A congenital or acquired bilio-bronchial fistula (BBF) is an abnormal communication between the biliary tract and respiratory system. The most prevalent type is acquired BBF, which is caused by hydatid cysts of the liver, amoebic liver abscess, liver cancer, choledocholithiasis-related strictures, and interventions such as tumor resection or ablation, radiation, and chemotherapy. Congenital BBF is rare and only a few cases presenting in adulthood have been described. We describe a brief review of the related studies on congenital BBF and report a case of recurrent BBF in a young woman who was effectively treated with laparoscopic fistula tract excision and in addition.

A literature review was conducted for comparable cases in Medline between 1850 and 2023, using the following keywords: bronchobiliary fistula, BBF, biliary-bronchial fistula, tracheobiliary fistula, hepatobronchial fistula, bronchopleural fistula, congenital, and bilioptysis. Two reviewers independently searched the literature to identify articles appropriate for inclusion in this review. Further articles were identified through cross-referencing. As a result, 42 cases were reviewed and data on demographic details, type of fistula, associated anomaly, management, and reported outcomes were collected. The data was abstracted into the evidence table template (Table 1 [1-39]).

Table 1 . Reported cases of congenital bilio-bronchial fistula with management.

Case No.	Study	Age at diagnosis	Sex	Type of fistula	Associated anomaly	Diagnosis	Treatment	Outcome
1	Neuhauser et al., 1952 [1]	5 months	Girl	RMB	Nil	Sputum bile positive fluoroscopic examination	Medical	Died
2	Enjoji et al., 1963 [2]	7 months	Boy	Carina	Nil	Autopsy	Laparotomy, thoracotomy	Died
3	Weitzman et al., 1968 [3]	2 years 9 months	Boy	RMB	Nil	Bronchoscopy	1. Heineke-Mikulicz pyloroplasty and a feeding jejunostomy 2. Right thoracotomy and excision of thoracic part of fistulous tract	Survived
4	Wagget et al., 1970 [4]	21 days	Girl	LMB	Nil	Bronchoscopy	Thoracotomy and excision of thoracic part of fistulous tract	Survived Involution of the left lobe of liver
5	Sane et al., 1971 [5]	28 days	Girl	-	Nil	Radiology	Surgical ligation and partial excision of the communication	Survived
6	Kalayoğlu and Olcay, 1976 [6]	4 days	Girl	RMB	Proximal esophageal atresia and distal tracheoesophageal fistula	Radiology, surgical exploration	Thoracotomy, cervical esophagostomy and distal tracheoesophageal division, and gastrostomy Excision of thoracic part of fistulous tract	Died
7	Chan et al., 1984 [7]	4 days	Girl	RMB	Biliary obstruction	Fluoroscopic examination, autopsy	Thoracotomy, excision of thoracic part of the fistulous tract	Died
8	Chang and Giulian, 1985 [8]	7 days	Boy	RMB	Nil	Bronchography	Thoracotomy and excision of thoracic part of fistulous tract	Survived
9	Lindahl and Nyman, 1986 [9]	3 days	Girl	RMB	Nil	Bronchoscopy	Thoracotomy and excision of thoracic part of fistulous tract	Survived
10	Levasseur and Navajas, 1987 [10]	22 years	Female	Carina	Nil	Bronchoscopy, CT	Thoracotomy and excision of thoracic part of fistulous tract with pneumonectomy	Survived
11	de Carvalho et al., 1988 [11]	32 years	Female	RMB	Nil	Sputum bilirubin, bronchoscopy, HIDA scan, cholangiography	Thoracotomy and excision of thoracic part of fistulous tract	Survived
12	Yamaguchi et al., 1990 [12]	32 years	Male	RMB	Nil	Bronchoscopy	Thoracotomy and excision of thoracic part of fistulous tract	Survived
13	Gauderer et al., 1993[13]	Case 1: 7 years Recurrence (case 5)	Female	RMB	Left biliary obstruction	Bronchoscopy	7 years: thoracotomy, excision of fistulous tract 1 year later: third thoracotomy, biliary cyst drainage f/b laparotomy, left hepatic lobe resection	Survived
14		Case 2: 23 months	Boy	Carina	D/D - GERD Left biliary obstruction	Bronchoscopy	9 months: fundoplication and gastrostomy 23 months: thoracotomy and excision of thoracic part of fistulous tract f/b laparotomy and left hepatic lobe resection	Survived
15		Case 3: 1 year	Girl	LMB	D/D - GERD Left biliary obstruction	HIDA scan, bronchoscopy	1 year: fundoplication and gastrostomy Few weeks later: thoracotomy and excision of the thoracic part of the fistulous tract 2 days later: laparotomy and RNY fistulojejunostomy	Survived
16	Ferkol et al., 1994 [14]	23 months	Boy	LMB	D/D - GERD Left biliary obstruction	Bronchoscopy, bronchography, biliary secretion positive for bile salts	10 months: fundoplication and gastrostomy 23 months: 2-stage thoracotomy and excision of thoracic part of fistulous tract f/b laparotomy and resection of distal part of fistulous tract with associated liver segments	Survived
