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Von Hippel-Lindau Disease

Von Hippel-Lindau Disease (VHL) is a rare inherited genetic condition involving the abnormal growth of tumors in parts of the body which are particularly rich in blood supply. In people with VHL little knots of capillaries sometimes occur called angiomatosis. The tumors of the central nervous system (CNS) are called hemangioblastomas These tumors, whether benign (usual) or malignant (rarer), may cause problems, for example angiomas in the brain or spinal cord may press on nerve or brain tissue. As an angioma grows, the walls of the blood vessels may weaken and leak, causing damage to surrounding tissues. Blood leakage from angiomas in the retina can interfere with vision. Cysts may also grow around angiomas.

VHL is a autosomal dominant disorder, but there is a wide variation in the date of onset of the disease, the organ system affected and the severity of effect. Untreated, VHL may result in blindness and permanent brain damage, death is usually caused by complications of malign tumors in the brain or kidney.

Dr. Eugen von Hippel described the angiomas in the eye in 1904. Dr. Arvid Lindau described the angiomas of the cerebellum and spine in 1926.

The disease is also called Angiomatosis Retinae, Angiophakomatosis Retinae et Cerebelli, Familial Cerebello-Retinal Angiomatosis, Cerebelloretinal Hemangioblastomatosis, Hippel Disease, Hippel-Lindau Syndrome, HLS, Lindau Disease, or Retinocerebellar Angiomatosis.



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