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A to Z: Von Hippel-Lindau Disease

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May also be called: VHL; Von Hippel-Lindau Syndrome

Von Hippel-Lindau (vahn HIP-ul LIN-dow) disease, or VHL, is a rare genetic disorder that causes blood vessels to grow abnormally. A genetic disorder means it’s the result of a change in genes that was either inherited (passed on from parent to child) or happened during development in the womb.

More to Know

Blood vessels usually grow like branches on a tree, but in people with VHL, they form small tumors called angiomas or hemangioblastomas. VHL usually affects certain areas, such as the brain and other parts of the central nervous system, the retina of the eye, the adrenal glands, the kidneys, or the pancreas. Depending on where they are in the body, angiomas can cause other medical problems (for example, angiomas on the retina may lead to vision loss).

Symptoms usually appear when someone is between 10 and 30 years old. Symptoms depend on the size and location of the angiomas and can include headaches, balance problems, dizziness, weakness, vision problems, and high blood pressure. Fluid-filled cysts or tumors (benign or cancerous) may develop around the angiomas, making these symptoms worse. People with VHL have a higher risk of developing cancer, especially kidney cancer.

VHL treatment depends on the size and location of the angiomas. The goal is to treat the tumors while they’re small and before they put pressure on any of the major organs, such as the brain or spine. Surgery may be needed to remove the tumors before they cause severe problems.

Keep in Mind

The outlook for VHL patients depends on where the tumors are in the body and the complications they cause. Fortunately, early detection and treatment can improve a patient’s outcome.

All A to Z dictionary entries are regularly reviewed by KidsHealth medical experts.