17	Tekant et al., 1994 [15]	15 days	Girl	Carina	Extrahepatic biliary obstruction	Bronchoscopy	Thoracotomy and excision of thoracic part of fistulous tract f/b laparotomy with cholecystojejunstomy	Survived
18	Egrari et al., 1996 [16]	1 day	Girl	Carina	Nil	Bronchial aspirate positive for bile, HIDA scan, bronchography	Thoracotomy and excision of thoracic part of fistulous tract f/b minilaparotomy with cholecystography	Survived
19	Fischer, 1998 [17]	16 days	Girl	LMB	D/D - GERD	UGI endoscopy, 24-hr pH study, bronchoscopy, bronchogram, HIDA scan	Fundoplication, thoracotomy and excision of thoracic part of fistulous tract	Survived
20	Tommasoni et al., 2000 [18]	Case 1: 21 months	Girl	Carina	D/D - GERD	Bronchoscopy, fistulography	14 months: fundoplication 21 months: thoracotomy and excision of thoracic part of fistulous tract	Survived
21		Case 2: 30 months	Boy	Carina	D/D - celiac disease	Bronchoscopy, fistulography	Thoracotomy and excision of thoracic part of fistulous tract	Survived
22	Duong et al., 2000 [19]	3 years	Girl	Carina	Nil	Bronchoscopy, HIDA scan	Thoracotomy and excision of thoracic part of fistulous tract	Survived
23	DiFiore and Alexander, 2002 [20]	1 day	Boy	Carina	Right-sided CDH	Laparotomy	Right subcostal incision, diaphragmatic hernia contents reduced and fistulous tract excised and primarily closed	Survived
24	Hourigan et al., 2004 [21]	13 days	Boy	RMB	Left biliary obstruction	MRI	Thoracotomy and excision of thoracic part of fistulous tract f/b laparotomy with cholecystofistulostomy	Survived
25	Aguilar et al., 2005 [22]	6 years	Girl	Carina	Nil	HIDA scan	Thoracotomy and excision of thoracic part of fistulous tract	Survived
26	Uramoto et al., 2008 [23]	65 years	Female	Carina	Nil	Bronchoscopy, MDCT, cholangiography, cholescintigraphy	No surgery	Survived
27	Chawla et al., 2008 [24]	1 day	Boy	Carina	Extrahepatic biliary obstruction	Bronchoscopy, MDCT, MRCP	Thoracotomy and excision of thoracic part of fistulous tract 1 month later: laparotomy and cholecystoduodenostomy	Survived
28	Günlemez et al., 2009 [25]	9 days	Girl	LMB	Extrahepatic biliary obstruction	MDCT, HIDA scan	Thoracotomy and excision of thoracic part of fistulous tract f/b laparotomy with cholecystojejunstomy	Survived
29	Najdi et al., 2009 [26]	6 days	Girl	Carina	Nil	Bronchoscopy, CT	Day 26: thoracotomy and excision of thoracic part of fistulous tract	Died
30	Croes et al., 2010 [27]	5 days	Girl	Carina	Left biliary obstruction	Bronchoscopy	Thoracotomy and excision of thoracic part of fistulous tract f/b RNY hepaticojejunostomy	Survived
31	Tan et al., 2010 [28]	51 years	Female	RMB	Nil	Bronchoscopy, ERCP, CT (abdomen and thorax)	Thoracotomy and excision of thoracic part of fistulous tract	Survived
32	Sachdev et al., 2011 [29]	2 years 10 months	Boy	Carina	Nil	Bronchoscopy, CT, HIDA scan	Thoracotomy and excision of thoracic part of fistulous tract	Survived
33	Kumagai et al., 2011 [30]	28 days	Boy	Carina	Nil	Bronchoscopy, MRCP	Day 29: thoracotomy and excision of thoracic part of fistulous tract At 2 months: transverse upper abdominal incision, excision of remnant sac	Survived
34	Yu et al., 2015 [31]	2 days	Boy	Carina	Left ventricular hypoplasia Extrahepatic biliary obstruction	Bronchoscopy	Day 5: Thoracotomy and excision of thoracic part of the fistulous tract 2 months later: bilateral percutaneous drainage f/b hepaticojejunostomy	Survived
35	Na et al., 2016 [32]	18 years	Male	RMB	Nil	CT, bronchoscopy	Laparoscopic excision of abdominal part fistulous tract f/b thoracoscopic excision of fistulous tract	Survived
36	Pérez et al., 2016 [33]	22 days	Boy	RMB	Extrahepatic biliary obstruction	Bronchoscopy, CT	Thoracoscopic excision of thoracic part of the fistulous tract 11-days later: left hepatectomy and bilio-enterostomy	Survived
37	Netto et al., 2018 [34]	21 days	Girl	Carina	Left biliary obstruction	Bronchoscopy, CT	Thoracotomy and excision of thoracic part of fistulous tract f/b external deviation of left biliary tree, elective, left hepatectomy	Survived
38	Li and Zhang, 2019 [35]	5 days	Girl	RMB	Nil	CT, bronchoscopy	Laparoscopic biliary tract exploration and thoracotomy and excision of thoracic part of the fistulous tract	Survived Postoperative cholestasis treated with steroids
39	Wang et al., 2020 [36]	42 months	Girl	RMB	Nil	CT/MRI, UGI scopy, bronchoscopy	VATS assisted excision of thoracic part of the fistulous tract	Survived
40	Thuong Vu et al., 2021 [37]	2 months	Girl	Carina	Nil	CT, bronchoscopy	Thoracotomy and excision of thoracic part of fistulous tract	Survived
41	Bing et al., 2021 [38]	2 years	Boy	RMB	Nil	CT, bronchoscopy	VATS assisted excision of thoracic part of the fistulous tract	Survived
42	Wilson et al., 2021 [39]	4 days	Girl	Carina	Nil	Bronchoscopy, MRCP	VATS assisted excision of thoracic part of the fistulous tract	Survived

