First described by Peacock in 1850, a bronchobiliary fistula (BBF) is a rare complication after hepatectomy, which consists of an abnormal intercommunication between the biliary tract and bronchial tree (1). This may occur due to an obstruction of the biliary tract, trauma or hepatic abscess. Especially in endemic regions, hydatid or amebic disease of the liver are common causes of BB (2,3). The classic clinical presentation includes cough producing yellow sputum, known as bilioptysis, which represents a pathognomonic sign of BBF (4). Of note, BBF is associated with high morbidity and mortality rates. The management of this rare entity is challenging with limited current evidence to date on how to treat this condition (5). Herein, we present a case of BBF following a central hepatectomy and the successive steps of the management. In addition, we sought to conduct a review the literature to summarize all the available evidence regarding post-hepatectomy BBFs.

A 64-year-old Caucasian male was diagnosed with adenocarcinoma of the rectum in 2012 for which he underwent low anterior resection with adjuvant chemotherapy (FOLFOX). The histological report of the specimen revealed a moderate to poorly differentiated adenocarcinoma of the rectum with T3c invasion of the bowel wall and metastases to mesorectal lymph nodes (stage IIC). The patient was referred to the oncology department for further treatment. During a routine follow-up at three years, the computer tomography (CT) scan demonstrated an asymptomatic solitary hypoechoic lesion in segment VIII measuring approximately 5 cm in diameter (Fig. 1). Given the absence of other comorbidities, the multidisciplinary team decided that liver resection was appropriate.

During exploratory laparotomy, intraoperative findings with hepatic U/S confirmed a solitary lesion in segment VIII of the liver. A central hepatectomy was performed without any intraoperative complications and the immediate postoperative clinical course was uneventful. Histological report confirmed that the lesion was of metastatic nature, from poorly differentiated adenocarcinoma of colon, and the patient was referred to the oncology department.

Three months later, the patient was admitted under the care of the medical oncology team with a history of fatigue for two days, weight loss and fever up to 39°. A CT scan demonstrated a postoperative intrahepatic collection (Fig. 2A) and a 12F drain was inserted by a radiologist with CT guidance. An endoscopic retrograde cholangiopancreatography (ERCP) was performed which demonstrated a bile leak, and a 9 cm 10 F stent was placed (Fig. 2B). The patient was discharged 20 days later with the drain in situ and was examined regularly in our outpatient clinic.

However, 14 months postoperatively, the patient presented to the oncology department complaining of new onset fever and expectoration. A chest x- ray was performed demonstrating atelectasis of the lower right lobe and was treated as suspected pneumonia. Given the patient previous history, an inflammatory tumor was included in the differential diagnosis. Although afebrile, the patient reported episodes of a productive cough with dark-yellow sputum, raising concerns for bronchobiliary fistula. The patient underwent a CT scan which confirmed the clinical suspicion and he was subsequently referred to our department for further treatment. Since the patient had a biliary stent in situ, the surgical team decided that a further operation was required. Using a transabdominal approach, a communication between the initial intrahepatic collection and one inferior lobular bronchus was demonstrated. Transhiatal removal of fistula was performed with closure of the defect through the abdomen (Fig. 3). There was no bile leak through the remaining liver parenchyma.

The postoperative course was uneventful with no further bilioptysis or other pulmonary symptoms. At radiological follow up one month postoperatively, a CT showed a postoperative biloma for which the patient underwent CT guided drainage with a 12F catheter. A further ERCP revealed leakage and communication of the bile duct with the biloma which was drained and a stent was inserted. The patient was discharged 2 months after surgery and was examined regularly in the outpatient clinic. He was readmitted on a schedule to remove the plastic stent by ERCP. Currently, there are no signs of further bile leakage, BBF or pulmonary symptoms.

BBP is an uncommon condition whose etiology can differ by geographical region; in developing countries echinococcal and amebic disease are the most common causes, whereas in developed countries trauma and biliary obstruction predominate (6). According to a recent systematic review, tumor is the most frequent cause (32.3%) of BBF formation, followed by bile duct obstruction (30.8%) (7). Other causes have also been identified, such as chronic pancreatitis, trauma and congenital malformation. The pathogenesis of fistula formation has not been completely understood yet, although increased pressure within the biliary tree and local inflammation appeared to be the two major factors that contribute to the development of BBF. In our case, intrahepatic abscess triggered the formation of adhesions between the diaphragm and the lung and was thought to be the cause of the BBF formation (4,8–10).

The clinical presentation is variable, and may include fever, irritating cough, jaundice, and abdominal pain. The pathognomonic feature is a productive cough of bile stained sputum (7,11). Chest pain or episodes of dyspnea occur only in a minority of patients, while nausea, vomiting, portal hypertension and liver dysfunction are rarely found. In terms of diagnosis, CT imaging is usually the predominant modality or biliary scintigraphy with hepatic iminoacetic acid (HIDA) which demonstrates initial tracer activity in the liver which migrates into the chest cavity (12). BBF can also be diagnosed by interventional techniques, such as ERCP, percutaneous transhepatic cholangiography, bronchoscopy or fistulography (4,13,14).

Currently, there are no guidelines on how to treat this rare complication with both conservative and surgical approaches considered acceptable to date. Some authors suggest that BBF associated with benign causes should be treated conservatively; endoscopic retrograded biliary drainage (ERBD), endoscopic nasobiliary drainage (ENBD), percutaneous transhepatic cholangiography and biliary drainage (PTCD), and abscess drainage (15–17). Adjunct use of octreotide might have a role in some cases due to reports of successful resolution or reduction of symptoms with its use (18). On the other hand, surgical management should be considered in case BBF has developed in a background of tumor, trauma or obstruction and conservative therapy has previously failed (7). Operative exploration and repair of the initial injury is usually performed, whereas resection of the involved pulmonary area and removal of the fistula is advised in the form of thoracobiliary fistula decortication (18).

Table I summarizes a brief review of the literature on bronchobiliary fistulas after liver surgery for both benign and malignant diseases (9,11,19–30).

Due to low incidence of BBF, there is no clear consensus on the treatment of this uncommon complication to date. Multidisciplinary management of such patients should be considered taking into consideration the underlying pathology leading to this rare complication. Conservative treatment should be considered first, while surgical resection of the BBF remains an option when other therapies have failed. Surgeons should have low suspicion of diagnosing and managing this complication after biliary tract operations.