1. Background

• Bronchobiliary Fistulas can be congenital or acquired (resulting from a biliary infection, malignancy, or trauma)

2. Etiology

• unknown but two pathogenetic mechanisms proposed:
  • duplication of the upper gastrointestinal (GI) tract or
  • union of an anomalous bronchial bud with an anomalous bile duct
• chronic exposure of the respiratory tract to bile acid can cause persistent inflammation of the lower airways causing recurrent lower respiratory illness and hyperreactive airways and persistent inflammation of the upper airways resulting in chronic sinusitis and nasal symptoms

1. Respiratory Manifestations

• recurrent pneumonias
• chronic cough and wheeze
• respiratory distress
• others
  • persistent yellow nasal discharge
  • pansinusitis

2. Other Manifestations

• bilious vomiting
• failure to thrive
• coexisting malformations of the GI tract and biliary tree
  • biliary atresia

1. Flexible Bronchoscopy

• diagnostic and procedure of choice
• usually the fistula arises from the right mainstem bronchus adjacent to the carina ("trifurcation of the bronchi") and communicates with the biliary system arising from the left lobe of the liver
• radio-opaque contrast material (Hypaque) introduced into the fistula by the bronchoscope can demonstrate the bronchobiliary fistula under fluroscopy
• endobronchial secretions contain bile salts

2. Imaging Studies

1. Supportive

• symptomatic
• antibiotics for recurrent pneumonias
• bronchodilators and steroids for hyperreactive airways

2. Surgery

• curative
• excision of the fistula +/- partial hepatectomy

3. Prognosis

• 30% mortality rate from pulmonary complications in the first year of life if not surgically repaired
• risk of reactive airway disease after surgical repair due to exposure of bronchus to bile salts