/503.8FlatYesAbsorbed50 J/cm2212single spotSchmidt-Erfurth, et al3715 cases*Juxtapapillary20/12520/80*3.8*FlatYesAbsorbed100 J/cm21–412–5012 casessingle spotSubfoveal3 cases*The characteristics of individual cases were not described in this report. Figures represent mean values.Circumscribed choroidal hemangioma Mashayekhi and Shields149with longer follow-up periods are necessary to answer17Fuchs AV, Mueller AJ, Grueterich M, et al.: Transpupillary thermotherapy(TTT) in circumscribed choroidal hemangioma. Graefes Arch Clin Exp Oph-these questions.thalmol 2002, 240:7–11.18Schilling H, Sauerwein W, Lommatzsch A, et al.: Long-term results after lowConclusionsdose ocular irradiation for choroidal haemangiomas. Br J Ophthalmol 1997,Circumscribed choroidal hemangioma is a benign vascu-81:267–273.lar tumor capable of causing decreased vision secondary19Sanborn GE, Augsburger JJ, Shields JA: Treatment of circumscribed choroi-dal hemangiomas. Ophthalmology 1982, 89:1374–1380.to exudative retinal detachment. Despite its rather char-20Othmane IS, Shields CL, Shields JA, et al.: Circumscribed choroidal heman-acteristic ophthalmoscopic appearance, many patientsgioma managed by transpupillary thermotherapy. 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