Congenital tracheo-bilary fistula: Case Report

Abstract

Introduction: Congenital tracheo-bilary or broncho-biliary fistula, is a rare malformation with a high risk of morbidity and mortality. It is characterized by an abnormal connection between the respiratory system (traqueo/bronchus) and the biliary tract. The main symptoms are recurrent pneumonia and bile-stained sputum. In addition, this unusual congenital disease should be considered in neonates with respiratory distress and bilious discharge through the airway. Case presentation: The case report presents a 7-day-old male, with a 1-day history of non-cyanotic emetic wet cough, nasal secretions with bilious characteristics associated with increased respiratory distress; needing management in the pediatric critical care unit, where the diagnosis was achieved and the multidisciplinary management was initiated. Results: During the hospitalization in the pediatric critical care unit, it was required invasive mechanical ventilation, broad-spectrum antibiotic therapy and conservative management of the right apical atelectasis for two weeks with no improvement, also with persistence of bilious respiratory secretions. A diagnostic flexible bronchoscopy was performed, which reported a congenital broncho-biliary fistula. Cholangioresonance was also performed, which reported a congenital tracheal-biliary fistula: a 2.5mm hypointense tubular image. A ligation with the resection of the fistulous tract was successfully performed, the patient is stable at home without oxygen requirements. Conclusions: This case stands out because it is a very uncommon disease. Bronchoscopy has proven to be the gold standard for achieving an accurate diagnosis, followed by computed tomography with 3D reconstruction. Surgical treatment is the gold standard, one approach is to perform a ligation with or without the resection of the fistulous tract, and the other approach is the hepatic lobectomy and reimplantation of the fistula in the gallbladder or intestine. Interdisciplinary management is essential for its diagnosis and effective treatment.