Retinal Cavernous Hemangioma
Although most cases of cavernous hemangioma are sporadic and restricted to the retina or optic nerve head, they may occur in a familial (autosomal-dominant) pattern and may be associated with intracranial and skin hemangiomas. For this reason, cavernous hemangioma may be considered one of the phakomatoses.
Retinal cavernous hemangioma is characterized by the formation of grapelike clusters of thin-walled saccular angiomatous lesions in the inner retina or on the optic nerve head (Fig 7-15). The blood flow in these lesions is derived from the retinal circulation and is relatively stagnant, producing a characteristic picture on fluorescein angiography. These dilated saccular lesions fill slowly during angiography, the sluggish blood flow results in plasma-erythrocyte layering. Fluorescein leakage is characteristically absent, correlating with the absence of subretinal fluid and exudate in the retinal cavernous hemangioma and serving to differentiate the condition from retinal telangiectasia, retinal hemangioblastomas, and racemose hemangioma of the retina.
These hemangiomas usually remain asymptomatic but may bleed into the vitreous in rare instances. Vitreous traction is thought to be the cause of the hemorrhage in these cases. Treatment of retinal cavernous hemangiomas is usually not indicated unless vitreous hemorrhage recurs, in which case photocoagulation or cryotherapy may be effective. See also BCSC Section 4, Ophthalmic Pathology and Intraocular Tumors.
Figure 7-15 Retinal cavernous hemangioma. A, Fundus photograph of the left eye shows a retinal cavernous hemangioma in the temporal retina. B, These tumors characteristically appear as a grape-like saccular cluster of dark intraretinal venous aneurysms (arrowheads), typically located along the course of a retinal vein, without a feeding artery. White, fibroglial tissue is commonly present on the tumor surface (arrow), suggesting previous vitreous or preretinal hemorrhage. C, Early fluorescein angiography typically shows the very slow appearance of dye within the venous aneurysms. D, The tumor eventually fills with dye, without leakage. E, Fundus photograph of the right eye in a 12-year-old boy shows a larger characteristic retinal cavernous hemangioma in the temporal retina. F, Fluorescein angiography image of this tumor also demonstrates late filling of the intraretinal venous aneurysms, without leakage of dye.
(Courtesy of Brian Leonard, MD.)
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.