Discussion
Esophageal lung is a rare type of CBPFM, in which the entire lung or
part of it communicates with the esophagus by a bronchus, and does not
communicate with the native tracheobronchial tree. CBPFM is
differentiated from pulmonary sequestration by its arterial supply,
extent of pulmonary involvement, and the esophageal origin of the
involved main stem bronchus (12,14,18). The left lower lobe and the
entire right lung are the most common locations of pulmonary
involvement. Most frequently, communication with the esophagus is found
in the distal portion of the esophagus or gastroesophageal junction. The
majority of patients with unilateral disease (60%) present in the first
eight months of life, and the incidence in females is nearly twice that
in males 15,17 . Almost 50 percent of the patients
have associated esophageal atresia or tracheoesophageal fistula12, 17. The arterial supply derives from the pulmonary
artery or an independent branch of the aorta, or both4, 14. Venous drainage is to the pulmonary vein, the
portal vein, or the azygous system.
Chronic cough, recurrent pneumonia, and respiratory distress are the
most common presentations of CBPFM 3). Bronchiectasis,
gastrointestinal hemorrhage, dysphagia, and hemoptysis are rare
presenting symptoms 12, 14. Associated anomalies in
CBPFM include pulmonary hypoplasia; malformations of the heart, kidneys,
ribs, or vertebral columns; and anomalies of the digestive tract. In
some patients, esophageal atresia with tracheoesophageal fistula and
VACTER association can also co-exist 5,10,11,14,17.
The clinical features in our patient, including hemivertebra, vestibular
anus, cardiac anomaly (double outlet right ventricle, ventricular septal
defect, and atrial septal defect), and right renal agenesis, are
compatible with the definitions of both VACTER association and
esophageal lung19. To our knowledge, this is the first
report of esophageal lung associated with the VACTER group of anomalies
combined with dysmorphic features. In 1995, Noriaki Usui et al.
described a neonate who, in addition to an esophageal lung, had
dysmorphic features with anomalies that were not compatible with the
VACTER association 20. This is the sixth patient
reported to have an association between an esophageal lung and the
VACTER group of anomalies 12, 17, 21.
At most centers, pneumonectomy is the usual treatment offered to
patients with esophageal lung. Reimplantation to the trachea with an
uneventful follow-up period has been reported in a number of patients20,22,23. The best prognosis is correlated with
pneumonectomy, older age, and term birth15, 22, 23 .
In conclusion, diagnoses of
esophageal lung and other bronchopulmonary foregut malformations require
a high index of suspicion. Recurrent lower respiratory infections and
respiratory distress are common presenting symptoms. The condition
should be included in the differential diagnosis of non-resolving lung
collapse, especially in the neonatal period10,12.
Additionally, the presence of tracheoesophageal fistula or VACTER
association may suggest the diagnosis. Therefore, complete evaluation of
the tracheobronchial pulmonary system is highly recommended in these
patients.