Pulmonary arteriovenous fistula

Pulmonary arteriovenous fistula (A-V fistula) is a rare malformation, mostly congenital but can also be acquired. It occurs as isolated malformation or in association with hereditary or acquired hemorrhagic telangiectasia. We present a boy aged 2.5 years with an A-V fistula in the right lower lobe. Notable clinical findings included cyanosis, clubbing, hypoxemia and polycytemia. Diagnosis was confirmed angiographically. Hereditary hemorrhagic telangiectasia was excluded. Surgical resection, with atypical cuneate excision of the fistula and adjacent parenchima, was carried out. Post-surgical follow-up showed that cyanosis, hypoxemia and polycytemia had disappeared.