Optical coherence tomography findings in macular cavernous haemangioma

Abstract

Editor, Cavernous haemangioma of the retina is an unusual vascular hamartoma with presumed autosomal dominant transmission, characterized by clusters of dark red, intraretinal aneurysms, described as ‘grape-like’ lesions (Alexander & Moates 1988). Usually unilateral and clinically silent, the lesion does not normally increase in size (Kushner et al. 1994); however, macular involvement or vitreous haemorrhage can lead to a decline in vision (Pierro et al. 1992). Herein, we present a rare case of macular cavernous haemangioma as well as its morphological peculiar findings as disclosed by optical coherence tomography (OCT). A 62-year-old woman presented with a progressive decrease in vision in the left eye that had been going on for 7 years. Best corrected visual acuity (VA) was 20/20 in the right eye and counting fingers at 1 metre in the left eye. Fundus examination of the left eye revealed an elevated, dark red, macular lesion, with multiple retinal aneurysmatic formations and subretinal as well as intraretinal haemorrhage (Fig. 1). Fundus examination of the right eye was normal. Fluorescein angiography of the left eye showed typical hyperfluorescent dot-like lesions with inferior blockage hypofluorescence known as ‘fluorescence capping’ (Fig. 2) (Messmer et al. 1983). Optical coherence tomography disclosed intraretinal cysts and highly reflective signals from a thickened macula (Fig. 3). At follow-up 2 years later, no changes were seen in this case of macular cavernous haemangioma. Colour fundus photography shows an irregularly elevated vascular mass in the macular region composed of dilated, oval or rounded, thin-walled, saccular blood vessels giving the appearance of a mass of grapes, with associated intraretinal and subretinal haemorrhages. Fluorescein angiography demonstrates multiple hyperfluorescent dots corresponding to aneurysmatic formations and late dye leakage. A typical finding known as ‘fluorescence capping’, characterized by delayed and incomplete perfusion, with progressive filling of the dye in the upper part (arrows) and blocked hypofluorescence in the lower part (arrowheads), is seen in larger aneurysmatic formations. A linear, 5.92-mm OCT scan, centred on the fovea, discloses retinal elevation caused by the tumoral tissue. Optically clear cystic spaces and high reflective signals are seen arising particularly from the inner part of the thickened neurosensory retina. Underneath the lesion, few reflective signals from the retinal pigment epithelium and choroid are observed. No tomographic signals suggesting fluid accumulation are seen in the vicinity of the lesion. Skin and cerebral anomalies have been reported to eventually occur in patients with cavernous haemangioma of the retina. Therefore, an appropriate medical evaluation should be strongly considered in such patients. Family members should also be examined as a dominant inheritance has been suggested (Schwartz et al. 1984; Dellemijn & Vanneste 1993). In our case, no systemic finding was found after clinical and neurological comprehensive evaluation and no other family member was found for evaluation. In respect to lesion location, the optic disc is the most common locality for the tumour and generally no decrease in vision is noted; however, as in the case presented here, decreased VA may occur due to direct macular involvement (Naftchi & la Cour 2002) or, particularly, as secondary to vitreous haemorrhage (Messmer et al. 1983). Optical coherence tomography was useful to reveal low reflectivity vesicular formations and focal regions of highly reflective signals, most likely corresponding to the red blood filled ‘cysts’ that, at least in part, lie in the plane of the inner retinal layers; additionally, no evidence of perilesional exudation was seen. These findings are consistent with a previous histopathological description of the entity, in which the tumour was composed of telangiectatic retinal vessels displaying anatomical features normally encountered in retinal vessels and mostly occupying the inner half of the retina (Messmer et al. 1984).