Bilateral circumscribed choroidal hemangioma with retinal and choroidal venous abnormalities

Abstract

Choroidal hemangioma is a rare, benign vascular hamartoma that can be circumscribed or diffuse. The circumscribed type is usually not associated with cutaneous or systemic abnormalities, whereas the diffuse type is associated with encephalofacial angiomatosis, e.g, SturgeWeber (SW) syndrome or Klippel-Trenaunay-Weber (KTW) syndrome [1,2]. Choroidal hemangioma is almost always unilateral. We present a case of bilateral circumscribed hemangioma of the choroid with retinal and choroidal vascular abnormalities. Case reportA 72-year-old man had progressive loss of vision in his left eye. He had had hypertension, chronic heart failure, and chronic renal failure for over 20 years. Systemic evaluations did not identify any cutaneous or orthopedic abnormalities.His best-corrected visual acuity was 0.9 OD and 0.5 OS, and the refractive degree (spherical equivalent) was ?1.0 diopters in both eyes. Ophthalmoscopy showed an engorgement of the retinal vein in the left fundus and a reddish subretinal macular mass in both eyes (Fig. 1). A hyperfluorescent mass was observed in the left fundus in the late phase of fluorescein angiography (FA; Fig. 1e). The area of the subretinal mass observed ophthalmoscopically in the right eye showed irregular hyperfluorescence (Fig. 1c). Indocyanine green angiography (ICGA) showed that the mass was intensely hyperfluorescent in both eyes. A relative decrease in fluorescence in the late ICG angiogram (''washout'' of the dye) was not obvious for up to 7 min. The choroidal veins exited nasal to the optic disc in both eyes (Fig. 2b, d). Optical coherence tomography (OCT) showed an elevation of the retinal pigment epithelium (Fig. 2, arrow) overlying the tumor. B-scan ultrasonography and computed tomography did not show any areas of high reflectivity suggestive of a choroidal osteoma. CommentsFrom the ICGA findings and its restricted location, we considered the mass in both eyes to be a circumscribed choroidal hemangioma, although the subretinal mass in the right fundus was tiny. Our patient did not have any cutaneous or osseous complications suggesting the SW or KTW syndromes. A choroidal hemangioma is almost always ipsilateral, irrespective of whether it is diffuse or circumscribed. Lindsey and colleagues [3] reported two cases of bilateral choroidal hemangiomas and facial nevus flammeus, in which the hemangioma was diffuse and involved over half of the choroid. A PubMed search did not extract any cases of bilateral circumscribed choroidal hemangioma. However, without ICGA, the small lesion in the right eye might have been missed, suggesting that bilateral cases may have been diagnosed as unilateral circumscribed hemangioma of the choroid.