Congenital bronchopulmonary vascular malformations: clinical application of a simple anatomical approach in 25 cases

Congenital malformations of the bronchopulmonary airway and related arterial blood supply are a complex group of lesions in which abnormalities of venous drainage and lung parenchyma may coexist. Twenty five cases have been analysed, by a method whereby each anatomical component is separately considered. All 25 patients had abnormalities of the tracheobronchial tree, with no connection to the abnormal segment in nine cases. The aberrant arterial blood supply was single in 16 cases and multiple in nine cases, one patient from the latter group having a mixed pulmonary and systemic arterial supply to a part of the abnormal segment. Seventeen patients had anomalous venous drainage. In nine of these the vein or veins (they were multiple in four cases) drained the major part or the whole of the lung, whereas the aberrant arterial supply was limited to the right lower zone--that is, mismatched anomalous venous drainage. Abnormalities of lung parenchyma included changes within the lesion (for example, cysts, foregut inclusions) and associated abnormalities of surrounding lung (for example, hypoplasia, abnormal lobation). This information, together with the clinical features and haemodynamic data, was found to be essential for decisions on appropriate management. Patients presenting in infancy with haemodynamic disturbance continue to present major management problems (50% mortality), particularly if there are associated congenital heart defects. The role of aberrant systemic artery occlusion or ligation as a first stage procedure is well established in patients with haemodynamic abnormalities. There may be a place for this procedure in selected patients who have no haemodynamic disturbance at presentation.