RMB, right main bronchus; LMB, left main bronchus; D/D, differential diagnosis; GERD, gastroesophageal reflux disease; CDH, congenital diaphragmatic hernia; CT, computed tomography; HIDA, hepatobiliary iminodiacetic acid; UGI, upper gastrointestinal; MRI, magnetic resonance imaging; MDCT, multidetector computed tomography; MRCP, magnetic resonance cholangiopancreatography; ERCP, endoscopic retrograde cholangiopancreatography; f/b: followed by; RNY, Roux-en-Y; B/L: bilateral; VATS, video-assisted thoracoscopic surgery..

A 24-year-old female patient was admitted with yellowish-colored expectoration. She had a medical history of recurrent chest infections managed with medication, dating back to her infancy. She appeared to have an average build and nutrition status. Biphasic coarse crepitation was observed when the left chest was auscultated. Her chest radiograph revealed left lung non-homogenous opacities and ectatic alterations, along with signs of left lung volume loss and compensatory right lung hyperinflation (Fig. 1). The entire left lung parenchyma was replaced by varicoid and cystic bronchiectatic alterations on contrast-enhanced computed tomography (CECT). Upper abdominal sections showed pneumobilia in the central hepatic duct and bile ducts with abnormal air-filled fistulous communication between the left main bronchus and left bile duct. A hepatobiliary scintigraphy was done which confirmed bile tracking into the airways and unobstructed biliary drainage into the duodenum. The bile was seen seeping to the left main bronchus through a 6-cm-long fistulous tract with a tapering end during fiber-optic video bronchoscopy. The fistula’s smooth, cartilage-free wall resembled that of the esophagus.

