Bronchial trifurcation in a congenital pulmonary venolobar syndrome

Congenital malformations of the tracheobronchal tree and the related arteri al blood supply are a complex group of lesions in which there are abnormali ties of the venous drainage and lung parenchyma. These malformations are ex amples of congenital pulmonary venolobar syndrome (CPVS). Tracheal trifurca tion is an extremely rare anomaly associated with CPVS. We report on an unu sual case of lower right extralobar sequestration connected to the trachea, plus a type I posterior laryngeal cleft, an aberrant systemic artery, and an anomalous route of the phrenic nerve. This paper discusses the place of this unusual abnormality in the spectrum of congenital bronchopulmonary vas cular malformations. (C) 2001 Wiley-Liss, Inc.