Tratamiento quirúrgico de fístula arteriovenosa pulmonar en telangectasia hemorrágica hereditaria (Enfermedad de Rendu Osler Weber)

Abstract

Pulmonary arteriovenous fistula in hereditary hemorrhagic telangiectasia. Report of one case Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) is an autosomal dominant vas- cular disease that can be associated with pulmonary arteriovenous fistulas. We report a 16 years old female with an Osler-Weber-Rendu syndrome and a history of frequent episodes of epistaxis since the age of 10 years. A Chest CAT scan performed on that occasion showed an arteriovenous fistula. The patient presented with progressive dyspnea, low arterial oxygen saturation and clubbing of fingers. An echocardiogram and lung perfusion scintigram confirmed the presence a right to left shunt. The patient was subjected to a lobectomy with a good postoperative evolution.