Figure 1. Chest X-ray posteroanterior view showing non-homogenous opacities and ectatic changes in the left lung, with left lung volume loss and compensatory right lung hyperinflation.

The patient was diagnosed with congenital BBF with a destroyed left lung due to cystic bronchiectasis. She was initially treated in the thoracic unit with bronchoscopy-guided glue obliteration of the fistulous tract. After wedging the bronchoscope at the mouth of the fistula, a catheter was advanced into the distal end of the fistula and 1-mL n-butyl-2-cyanoacrylate glue was instilled through the catheter into the fistula. Her symptoms improved, and after 3 months, she was optimized and underwent definitive open left thoracotomy and pneumonectomy, with removal of the tract identified as a fistula. However, her bilioptysis symptom recurred a month later.

She was reevaluated with repeat imaging, which showed a fistulous tract extending from the left bile duct to the left bronchial stump. Fiber-optic bronchoscopy also confirmed the same (Fig. 2). An endoscopic retrograde cholangiogram demonstrated the fistula tract, and biliary stenting was done (Fig. 3). Despite stenting, her symptoms did not improve. She underwent laparoscopic extrahepatic fistulous tract excision (Supplementary Video). Intraoperatively, a fistula tract was identified in the gastrohepatic fold, extending caudally from the left hepatic duct towards the diaphragm (Fig. 4). The tract was deperitonealized and looped. The stent was removed by fistulotomy, and the tract was excised with linear staplers at both ends (Fig. 5). Postoperative course was uneventful, and the patient was discharged on day 4. She is asymptomatic at 1 year and on follow-up.

Figure 2. Tracheal bifurcation with bilioptysis from the left bronchial stump.

Figure 3. The multiplanar reconstruction computed tomography (coronal view) of the fistulous tract extending from the left hepatic duct to the left main bronchus stump with stent in situ.

Figure 4. Schematic representation of fistula tract course and relationship to adjacent structures.

Figure 5. Intraoperative image with fistulous tract looped and stent removed by fistulotomy.

Historical perspective and demographic overview of congenital bilio-bronchial fistula

The first case of bronchobiliary fistula was described by Peacock [40] in 1850 as a hydatid cyst of the liver communicating with the lungs. However, congenital BBF was first reported much later in the 1950s by Neuhauser et al. [1], following which a total of 42 cases of congenital BBF have been reported in the literature. Basic demographic details of the cases published on congenital BBF are summarized in Table 1. Out of the 42 congenital BBF cases, the majority (n = 27) were females. Most cases are diagnosed in neonates and infancy, with few adult patients up to 65 years also reported. Levasseur and Navajas [10] first reported congenital BBF in an adult patient in 1987. Although our patient had symptoms since childhood, she was not evaluated with imaging, and the diagnosis was made at the age of 25 years. These fistulas opened mainly in the carina (n = 20, 48.7%), right main bronchus (n = 16, 38.1%), and rarely in the left main bronchus (n = 5, 12.2%). The histological features of the fistula resemble those of the respiratory tract at the tracheobronchial end (cartilage, respiratory glands, and smooth muscle), whereas it shows features of the gastrointestinal tract at the biliary end (stratified squamous epithelium) [18,35].

Pathophysiology of congenital bilio-bronchial fistulas

Distal physiological obstruction of bile at the ampulla results in poor drainage into the duodenum and causes pressure in the biliary tree and retrograde bile flow, resulting in a congenital rudimentary tract. Bile outside the bile duct is an irritant that causes inflammation, irritation, and necrosis of the diaphragm. It then erodes the bronchus and creates the fistulous tract between the lung and the liver. The persistence of this fistulous communication is favored by the pressure gradient due to the positive pressure in the bile duct and negative pressure in the bronchus [41].

