Complex thoracic fistula in hereditary hemorrhagic telangiectasia

Pulmonary arteriovenous fistula are not an uncommon finding in hereditary hemorrhagic telangiectasia; however, systemic pulmonary fistulas are exceptional. A case, characterized by its complex anatomical constitution, is described; the right suprascapular artery is the afferent systemic vessel and the efferent systemic and pulmonary drainage network sprung from a cirsoid aneurysmal tangle. The reasons for the development of the fistula are uncommon and probably multifactorial. Clinical diagnosis and management problems are discussed.