Bronchobiliary fistula: a rare complication after pancreaticoduodenectomy

Abstract

Biliary anatomic pathologies are rare disease occurrences that can result from a number of causes, most commonly iatrogenic, infectious and malignant aetiologies. Communications between the biliary and bronchial systems are even rarer and few cases have been documented in the literature. The present study describes a case of a 70-year-old man who underwent a pancreaticoduodenectomy (Whipple Procedure) as a curative procedure for an early stage neuroendocrine tumour. He presented nearly a decade after surgery with a communication between the biliary and bronchial tree initially manifesting as bilioptysis. His underlying medical conditions and poor clinical picture made treatment options limited. Therefore, he was treated conservatively with percutaneous biliary balloon dilation of the anastomotic stricture followed by temporary percutaneous internal/external drain and made a full recovery.

Background

Bronchobiliary fistulas are a rare complication leading to communication between the thorax and biliary system. There are numerous aetiologies leading to the development of a bronchobiliary fistula including: congenital, trauma, iatrogenic, infectious and neoplasms. Bronchobiliary fistulas can manifest weeks to many months, following offending biliary tract trauma. However, late presentations are extremely rare and even more so rare following iatrogenic aetiologies.

Case presentation

A 70-year-old man presented with progressive dyspnoea and fatigue. Initial complaints included 1 week of worsening fatigue and weakness in the setting of 3 months of intermittent bloody stools. He had a colonoscopy 2 months prior to his presentation which has showed two polyps which were removed. Haemoglobin on admission was 5.1 g/dL and mean corpuscular volume 77 fL. He received 3 units of packed red blood cells with stabilisation of haemoglobin to 7.0 g/dL. Workup was significant for severe iron deficiency anaemia. During his admission, he was evaluated by the inpatient gastroenterology consult service. Recommendations included intravenous iron infusion and deferment of endoscopic evaluation. Iron deficiency was attributed to iron malabsorption from prior bowel resections. He was discharged home.

Two days postdischarge, he returned with worsened dyspnoea and productive cough with bilious sputum. He was afebrile and haemodynamically stable. Chest auscultation revealed decreased breath sounds at the right lung base and rhonchi. Abdominal examination was significant for mild distention with no abdominal pain with palpation. Throughout the initial encounter, he had multiple episodes with copious bilious sputum production. The patient was not visibly jaundiced. Laboratory markers were notable for total bilirubin 2.5 mg/dL, direct bilirubin 1.5 mg/dL, aspartate aminotransferase 19 U/L, alanine aminotransferase 21 U/L and alkaline phosphatase 428 U/L. Notably alkaline phosphatase was found to be elevated ranging between 200 and 500 U/L in the last 24 months.

His medical history is significant for an ampullary neuroendocrine tumour status post ancreaticoduodenectomy complicated by a biliary stricture several months afterward later requiring hepaticojejunostomy anastomosis revision 8 years prior to presentation. Following revision, his postsurgical condition has been uncomplicated. Interestingly, he had an admission 4 years prior with an episode of ascending cholangitis. Imaging at that time revealed postsurgical changes of intrahepatic ductal dilation of 7.9 mm.

Treatment

Initial clinical impression was concerning pneumonia. However, he also had unexplained hyperbilirubinaemia. Right upper quadrant ultrasound demonstrated cirrhotic appearing liver, perihepatic ascites and right pleural effusion all in the presence of negative serological markers for liver injury including negative hepatitis serologies and normal ferritin. CT scan demonstrated a diaphragmatic fistula (figure 1). Cholangiogram showed a stricture at the afferent limb of hepaticojejunostomy and biliary duct dilation (figure 2A). He was started on antibiotics and a percutaneous dilation and biliary drain was placed (figure 2B,C). His bilioptysis subsided. Based on the difficulty in accessing the biliary system in the setting of a previous pancreaticoduodenectomy and revision, the decision was made to access the biliary system through percutaneous route. The decision of evaluating the stricture was felt to be due to an anastomotic stricture and therefore was not brushed. He underwent percutaneous anastomotic balloon dilation with temporary internal–external drain, by interventional radiology. His follow-up included 10 percutaneous dilation and drain exchanges in 8–12 week intervals.

Figure 1.

CT scan demonstrating a biliary fistula with breach in hemidiaphragm into the right lung cavity in the coronal (blue arrow) plane.

Figure 2.

(A) Cholangiogram image demonstrating the tight stricture of the choledochojejunostomy anastomosis (blue arrow) with marked dilation of the remnant biliary system. (B) Percutaneous dilation image of the stricture. (C) Postdilation image of the anastomotic stricture.

