Bronchobiliary fistula caused by diaphragmatic invasion of a hepatic tumour

Abstract

A 71-year-old man presented with a productive cough consisting of yellow fluid. He had previously been treated for pneumonia without resolution in his symptoms. Sputum was tested for bilirubin using a urine dipstick given its similar appearance to bile, which was positive. Hepatobiliary scintigraphy scan revealed uptake of radiotracer in the right lower lobe of the lung. Endoscopic retrogade cholangiopancreatography confirmed diagnosis of a bronchobiliary fistula. The patient had a stent placed in the common bile duct promoting anterograde bile flow with complete resolution of symptoms.

Background

Hepatocellular carcinoma (HCC) is the most common primary liver malignancy with an incidence of 1.9 per 100 000 per year in the USA and higher incidences up to 150 per 100 000 in other areas of the world.¹ Risk factors include alcoholic cirrhosis, non-alcoholic fatty liver disease and hepatitis B and C viruses, the ladder of which explains the high incidence of HCC in certain endemic areas. Bronchobiliary fistula (BBF) is a rare complication of HCC first described in the literature in 1850.² At that time, BBF was described as a complication of infection, but more recent literature suggests malignancy may be the most common etiology.² BBF normally presents with bilioptysis and may be associated with fever, jaundice, abdominal pain or chest pain.³

Case presentation

A 71-year-old Caucasian man presented with a 3-week history of cough productive of bright yellow fluid. He denied fevers, chills, weight loss, abdominal pain or dyspnoea. His physical examination revealed rhonchi alongside coarse crackles in the right lung base. No superficial lymphadenopathy. A non-contrast CT scan of his chest showed a necrotising infiltrate in the right lower lobe. His symptoms did not improve with Ampicillin-Sulbactam for presumed aspiration pneumonia, so he was transferred for higher level of care. The patient was observed to be coughing up thin, non-purulent, bright yellow sputum. His medical history was significant for chronic hepatitis B infection diagnosed 10 years prior as well as HCC diagnosed 16 months ago. His HCC was treated with transarterial chemoembolisation (TACE) 15 months prior.

Investigations

On admission, a sputum sample was collected and tested for bilirubin using a urine dipstick, which was strongly positive and microscopic examination revealed bilirubin casts.⁴ Hepatobiliary scintigraphy (HIDA) was performed. This revealed extravasation of radiotracer coursing from the dome of the liver into the right lower lobe (figure 1).

Figure 1.

HIDA scan demonstrating tracer uptake from liver dome into the right lower lung.

Differential diagnosis

Necrotising pneumonia, ruptured biloma, hydatid disease, bile duct obstruction, iatrogenic cause post-radiofrequency or chemoembolisation.

Treatment

A diagnosis of BBF was made. The case was discussed with gastroenterology, interventional radiology as well as surgical oncology. Our goal was to find the least invasive and most effective method to resolve his symptoms. Gastroenterology was consulted for ERCP. Extravasation of contrast originating from the right intrahepatic ductal branches was observed during the procedure (figure 2). Sphincterotomy was performed and a stent was placed into the common bile duct as a therapeutic intervention. If this intervention had not been effective, the next step would have been using to ask interventional pulmonology for recommendations verses surgical resection of the mass in the liver by surgical oncology. Thankfully, the ERCP was a success and we did not have to go down that route.

Figure 2.

Fistulogram prebiliary stent placement. The yellow arrows outline the interrupted right and left intrahepatic branches.

Outcome and follow-up

The patient had an excellent outcome from temporary stent placement with resolution in his symptoms and subsequently discharged home.

Discussion

This is a case of a 71-year-old man with a history of HCC, previously treated with TACE, now presenting with bilioptysis. The HIDA scan along with ERCP confirmed that there was extravasation of bilious fluid into the patient’s lung parenchyma. CT reconstruction revealed a supradiaphragmatic mass encasing the right lower lobe segmental airways (figure 3). Although very rare, in one case necrotic fluid from HCC ruptured into the pleura and lung parenchyma creating a hepatopulmonary fistula. Our case differs in that there was bilious fluid invading the lung parenchyma rather than necrotic fluid. Our patient had no systems signs, including fever or leucocytosis, to suggest that this was necrotic.

Figure 3.

3D segmented anatomy reveals supradiaphragmatic extension of liver mass (blue) encasing branches of anterior and medial basal segmental airways (beige). Liver (red).

Figure 4.

Hypodense liver mass eroding through the diaphragm (red arrows) into the chest.

Patient’s perspective.

I was very relieved to find out what was causing my problems. Although I am still experiencing periodic problems coughing up bile, it is not a continuous situation. It comes and goes, sometimes one-2 days at a time, with smaller amounts coughed up, but it does hamper my appetite when it does occur. The permanent stent is doing its job and the drain seems to handle a good amount of bile, although amounts vary daily however I sure would like to live without it. The tumour continues to bother my lung function at times and I do not have the same breathing capacity. Fortunately, I have no other problems from the tumour and normally feel pretty good. I have learnt to live with my condition and am pleased to submit my comments.

Learning points.

• Bronchobiliary fistula (BBF) is a rare diagnosis which can be misdiagnosed as pneumonia. The most specific symptom of BBF is expectoration of bile tinged sputum.

• The most common causes for BBF in the literature include primary hepatic tumours, hepatic hydatidosis, trauma and biliary stenosis.

• Regarding primary hepatic tumours, procedures such as transarterial chemoembolisation and radiofrequency ablation (are commonly used for treating unresectable tumours, but may be associated with the development of BBF.

• Prior to his presentation, the patient had a non-contrast chest CT, which revealed a mass in the anterior lower hemithorax on the right, representing a hepatic tumour invading through the diaphragm. Diagnostic imaging clearly demonstrated communication between the bile ducts and right lower lobe airways, which was responsible for the bilioptysis.