Surgical treatment of congenital pulmonary arteriovenous fistula in children.

Congenital pulmonary arteriovenous fistulas are rare vascular malformations in which an abnormal connection develops between an artery and a vein in the lung. We report the case of a 5-year-old boy with a history of cyanosis since birth and cough for 2 days. Physical examination revealed cyanosis of oral mucosa and extremities, digital clubbing, and 82% O(2) saturation on room air. Chest radiograph showed a nodule in left lower lobe; echocardiogram was normal, but chest computed tomography showed 2 nodules in left lower lobe. Arteriography showed 2 large arteriovenous fistulas in left lower lobe. Patient underwent left lower lobectomy, and surgical outcome was excellent. At hospital discharge, mucosal color was normal and O(2) saturation was 96% on room air. Congenital pulmonary arteriovenous fistulas should be suspected in children with cyanosis but no cardiac malformations. Symptomatic congenital pulmonary arteriovenous fistulas should be treated with embolization when fistulas are small and multiple, or with lung resection when they are large and localized.