Pulmonary sequestration and related congenital bronchopulmonary-vascular malformations: nomenclature and classification based on anatomical and embryological considerations.

The pulmonary sequestration spectrum and related congenital lung anomalies present an extremely complex and varied group of bronchopulmonary-vascular malformations. Previous attempts at nomenclature and classification have proved inadequate. In this article we present a classification of the newly named pulmonary malinosculation spectrum, which includes all congenital lung anomalies where there is abnormal connection (that is, malinosculation) of one or more of the four major components of lung tissue--namely, tracheobronchial airway, lung parenchyma, arterial supply, and venous drainage, which in various combinations make up these lesions. We feel that this simple descriptive approach will improve our understanding and management of these complicated lesions and this is supported by the clinical experience we report in the next article.