Hereditary Hemorrhagic Telangiectasia

Hereditary hemorrhagic telangiectasia is a familial disease characterized by the presence of telangiectasia of the skin and mucous membranes. It is indicated clinically by recurrent bleeding, most commonly epistaxis. Involvement of multiple systems occurs, and vascular lesions have been recognized in the lips, oral cavity, respiratory tract, alimentary tract, urinary tract, liver, spleen, eye, brain, meninges, spinal cord, and bone. The incidence of gastrointestinal hemorrhage is significant and has proved to be a difficult diagnostic and therapeutic problem. We report here the vascular abnormalities and hemodynamic alterations that have been found in an investigation of gastrointestinal bleeding in patients with hereditary hemorrhagic telangiectasia (Table I). There are many reports in the literature describing telangiectasia and phlebectasia of various abdominal organs. These lesions have been recognized at the time of gastroscopy and sigmoidoscopy and at surgery and autopsy. We have been able to find o...