West Coast Retina
Case of the Month
A 30-year-old woman noted to have a lesion in her left eye.
Presented by Sara Haug, MD, PhD
Figure 1A: Color fundus photograph montage of the left eye. Note the lesion in the far inferior periphery with fibrosis, and aneurysms and some retinal hemorrhage.
Figure 1B: Color fundus photograph of the left eye showing a more detailed view of the lesion.
A 30 year-old woman was referred for evaluation of a lesion in her left eye. Past medical history was significant for Thalassemia Minor. Ocular history was significant for myopia.
On examination, best-corrected visual acuity was 20/32 in the right eye, 20/16 in the left. Intraocular pressures were 12 in both eyes and blood pressure was 88/68. Anterior segment examination was quiet bilaterally with no evidence of cataract. The remainder of the examination of the right eye was normal.
In the left eye, in the far inferior periphery, a collection of retinal aneurysms with some hemorrhage and overlying fibrosis was noted. (Figs 1A and 1B). Fluorescein angiogram revealed slow early vascular filling of many of the aneurysms and some hypofluorescent areas due to incomplete filling and blood.The areas of hyperflourescence persisted into the late phases of the angiogram (Figure 2). No vascular leakage was observed.
Figures 2A and B: Fluorescein angiogram, early and late phase. Not the sluggish filling of many of the aneurysms. Hyperfluorescence persists into the late phases of the study. No vascular leakage is noted.
Figure 3: Montage of the color fundus photograph, early and late fluorescein angiogram. Sluggish flow within the aneurysms is present.
What is your Diagnosis?
This lesion demonstrates a distinct cluster of aneurysms and slow flow. Common causes of peripheral vascular abnormalities such as venous occlusion or retinal telangiectasis can be readily excluded from the differential. Vasoproliferative tumor typically produces peripheral vascular lesions that are frequently inferior in location as seen here. However, the sluggish flow, and grape-like clusters of aneurysms without vascular leakage point us towards the diagnosis of cavernous hemangioma of the retina.
Cavernous hemangiomas are usually sporadic but can also be associated with intracranial cavernous hemangiomas1, therefore further imaging was performed on our patient. MRI of the brain with and without contrast was normal with no findings of intracranial cavernous hemanigoma or arteriovenous malformations.
Cavernous hemangiomas of the retina are rare, congenital hamartomas composed of multiple, thin-walled dilated vascular channels with surface gliosis.2 The lesions are generally asymptomatic unless the macula is involved or there is associated intravitreal bleeding, which is a rare complication thought to be related to vitreous traction. If the vitreous hemorrhage is large and recurrent, vitrectomy can be performed.3,4 Cavernous hemangiomas can arise from all regions of the retina and also from the optic disc.3,4
Classic fluorescein angiogram findings show that the vascular dilations slowly fill with dye in the late venous phase, often with a fluid level from the stagnant erythrocytes. The hyperfluorescence lasts long after normal intravascular fluorescence has faded. Vascular leakage is usually not observed due to an intact endothelium, which distinguishes the lesion from retinal telangiectasias.5
Typically, cavernous hemangiomas do not progress and are not associated with any systemic vascular disease. However, numerous case reports document an autosomal dominant multi-system syndrome that can involve intracranial, spinal, and skin hemangiomas.2,6,7 For this reason, cavernous hemangiomas may be considered one of the phakomatoses.
Take Home Points
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