Double congenital esophageal bronchus with microgastria: a case report

Congenital esophageal bronchus (CEB) is a rare malformation sometimes associated with microgastria and/or other congenital anomalies. It may present later in life with recurrent pulmonary symptoms. A term baby was born with an antenatal diagnosis of multiple congenital anomalies including absence of stomach bubble, congenital heart disease and a lumbosacral cystic lesion. Post natal diagnosis confirmed dextrocardia, unbalanced atrioventricular canal defect with hypoplastic right ventricle and pulmonary stenosis, situs inversus totalis, asplenia, and abnormal lumbar and sacral vertebrae with a skin covered lipomyelomeningocele. After birth, an orogastric tube was placed to 15 cm, which on chest x-ray (CXR) stopped at the lower 1/3 of the esophagus. Gas was seen in the abdomen. A contrast study demonstrated microgastria, a gastroesophageal junction (GEJ) within the chest, and a CEB. The CXR was suspicious for a lesion in the lower left lobe (LLL); CT-angiogram confirmed no airway or systemic blood vessels to the LLL. Esophagoscopy revealed an elevated, tight GEJ and 2 distal openings towards the lung. A thoracotomy was performed at 3 weeks of age to close the CEB and resect the LLL; a chest tube was left in situ. Feeds were initiated 1 week after surgery when a contrast study revealed no leak. The baby is tolerating full bolus feeds and at 3 months of age remains in hospital awaiting cardiac surgery. CEB is a rare anomaly and should be considered when a nasogastric tube cannot be advanced easily, particularly in the setting of multiple congenital anomalies.