Late presentation of esophageal bronchus in a patient with contralateral pulmonary hypoplasia: a diagnostic and therapeutic challenge.

Abstract

Introduction: Congenital bronchopulmonary foregut malformations are infrequent, with less than 60 cases reported. Group 3 or esophageal bronchus involves an abnormal origin of a lobar bronchus in the esophagus.

Clinical case: Patient with a history of heart disease requiring surgery and with left pulmonary hypoplasia. At 9 years of age, a chest CT-scan was carried out as a result of coughing following liquid intake. It revealed an esophageal bronchus of the right upper lobe. Given the history of pulmonary hypoplasia, a ventilation-perfusion scintigraphy was requested. It demonstrated lack of functionality of the right upper lobe. A right upper lobectomy was scheduled, with resection of the esophageal bronchus. 3D reconstruction was requested as part of preoperative planning. Following surgery, clinical signs disappeared, with an adequate respiratory function.

Discussion: Esophageal bronchus typically manifests in early childhood in the form of persistent atelectasis and repeated infections. However, in cases of stenotic bronchi, clinical signs may be unspecific, which requires multiple complementary tests and involves a high diagnostic suspicion. Late diagnosis usually leads to pulmonary resection. However, the latter is well tolerated even in patients with respiratory and cardiac pathologies. Patients with complex malformations benefit from preoperative planning with 3D reconstructions.