Von Hippel-Lindau Disease

von Hippel-Lindau disease (VHL) is a rare, genetic multi-system disorder characterized by the abnormal growth of blood vessels in certain parts of the body (angiomatosis). The blood vessels "knot" together to form benign (non-cancerous) growths called angiomas.

Angiomas may develop in the brain, the retina of the eyes, and other areas of the nervous system. Other types of tumors develop in the adrenal glands, the kidneys, or the pancreas.

Symptoms of VHL vary among patients and depend on the size and location of the anigomas. Symptoms may include headaches, problems with balance and walking, dizziness, weakness of the limbs, vision problems, and high blood pressure. Cysts (fluid-filled sacs) and/or tumors (benign or cancerous) may develop around the angiomas and cause the symptoms listed above. Individuals with VHL are also at a higher risk than normal for certain types of cancer, especially kidney cancer.

Treatment for VHL varies according to the location and size of the angiomas. In general, the objective of treatment is to treat the growths when they are small so that they do not cause permanent problems by putting pressure on the brain or spine, blocking the flow of cerebrospinal fluid in the nervous system, or impairing vision. Treatment of most cases of VHL usually involves surgery to remove the tumors before they become harmful. Individuals with VHL need careful monitoring by a physician and/or medical team familiar with the disorder.

The prognosis for patients with VHL depends on the location and complications of the angiomas. Untreated, VHL may result in blindness and/or permanent brain damage. With early detection and treatment the prognosis is significantly improved. Death is usually caused by complications of brain tumors or kidney cancer.

Information provided by the
National Institute of Neurological Disorders and Stroke
National Institutes of Health

Each year, Medical College of Wisconsin physicians care for more than 180,000 patients, representing nearly 500,000 patient visits. Medical College physicians practice at Children's Hospital of Wisconsin, Froedtert Memorial Lutheran Hospital, the Milwaukee VA Medical Center, and many other hospitals and clinics in Milwaukee and southeastern Wisconsin.