Clinical manifestations and diagnostic approaches in bilio-bronchial fistulas

The symptoms include cough, bilioptysis, fever, and pain. Bilioptysis is often mistaken for purulent expectoration if the expectoration is not examined by the physician. Few cases were misdiagnosed as reflux disease and had surgery for gastroesophageal reflux disease. Electrolyte imbalance and fat malabsorption can also occur. In our patient, recurrent respiratory tract infections since childhood pointed toward a congenital origin of the fistula. Chest radiographs frequently show right-sided pathology such as right pleural effusion, basilar atelectasis, or lung abscess [41]. However, our patient had left lung destruction with bronchiectatic changes, due to long-standing bile regurgitation, which caused pneumonitis.

CECT reveals a fistulous tract between the lung and the liver, and air within the biliary tree (pneumobilia). The coronal sections provide a better view of the tract. It is important to look for biliary tract anomalies, such as biliary atresia. Bronchoscopy is a definitive diagnostic procedure. The bronchial and tracheal mucosa may exhibit a yellowish tint at the opening of the fistula tract in the respiratory tract, which can be seen along with bile seeping from the opening. BBF can be confirmed by endoscopic retrograde cholangiopancreatography, percutaneous transhepatic cholangiography, bronchography, magnetic resonance imaging, or hepatobiliary nuclear imaging [16,21]. Endoscopic cholangiogram with biliary stenting and bronchoscopy-guided glue instillation may be added to delay surgery in premature infants or unsuitable patients as temporary solutions.

Evolution of surgical management in bilio-bronchial fistulas

Surgical excision of the fistulous tract is the definitive treatment. Initially, the patients were managed with right thoracotomy and excision of the thoracic fistulous tract. Gauderer et al. [13], in a series of three patients, showed recurrent fistula or residual cyst in patients treated with resection of the thoracic part of the tract alone due to an associated biliary tract anomaly. Recently, surgeons have combined laparotomy with thoracotomy to establish biliary continuity and remove the thoracic portion of the fistula. In our review of 42 patients, about one-third had associated biliary abnormalities in the form of distal biliary obstruction or left biliary obstruction necessitating additional biliary drainage or hepatic resection. With technological advancements, there have been recent case reports of minimally invasive surgeries. Na et al. [32] first described thoracoscopic and laparoscopic excision of thoracic and abdominal parts of the fistulous tract simultaneously in 2016. In the abdomen, the fistula tract runs cranially in the gastrohepatic fold from the left hepatic duct towards the diaphragm [2]. Furthermore, in BBFs, the biliary end is the high-pressure region, and it should be excised to avoid disease recurrence. Finally, additional surgical evaluation may be required in this rare illness, to determine whether transabdominal fistulous tract excision with intraoperative cholangiogram is adequate in eluding thoracotomy or thoracoscopy for unresolved cases.

BBF is a rare and complex anomaly that affects the quality of life of the patient. Bronchoscopy and radiological imaging along with hepatobiliary scintigraphy can diagnose and delineate the extent of the fistula. Laparoscopic excision is the least morbid and most effective procedure for managing congenital BBF.

Ethical statements

Since this study is a case report with a surgical description that reviewed electronic media and computed tomography readings, and personal information protection measures are well-established. Written informed consent was obtained from the patient for clinical research and publication of information anonymously.

Authors’ contributions

Conceptualization: BP, DPD

Data curation: CM, MB

Formal analysis, Investigation, Methodology, Visualization: All authors

Project administration: BP, DPD, HM, PM

Writing–original draft: CM, MB

Writing–review & editing: BP, DPD, HM, PM

All authors read and approved the final manuscript.

Conflict of interest

All authors have no conflicts of interest to declare.

Funding/support

None.

Acknowledgments

The authors appreciate Prof. Ram Kumar and Dr. Selva Ganesan for providing computed tomography reconstruction images. The image that constitutes Fig. 3 was provided by the Department of Radiology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.

Data availability

The data presented in this study are available upon reasonable request to the corresponding author.

Supplementary materials

Supplementary materials can be found via https://doi.org/10.7602/jmis.2024.27.1.1.