Introduction
Communicating bronchopulmonary foregut malformations (CBPFM) are rare congenital malformations that arise from faulty separation of tracheal and esophageal tissue during embryological development.1–4 The spectrum includes congenital cystic adenomatoid malformation, intralobular and extralobular pulmonary sequestration, associated foregut diverticula, duplication cyst, tracheoesophageal fistulae, and bronchoesophageal fistulae4–8.
Esophageal lung is a rare variety of CBPFM, characterized by a fistula between an isolated portion of respiratory tissue and the alimentary system, namely the esophagus or stomach 9. CBPFM may involve the entire lung or one of the pulmonary lobes1–4, 10,11 .Srikanth et al. suggested four major groups for classifying CBPFM 12.When the connection to the esophagus is by a lobar bronchus, CBPFM is classified as esophageal bronchus. When the connection is by a mainstem bronchus, CBPFM is classified as esophageal lung 4, 13,14.
Typically, esophageal lung malformations are associated with esophageal atresia 2. In this context, the right main bronchus is usually anomalously positioned and connects to the distal esophagus. In addition there is usually a tracheoesophageal fistula2, 15-17.
In this report, we present an extremely rare presentation. To our knowledge, this is the first patient described with unique dysmorphic features, in conjunction with the VACTER group of anomalies, and the sixth described with unilateral esophageal lung associated with the VACTER group of anomalies. The patient was successfully treated by resection of the hypoplastic lung and repair of the esophagus.