Congenital pulmonary arterio-venous fistula in childhood.

Pulmonary arterio-venous fistula is a rare congenital lesion of the pulmonary vasculature, causing cyanosis in children. The lesion can be solitary or multiple, usually involves the lower lobes and is often associated with the Rendu-Osler-Weber syndrome. The case of a 9-year-old boy is reported in whom diagnosis was made on pulmonary angiophotoscintigraphy and confirmed at angiography. The usual treatment of choice of these lesions is lobectomy which promptly relieves the symptoms.