Congenital tracheo-biliary fistula: staged surgical treatment

Abstract

Congenital tracheobiliary fistula is an extremely rare malformation which involves an anomalous connection between respiratory and biliary trees. There are few cases described on literature, and so far at the moment there are 36 cases described. Patients usually show up with respiratory distress and bile stained expectoration. The diagnosis is made with images exams, like bronchoscopy, broncography, cholangiography, CT or MRI scan. The surgical approach must be as early as possible to prevent respiratory sequel. There are two hypotheses of etiology, duplication of upper gastrointestinal tract or anomalous fusion of a bronchial tree with the bile duct. Here we present a case of a female patient, 21 days, 3,8 kg, with historical of respiratory discomfort and cyanosis after breastfeeding since de fourth day of life. On ICU admission, bile stained sputum was noticed at oro-tracheal tube. Computer Tomography showed aerobilia and an anomalous tract communicating the trachea from carina through the left hepatic lobe, what confirmed the diagnosis of tracheobiliary fistula. The surgical treatment was a staged approach with the thoracic surgery first and the fistulous tract resection, followed a left hepatectomy. Keywords: Tracheobiliary fistula, Persistent cough, Biliary fistula