Complications of Pulmonary AVMs in a Patient with Rendu-Osler-Weber Disease
A B S T R A C T
Rendu-Osler-Weber Disease, also called hereditary hemorrhagic telangiectasia (HHT), is a rare, genetically determined complex disease with a spectrum of associated manifestations which extend beyond the typical pathology of arteriovenosus malformations (AVMs). Our subject, a 54-year old male patient with a long history of HHT has a typical mucocutaneous telangiectasias and bleeding from the nose, but the most important manifestations of the disease are those related to pulmonary AVMs. A variety of complications, such as hypoxemia, polycythemia, cyanosis, clubbing fingers and brain abscesses, have developed over time as a consequence of a-v shunt in the pulmonary AVMs. A large pulmonary AVM necessitated a surgical intervention when the patient was in his thirties, and two more surgeries followed later, due to brain abscesses. Despite intensive manifestations of the disease and episodes of life threatening complications, the patient has adjusted well to his chronic medical condition and lives a near-normal life.
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