A bronchobiliary fistula, which is a challenging entity to diagnose, typically presents with a chronic productive cough and greenish sputum. Common causes of bronchobiliary fistula include liver abscess, hydatid cysts of the liver, post-liver resection complications, and chronic pancreatitis. It can also develop as a secondary complication following transarterial embolization. However, the occurrence of a bronchobiliary fistula in conjunction with portal cavernoma cholangiopathy is exceedingly rare.

A 42-year-old man was admitted to our hospital’s pulmonology department with a complaint of a chronic productive cough persisting for 2 months. His cough worsened when he lay down, accompanied by green-colored phlegm. No other unusual symptoms were reported.

The patient’s sputum microbiology studies—including culture, GeneXpert for tuberculosis, and MGIT culture—yielded negative results. Additional laboratory tests, such as the blood count and liver and kidney function tests, were within normal limits. Chest radiography showed no abnormalities. However, the sputum analysis revealed the presence of bile pigments.

Abdominal computed tomography and chest high-resolution computed tomography revealed consolidation with air bronchogram in the subpleural area of the right lower lobe, measuring 11 mm × 11 mm × 13 mm. This consolidation featured a central necrotic area with several air foci. A necrotic collection was noted that connected to segment VII of the liver via a transdiaphragmatic fistula tract measuring 25 mm × 7 mm. Additionally, a hepatic abscess measuring 14 mm × 14 mm × 12 mm was identified in the subcapsular region of segment VII of the liver. This abscess appeared to communicate with the biliary nerve roots, indicative of a bronchobiliary fistula with minimal communication (Fig. 1). Furthermore, a portal cavernoma was seen compressing the common bile duct, leading to intrahepatic biliary dilatation. The presence of multiple mesenteric collaterals and mild splenomegaly was also noted. During bronchoscopy, bile was confirmed in the right bronchus (Fig. 2).

Figure 1. Sagittal section of the chest with the abdomen showing a fistulous communication (arrow showing the fistulous track), with the liver abscess emerging transdiaphragmatically into the lung parenchyma.

Figure 2. Flexible bronchoscopic view of the bile seen in the right bronchus.

Endoscopic retrograde cholangiopancreatography was performed using a plastic biliary stent to create a low-resistance pathway, through which the abscess and fistula could be drained and sealed (Fig. 3). Our patient experienced immediate relief. His cough production almost completely subsided on the same day and he could sleep well.

Figure 3. Cholangiogram showing leak of contrast into the hepatic parenchyma.

The thrombophilia workup for the patient’s portal cavernoma cholangiopathy returned negative results. Gastroscopy showed a single small columnar varix accompanied by mild hypertensive gastropathy. Serological testing for amebiasis was negative.

The patient was treated with a course of antibiotics and discharged a few days later, with a prescription for oral antibiotics. One month after discharge, a follow-up abdominal ultrasound demonstrated that the abscess had resolved. Subsequently, a repeat cholangiogram was performed, which confirmed the closure of the previously identified fistulous communication.

The study was approved by the ethical committee of Lokmanya Tilak Municipal Medical College and Sion Hospital, Mumbai. Written informed consent was taken from the patient and his relatives.

Bilioptysis is a rare condition encountered in everyday clinical practice, yet it is pathognomonic for a bronchobiliary fistula. It was first documented by Peacock¹ in 1850. To date, only sixty-eight cases have been reported in the literature from 1980 to 2010.² Hepatic tumors, both primary and metastatic, are the most common causes of biliary-bronchial fistula formation, with bile duct obstruction as the second leading cause.² There have also been reports of congenital cases presenting in adults.³

In developing countries, echinococcal cysts, amebiasis, and pyogenic liver abscesses are likely the most common causes of bronchobiliary fistulas.⁴ One study reported that up to 2.4% of cases undergoing percutaneous biliary drainage developed bronchobiliary fistula as a complication of the procedure.⁵

Most bronchobiliary fistulas are attributable to lesions in the right lobe of the liver, particularly those associated with bile duct obstruction. Obstruction of the bile duct can lead to cholangitis, intrahepatic abscess formation, and subsequent rupture of the abscess into the pleural space or bronchi. This accounts for the predominant occurrence of bronchobiliary fistulas on the right side of the chest.⁴ However, there have been some exceptional cases where bronchobiliary fistulas are located on the left side.⁶ In addition to bilioptysis, patients may present with fever, abdominal pain, jaundice, pneumonia, and an intractable cough. Approximately 75% of patients with a broncho-biliary fistula may have undergone previous surgery or an invasive procedure before the development of the condition.² Treatment options include endoscopic retrograde cholangiography with plastic stenting, the placement of a naso-biliary drain, percutaneous drainage, or surgery.²

Eliminating the pressure gradient between the sphincter of Oddi and the common bile duct through sphincterotomy and/or plastic biliary stenting reduces the output of the fistula, thereby facilitating its rapid closure.⁴ However, a failure rate of approximately 10%–30% has been observed with both endoscopic and percutaneous methods.^7–9 There are two reported cases wherein bronchobiliary fistulas were treated using N-butyl cyanoacrylate using an endobronchial approach.^10,11

Surgical options include pulmonary lobectomy, resection, subdiaphragmatic exploration, complete exposure of the fistulous tract, hepatic lobectomy, hepaticoenterostomy, and abscess drainage, either alone or in combination. However, endoscopy is considered a safer alternative.² Surgical interventions should be pursued if endoscopic methods are unsuccessful in managing the disease.

Our patient presented with symptoms of bilioptysis, which were attributed to portal cavernoma cholangiopathy compressing the biliary tract. This compression led to the development of a liver abscess that subsequently ruptured into the bronchi. Endoscopic retrograde cholangiopancreatography with a plastic stent resolved his complaints almost instantaneously. Surgical options carry a higher risk of mortality; hence, the endoscopic method was preferred.

None.

The data that support the findings of this study are available from the corresponding author upon reasonable request.

No potential conflict of interest relevant to this article was reported.