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Bilateral circumscribed haemangioma of the choroid not associated with systemic vascular syndrome
  1. Department of Ophthalmology, University of Ferrara, Ferrara, Italy
  1. Paolo Perri, MD, Sezione di Clinica Oculistica, Dipartimento di Discipline Medico-Chirurgiche della Comunicazione e del Comportamento, Università degli Studi di Ferrara, Corso Giovecca 203, 44100 Ferrara, Italy sbd{at}   Accepted for publication 5 March 2001

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Editor,—Circumscribed choroidal haemangioma (CCH) is considered congenital, vascular, relatively rare hamartoma which typically occurs as a localised, monolateral lesion in patients without other vascular malformation. This tumour generally is discovered in adulthood and it is located in the macular area. CCH may be ophthalmoscopically confused with amelanotic melanoma, metastatic tumour, choroidal osteoma, disciform scar, serous detachment, and central serous chorioretinopathy, but may be differentially diagnosed with fluorescein angiography (FA), indocyanine green angiography (ICGA),1-3 ultrasonography, and periodic observation.4 The bilateral CCH localisation represents an extremely uncommon condition which, in literature, has been only reported in association with Sturge-Weber syndrome5 6 or Klippel-Trenaunay-Weber syndrome.7 To the best of our knowledge, this is the first documented case of bilateral CCHs in the absence of any other evidence of vascular systemic abnormalities.


A 81 year old white man was referred to our institution in June 1999 to undergo conservative therapy because of malignant choroidal melanoma of the left eye. He reported a 6 month history of bilateral, progressive reduction of the central vision, greater in his left eye. His best corrected visual acuity was 20/30 in the right eye and 20/40 in the left. Biomicroscopy of the anterior segment did not reveal any notable alterations with the exception of a bilateral nuclear cataract, more evident in the left eye. Intraocular pressure was 18 mm Hg in both eyes. Ophthalmoscopic examination of the left temporal posterior pole showed a lesion, about five optic disc diameters in size and red-orange in colour (Fig 1B), while, in the right macular area, an irregular appearance of the retinal surface was detected (Fig 1A). Bilateral B-scan echography confirmed the presence of a dome-shaped solid lesion, with regular profile and without choroidal cup, in the left eye, revealing a small solid lesion also in the right posterior choroid. Standardised A-scan ultrasonography documented that the maximum thickness of these solid lesions was 1.56 mm in the right eye (Fig 2A) and 3.32 mm in the left (Fig 3A). In the left eye the high and regular internal reflectivity of the lesion was consistent with the presence of a benign tumour, reliably of an angiomatous type. FA did not detail any significant abnormality in the right posterior pole (Fig 2B), showing an irregular fluorescence of the orange-coloured lesion previously described in the left eye (Fig 3B). ICGA confirmed the diagnosis of CCH of the left eye (Fig 3C, D) and documented an early hyperfluorescence, followed by a relative decrease in fluorescence (“washout”), corresponding to the echographic findings observed in the right macula (Fig 2C, D). The patient underwent chest xray, abdominal and chest computed tomographies, total body scintigraphy, liver ultrasonography, blood, and urine analyses. These investigations did not show any abnormality, reliably excluding the possible metastatic origin of the bilateral choroidal lesions. In the course of a 15 month follow up period, we periodically reassessed this patient, and did not diagnose any ocular or systemic modification.

Figure 1

(A) Photograph of the right macular area reveals an irregular appearance of the retinal surface. (B) Photograph of the left temporal posterior pole shows a lesion, about 5 optic disc diameters in size and red-orange in colour.

Figure 2

(Right eye). (A) Standardised A-scan ultrasonography at 1660 m/s shows the high internal reflectivity of the choroidal solid lesion at the level of the macular area. The maximum thickness of this small circumscribed lesion is 1.56 mm. (B) Late phase fluorescein angiogram reveals a hyperfluorescent area inferiorly located at the inferior posterior pole, secondary to a degenerative change of the retinal pigment epithelium. (C) Early indocyanine green photograph documents the filling of the choroidal macular haemangioma near to a sector of reduced choroidal perfusion (arrows). (D) Late indocyanine green angiogram shows an ill defined, relative macular hypofluorescence, corresponding to the previously described hyperfluorescent area, reliably the result of the clearing of the dye from the small haemangioma (arrows).

Figure 3

(Left eye). (A) Standardised A-scan ultrasonography at 1660 m/s demonstrates the temporal paramacular solid lesion of the choroid, with its high and regular internal reflectivity, consistent with the presence of an angiomatous benign lesion. The maximum thickness of this circumscribed choroidal haemangioma is 3.32 mm. (B) Late phase fluorescein angiogram shows an ill defined hyperfluorescent and hypofluorescent area in correspondence with choroidal haemangioma (arrows). (C) Early indocyanine green photograph reveals a rapid and complete fill up of the lesion. The haemangioma has a “mulberry appearance” at the stage of maximal fluorescence. (D) Late indocyanine green angiogram shows clearing of the dye from the tumour, associated with diffusion into the choroidal and subretinal space (“washout” phenomenon).


Atypical CCH can cause differential diagnostic problems by its appearance at the time of presentation. Moreover, bilateral choroidal localisation of tumoral lesions raises the question about their primary or metastatic onset.8 At our department we observed approximately one haemangioma of the choroid for every 15 malignant melanomas, referred to us yearly for conservative treatment. In spite of this relatively high frequency of haemangioma, this represents the first case in whom we diagnosed a bilateral circumscribed vascular hamartoma, which was not associated with any systemic syndrome. During the mid-term follow up (15 months) there were neither ocular nor systemic significant modifications. The echographic9 and ICGA1-3 10 features of these choroidal lesions, together with the lack of neoplasm or vascular abnormality in another part of the body, lead us to confirm the first documented diagnosis of bilateral CCHs. Last but not least, our findings demonstrate that FA and echography are not always capable of documenting the specific characteristics of small CCH; thus, when this kind of lesion is suspected, ICGA represents the most important non-invasive tool for the diagnosis2 to differentiate amelanotic choroidal melanoma, choroidal metastasis, and choroidal haemangioma.10


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