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.