Outcome and follow-up

Eighteen months after initial presentation, the percutaneous biliary drain was removed. The follow-up of the bronchobiliary fistula was with interventional radiology and the plan was for conservative management. Following the final biliary drain removal, repeat CT imaging approximately 3 months from the last dilation and exchange showed a patent bile duct with no evidence of recurrence of the stricture or diaphragmatic fistula.

Discussion

A bronchobiliary fistula is a rare, known complication of pancreaticoduodenectomy. A bronchobiliary fistula is a disease process that reflects an abnormal communication between the abdomen and the thoracic cavity, through the development of a fistula from the biliary tract, pleural space and lung parenchyma to the respiratory tract. As bile is a strong irritant, erosion can occur through the lung parenchyma and into the bronchial wall.¹ The first bronchobiliary fistula was described by Peacock in a patient with a hydatid cyst in the liver in 1850. However, since that time, bronchobiliary fistula remains a rare disease entity.²

The aetiologies of bronchobiliary fistulas are numerous and include congenital and acquired. Acquired bronchobiliary fistulas result from iatrogenic, trauma, biliary obstruction, neoplastic or infectious processes. In the cases of congenital bronchobiliary fistula, presentation occurs early in life and is often repaired surgically with resection.³ In acquired cases of bronchobiliary fistulas, neoplasm (either primary or metastatic) cause approximately 32% of cases followed by biliary obstruction (including cholelithiasis) 31%, infectious process 12%, trauma 10%, chronic pancreatitis 3% and other multifactorial aetiologies accounting for 12% of cases.⁴

Apart from a congenital bronchobiliary fistula, despite multiple aetiologies, manifestation is largely the same. The offending aetiology typically predates presentation approximately 2 weeks to 6 months.⁵ Few case reports have documented late manifestations, with the longest recorded time frame occurring approximately 16 months following offending biliary tract trauma. Presenting symptoms will typically include bilioptysis (expectoration of bile), ranging from low volumes (approximately 100 mL per day) to high volume (>1 L per day). Bilioptysis is pathognomonic for a bronchobiliary fistula. The remainder of symptoms are non-specific and include pneumonia, pleural effusion, fever, ascites and abdominal pain.⁴

Definitive workup involves CT scans of the chest and abdomen. To demonstrate the fistulous tract, Endoscopic retrograde cholangiopancreatography (ERCP) or cholangiogram is helpful in defining biliary anatomy in planning management, as in our patient’s case.⁶

Given the rarity of the disease, there is not a gold-standard imaging modality. Chest X-ray will likely demonstrate a right-sided pleural effusion, with or without underlying lobar consolidation, if pneumonia is present. Sputum analysis and culture can be helpful to distinguish mucus versus bilious content.

Treatment options range markedly from no intervention to open surgical treatments. However, numerous case reports have documented clear or complete resolution of disease process, with non-surgical approaches with biliary drainage.

In summary, we report a case of a bronchobiliary fistula which occurred with an atypical late presentation after pancreaticoduodenectomy. The aetiology of the development of our patient’s bronchobiliary fistula likely resulted from biliary obstruction resulting from an anastomotic biliary stricture as indicative by elevated conjugated bilirubin, alkaline phosphatase, aspartate aminotransferase, alanine aminotransferase and advanced imaging demonstrating biliary dilation. The development of a bronchobiliary fistula is an extremely rare occurrence and diagnosis requires a high index of suspicion and early intervention to prevent progression and worsened clinical picture. In most cases of bronchobiliary fistula, development occurs as a complication after 6 months at most. Our case is markedly atypical in that it occurred 8 years following pancreaticoduodenectomy, as a result not of biliary tract trauma, but the development of scarring secondary to pancreaticoduodenectomy, ultimately leading to obstruction of afferent limb of small bowel and then elevated pressure in the biliary tract. This case highlights yet another mechanism of the development of bronchobiliary fistula, as well as highlighting a conservative treatment option. Given the patient’s overall poor surgical candidacy, percutaneous anastomotic balloon dilation with temporary internal/external drainage offered a way to decompress the biliary system, further demonstrating that conservative management is a viable option in patients deemed to be poor surgical candidates with the potential for favourable outcomes.

Learning points.

• Understand the pathophysiology of bronchobiliary fistulae.

• Understanding the diagnosis of bronchobiliary fistulae.

• Understanding the treatment of bronchobiliary fistulae.

• Keeping this diagnosis within the differential of patients who present with bronchobiliary contents during cough.