Multiple pulmonary arteriovenous fistulas in juvenile cirrhosis

Abstract

Clinical and pathologic findings in a case of juvenile cirrhosis associated with cyanosis, clubbing of the digits, greatly elevated cardiac output and a long bruit are presented. Although careful examination of the lungs at necropsy, both grossly and microscopically, failed to reveal any significant pathology, injection of a plastic solution into the pulmonary vessels revealed the presence of numerous abnormal vascular channels connecting the pulmonary arteries and veins. Thus the presence of multiple arteriovenous fistulas in the lungs, suspected and sought for during the patient's life, was finally confirmed. Pulmonary arteriovenous fistulas of the classical type may be multiple. The findings in the present case suggest an entirely different process. A review of the literature has failed to reveal similar reported cases, probably because injection studies of the pulmonary vascular tree have not been performed in instances of juvenile cirrhosis associated with cyanosis and clubbing.