Connected to the wrong pipe: esophageal bronchi mimicking bilateral bronchial atresia

Infants frequently present with respiratory symptoms, but diagnosing the underlying pulmonary condition is sometimes challenging. Here, we describe an infant presenting with respiratory distress due to a rare pulmonary condition. Different investigations eventually led to the correct diagnosis and the patient successfully treated. Rare respiratory conditions need to be considered to ensure early and appropriate care. A 2-week-old neonate was referred with a postnatal history of tachypnea, poor feeding and elevated plasma inflammation markers. The patient presented with mild episodes of coughing after feeding and diminished breath sounds of the right upper lung. A chest radiograph revealed bilateral consolidations. Whereas bronchoscopy was suggestive of bilateral bronchial atresia, computed tomography supported bronchial atresia of the right upper and middle lobe and a left-sided broncho-esophageal communication. Surprisingly, an upper gastrointestinal series revealed bilateral esophageal insertion of bronchi and the diagnosis of a communicating bronchopulmonary foregut malformation (CBPFM) was made. Two-stage lobectomy of the affected lobes and segments was performed at days 31 and 41 after birth. Histopathological examination exhibited hamartomatous lung tissue with purulent bronchopneumonia. At a follow-up examination after 4 years, the patient was asymptomatic and thriving well with oral feeds. CBPFM are rare malformations. This case highlights the clinical challenge of diagnosing this rare condition. There is a need to raise awareness for such uncommon conditions and improve diagnostic accuracy. For optimal management a multidisciplinary approach is essential.