Pulmonary arteriovenous malformations related to Rendu-Osler-Weber syndrome

Interest in Rendu-Osler-Weber (ROW) syndrome has been renewed because of new treatment for the pulmonary artery fistulae that occur in approximately one-half of the patients. Pulmonary arteriovenous malformations (AVM) can be occluded safely by the transvenous placement of a silicone balloon thus avoiding the many potential complications of thoracotomy. Thirty-three members of four generations of a family with ROW illustrate the varied manifestations of the syndrome and provide a basis for review of clinical findings and therapeutic approaches to the management of pulmonary AVMs during the last 25 years. Special attention is given to early detection and prevention of major complications. Since ROW syndrome is an inherited trait, informed genetic counseling is an important component of the overall management of families with this disorder.