Abstract:
Differential diagnosis of a white lung in a newborn includes well known entities such as complete lung atelectasis and pulmonary hypoplasia, however rare diagnoses should also be considered. Here we present a case of esophageal lung presenting as a white lung in a newborn. Esophageal lung is an extremely rare congenital malformation in which the main stem bronchus is abnormally derived from the esophagus. It is a form of Bronchopulmonary foregut malformations (BPFM).
Case report:
A 17 days old full term female infant with dysmorphic features presented with respiratory distress. Chest radiography demonstrated right lung atelectasis. Chest Computed-tomography (CT) and angiography demonstrated a right bronchus originating from the esophagus. Fiberoptic bronchoscopy demonstrated absence of carina with no passage to the right main bronchus. The infant underwent a successful right pneumonectomy with repair of esophagus. At 8 month old she is respiratory stable with nasal oxygen given at home.
Discussion and conclusion:
Esophageal lung is a rare type of BPFM in which the lung communicates with the esophagus. In literature poor prognosis is described with high mortality rate. Our patient also has features of VACTREL association. To our knowledge, this is the fifth case of association between esophageal lung and VACTER association and the first in which a bronchoscopy was part of the diagnosis. Presentation as a white lung of this patient highlights the importance of acknowledging rare causes in differential diagnosis of lung atelectasis.