Congenital respiratory-biliary fistula

Congenital respiratory-biliary fistula (also known as congenital bronchobiliary fistula or tracheobiliary fistula) is an extremely rare congenital malformation characterized by an abnormal communication between the biliary system (bile ducts of the liver) and the respiratory tract (bronchi or trachea). This anomalous connection allows bile to pass from the liver into the airways, leading to distinctive and often dramatic clinical presentations in the neonatal period or early infancy. The hallmark symptom is bilious expectoration — the coughing up of bile-stained (green or yellow) secretions — which is essentially pathognomonic for this condition. Affected infants typically present with recurrent respiratory distress, chronic cough, choking episodes, and recurrent pneumonia due to the irritating effect of bile on the lung tissue. Failure to thrive may also occur. The condition primarily affects the hepatobiliary and respiratory systems, and if left untreated, chronic bile aspiration can lead to progressive lung damage, bronchiectasis, and potentially life-threatening respiratory complications. Diagnosis is often established through imaging studies including hepatobiliary scintigraphy (HIDA scan), bronchoscopy, and contrast studies that demonstrate the fistulous tract. Treatment is surgical and involves division and ligation of the fistulous communication, sometimes combined with reconstruction of the biliary tract if associated biliary anomalies (such as biliary atresia) are present. Early surgical intervention generally leads to favorable outcomes, though the prognosis depends on the presence of associated congenital anomalies and the degree of pre-existing lung damage.

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