Retinal Cavernous Hemangioma

This healthy 25YO female presented with an asymptomatic retinal lesion in her right eye. She recalled being diagnosed with a retinal finding in the past and reported negative prior neurologic imaging. Vision was 20/20 bilaterally.

Optos RG color imaging shows extensive confluent variably sized grape-like clusters of aneurysms filled with dark venous blood. Some scattered gray fibrotic membranes cover its superficial surface.

Learning Points:
Retinal cavernous hemangiomas are usually unilateral, benign vascular abnormalities. Fluorescein angiography shows plasma-erythrocyte layering within these saccular lesions caused by sluggish blood flow. Vision is usually normal, and no treatment is required. Rarely, vision loss can occur from vitreous hemorrhage. Although most occur sporadically, some may be associated with an autosomal dominant neurocutaneous syndrome, including cutaneous and central nervous system hemangiomas. Neurologic imaging is therefore recommended.

Unfortunately, our patient was immediately lost to follow-up.

This 32YO male presented with several weeks of decreased vision in the right eye. Vision was 20/200 OD and 20/20 in his normal OS. Examination revealed a dense vitreous hemorrhage, initially attributed to Valsalva retinopathy. He was immediately lost to follow-up and came back six years later with complaints of recent blurred vision with floaters. Vision was 20/50.

Optos color RG imaging shows a multilobulated mass superotemporal to the optic nerve, consisting of large saccular aneurysms with some overlying gliosis and preretinal blood. Fluorescein angiography shows superior layering of fluorescein within the vascular lesions, with blockage by blood. On OCT, the vascular abnormalities protrude into the vitreous with posterior shadowing.

Learning Points:
Retinal cavernous hemangiomas are rare, benign vascular lesions composed of thin-walled saccular venous aneurysms resembling clusters of grapes. Although most are asymptomatic and discovered incidentally, vision loss can occur from macular involvement or vitreous hemorrhage. Fluorescein angiography reflects the sluggish blood flow, classically showing plasma-erythrocyte layering as in our patient.

Although most are sporadic, some may be associated with an autosomal dominant neurocutaneous syndrome, including cutaneous and central nervous system hemangiomas. Neurologic imaging is therefore recommended, and our patient’s MRI results are pending.

This healthy 5YO boy was referred for an asymptomatic retinal lesion in his left eye. Vision was 20/25 OU.

Optos color RGB imaging shows unusual temporal changes involving the venous vascular tree. Prominent dilated and tortuous retinal veins drain the supero- and inferotemporal periphery before merging into a single vessel more posteriorly.

Surrounding these larger veins, especially inferiorly, are networks of small, corkscrew venules, along with more scattered tiny red microaneurysmal-like structures. Temporally, within the area drained by the larger dilated veins, there is a broad area of variable preretinal fibrosis.

Learning Points:
Retinal cavernous hemangiomas are usually unilateral, inner retinal grape-like clusters of aneurysms filled with dark venous blood. A gray fibrotic membrane often covers its superficial surface.

Fluorescein angiography shows plasma-erythrocyte layering within these saccular lesions caused by sluggish blood flow. Vision is usually normal, and no treatment is required. Rarely can vision loss occur from vitreous hemorrhage.

Although most occur sporadically, some may be associated with an autosomal dominant neurocutaneous syndrome, including cutaneous and central nervous system hemangiomas. Neurologic imaging is therefore recommended.

We believe our patient most likely has an atypical retinal cavernous hemangioma, given the scattered microaneurysmal-like changes (likely small ‘grapes’) and preretinal fibrotic component. Fluorescein angiography would help establish this diagnosis, but this was not possible given our patient’s age. Genetic testing was negative.

Retinal cavernous hemangiomas are usually unilateral, inner retinal grape-like clusters of aneurysms filled with dark venous blood. A gray fibrotic membrane often covers its superficial surface.

Fluorescein angiogram shows plasma-erythrocyte layering within these saccular lesions, attributable to sluggish blood flow.

Vision is usually normal, and no treatment is required. Rarely, vision loss can occur from vitreous hemorrhage.

Learning Points:

Although most occur sporadically, some may be associated with an autosomal dominant neurocutaneous syndrome, including cutaneous and central nervous system hemangiomas. Neurologic imaging is therefore recommended.

Retinal cavernous hemangiomas are usually unilateral, inner retinal grape-like clusters of aneurysms filled with dark venous blood. A gray fibrotic membrane often covers its superficial surface.

Vision is usually normal, and no treatment is required. Rarely, vision loss can occur from vitreous hemorrhage.

Learning Points:
Although most occur sporadically, some may be associated with an autosomal dominant neurocutaneous syndrome, including cutaneous and central nervous system hemangiomas. Neurologic imaging is therefore recommended.

Retinal cavernous hemangiomas are usually unilateral, grape-like clusters of aneurysms filled with dark venous blood. A gray membrane often covers its superficial surface.

Vision is usually normal and no treatment is required. Rarely vision loss can occur from vitreous hemorrhage.

Learning Points:
Although most occur sporadically, some may be caused by an autosomal dominant neurocutaneous syndrome, including cutaneous and central nervous system hemangiomas. Neurologic imaging is therefore recommended.