Hereditary Hemorrhagic Telangiectasia (Osler Weber Rendu Syndrome)
Description
An inherited disease of blood vessels which causes excessive bleeding. Patients develop red or reddish-purple collections of abnormal blood vessels called telangiectases. Patients experience bleeding episodes from the nose, gastrointestinal tract and the brain.
Symptoms
Nosebleeds, vomiting blood, rectal bleeding, headaches, seizures, confusion.
Tests
A history and physical exam will be performed. Genetic testing can be done to detect the defect in the gene ALK1.
Treatment
Estrogen may decrease the number of telangiectasias. Bleeding is stopped with cautery, but when severe surgery or immobilization of the artery is required.
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