von Hippel Angioma

von Hippel angioma

Von Hippel angioma can grow within the retina or optic nerve. They characteristically have a "feeding" retinal arteriole and a "draining" retinal vein. Bilateral involvement can be seen in up to 50% of individuals.

Von Hippel angioma are hemangiomas, are not cancers and do not metastasize. Twenty percent of patients will be found to have the von Hippel Lindau Syndrome -associated with cerebellar hemangioma, pheochromocytomas, visceral cysts and renal cell carcinomas.

Symptoms
Von Hippel angioma patients either have no symptoms, or become symptomatic due to secondary retinal detachment or rarely neovascular glaucoma. The symptoms of retinal detachment are flashes of light, spots in the vision (floaters), and loss of vision. The symptoms of neovascular glaucoma are eye pain, light sensitiviy, vision loss, and headache.

Diagnosis
Some patients with von Hippel angioma will have a family history of this disease. Von Hippel angioma are usually visible by dilated eye examination (ophthalmoscopy). Ultrasound can be used to measure the tumor's size, and to evaluate for high internal reflectivity. Ophthalmoscopy typically reveals a dilated feeder artery and draining vein. An associated retinal detachment may be seen around the tumor or may be so large as to cover (obscure) an underlying von Hippel angioma.

Fluorescein Angiography: Eye-care specialists perform studies of the blood vessels in the eye with a vegetable dye called fluorescein. The dye is injected into the arm and travels to the blood vessels inside the eye. If a tumor is in the eye, we can see specific characteristics of its circulation which can help us differentiate between it and other types of tumors. Von Hippel angiomas have a unique pattern of circulation with a feeder arteriole and a draining vein. Since the tumor extends into the vitreous humor, von Hippel angiomas exhibit intense hyperfluorescence, often compared to a light-bulb.

Fluorescein Angiography - Note the feeder and draining vessels, as well as the bright "light-bulb" appearance.

Treatments
Von Hippel angiomas can appear in both an autosomal dominant hereditary or sporadic forms. All patients should be given periodic systemic examinations including imaging studies for cerebellar hemangiomas and renal cell carcinoma. Family members should be examined with indirect ophthalmoscopy. Genetic testing is available (see related links below).

The angiomas can occur beneath and on top of the retina. Should they leak, secondary retinal detachments can cause blindness. Direct treatment with laser and cryotherapy have been described. If no detachment is present and the tumor does not enlarge, it may be followed by close periodic observation.

A leaking von Hippel Angioma prior to laser photocoagulation.

Von Hippel angioma after laser photocoagulation.

Related Links
For more specific information on the genetic aspects of von Hippel Lindau Syndrome Click Here!

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