A 16-day-old newborn was transferred to our hospital for a bronchoscopy of a persistent opaque right lung on chest X-ray and persisting respiratory distress necessitating intubation.
The pregnancy and delivery had been uneventful. Radio-opaque right lung and respiratory distress started on day 3.
A control chest X-ray confirmed a persisting white right lung (Fig. 1).
A contrast-enhanced chest CT (Fig. 2 and 3) was then performed and shows a collapsed right hypoplastic lung, an absent right main stem bronchus, hypoplasia of the right pulmonary artery and one single pulmonary vein.
Bronchoscopy found a very narrow right main bronchus; the endoscope could not penetrate it.
An UGI (Fig. 4 and 5) series was performed thereafter. Opacification of the oesophagus revealed the visualization of a “fistula” that corresponded clearly, when completely opacified, to a bronchial tree, namely from the lower lobe.
The diagnosis of a bronchial oesophagus was thus ascertained.
An oesophageal bronchus (pulmonary oesophagus) is a rare type of the so-called “Broncho-pulmonary foregut malformation” where a bronchus connects to the oesophagus instead of to the bronchus or to the trachea. The mismatch probably occurs during the embryological separation between the primary gut and the developing tracheal bud.
The right side is mostly affected. The disorder is more common in women.
Chronic coughing, choking during feeding and chronic lung infections are the most common presentations [1, 2].
Unlike pulmonary sequestrations, the lung territory affected by the malformation is vascularized by a branch of the pulmonary artery and not through a systemic artery .
Opacification of the oesophagus is the most likely technique able to demonstrate the anomaly through opacification of a bronchial tree . Chest CT is able to demonstrate the abnormal lung and associated vascular anomalies.
Oesophageal atresia, tracheo-esophageal fistula and congenital heart disease are potential associated anomalies .
A right pneumectomy with closure of the tracheo-esophageal connection was performed.
Differential Diagnosis List
Pulmonary cystic adenomatoid malformation
Fistula (congenital or acquired – iatrogenic inflammatory or neoplastic)