Hereditary Haemorrhagic Teleangiectasia Localized to the Gastrointestinal Tract

Abstract

Jacobson, G. & Krause, U. 1970. Hereditary Haemorrhagic Teleangiectasia Localized to the Gastrointestinal Tract. Scand. J. Gastroent. 5, 283-288.

A family is described with 5 cases of hereditary haemorrhagic teleangiectasia, 4 of whom could be regarded as definite and one probable. None of the patients had any visible cutaneous or oronasal manifestations, and in some cases, therefore, the diagnosis could only be made from the familial collection of gastrointestinal haemorrhages. Selective inferior mesenteric arteriography greatly helped the diagnosis in one of our cases. The various gastrointestinal manifestations are discussed, as well as the possibility of a precancerous importance of gastric teleangiectasia. The mortality of the disease has been reported previously to be 4%, and despite the improved possibilities of treating acute haemorrhages there is probably still some mortality risk when lesions occur in places other than the mouth, nose, and skin.

Key-words:

• Gastrointestinal diseases
• melena
• teleangiectasia, hereditary haemorrhagic