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.