Case of bilioptysis following hydatid cyst removal

Abstract

Bilioptysis is a rare condition characterized by the presence of bile in the sputum, resulting from an abnormal fistulous connection between the biliary tree and the bronchus (biliobronchial fistula). Biliobronchial fistula is associated with high morbidity and mortality; hence, early diagnosis and timely treatment are crucial. We present a case of a 46-year-old male who presented with complaints of bilioptysis for 1 day. He had a history of a hydatid cyst of the liver, which was surgically treated 2 years ago. The patient subsequently developed recurrent fever and vomiting due to a persistent subdiaphragmatic collection. Multiple attempts to drain the abscess/collection were made using percutaneous drainage and endoscopic retrograde cholangio pancreatography (ERCP) with stenting to relieve intrahepatic biliary obstruction. Despite these interventions, the patient developed intrahepatic biliary radical (IHBRD) dilatation, which led to bile tracking through a diaphragmatic rent likely caused by percutaneous drain insertion, resulting in a fistulous connection with the right anterobasal and basolateral bronchial segments. A contrast computed tomography (CT) scan of the thorax and abdomen showed fistulous tract communication to lung parenchyma. He underwent an operative intervention, during which the fistulous tract was excised under intraoperative bronchoscopy guidance. This was followed by the excision of lung segments with a fistula and the repair of the diaphragmatic rent. ERCP and stenting were also done in the same sitting for source control. The patient was discharged with complete resolution of bilioptysis.

Introduction

Biliobronchial fistula is a rare complication seen due to an abnormal communication between the biliary tree and the bronchus [1]. Typically resulting from liver abscesses, it may also be seen with liver tumors [2]. The first case was described by Peacock in 1850 [3]. Ever since, only a handful of case reports and series have been published regarding this condition. Although rare, bronchobiliary fistulae carry a high mortality and morbidity rate of 12.5%, necessitating the need for early diagnosis and definitive treatment [2]. The pathognomic presenting symptom is bilioptysis, which should raise suspicion of a biliobronchial fistula.

Surgical treatment is the mainstay in biliobronchial fistula. Herein, we present a case of delayed development of an acquired biliobronchial fistula after liver hydatid cyst removal where both infective and obstructive biliary drainage etiology coexist, making it a conundrum as to which line of treatment should be taken.

Case presentation

A 46-year-old male presented to causality with massive bilioptyis and fever. He was continuously coughing out bile and unable to lie supine. He was a postoperative case of liver hydatid cyst enucleation with omentoplasty done 2 years ago. Subsequently, the patient underwent endoscopic retrograde cholangio pancreatography (ERCP) with stenting of the left hepatic duct due to high output from a subhepatic drain. He was discharged with a subdiaphragmatic drain in situ. The subhepatic drain was removed in the follow-up visit when the drainage had subsided. However, the patient returned to the hospital with complaints of weight loss after 3 months. An ultrasound (USG) of the abdomen revealed a subdiaphragmatic collection with mild right-sided pleural effusion. A USG-guided percutaneous drain (PCD) was inserted into the subdiaphragmatic space for drainage of the bilious collection by an intervention radiologist.

Over the following year, the patient presented multiple times with recurring episodes of vomiting, nausea, and fever, which were either associated with PCD dislodgement or a sudden increase in drain output. Magnetic resonance cholangio pancreatography (MRCP) during these episodes revealed intrahepatic biliary radicle dilatation. The patient underwent ERCP + Stenting four times owing to obstruction and disruption of intrahepatic biliary architecture on the right side with discontinuity in segments VII and VIII with substance defect in segments VIII and IV. The collection above the liver later turned purulent, and an aspirate tested positive for Escherichia coli (E. coli) and Klebsiella. Incision and drainage were performed from the previously healed PCD site. The patient was symptomatically better until 1 day before admission when he developed sudden onset of cough with copious amounts of yellowish green sputum.

Thoracic surgery consultation was sought in view of bilioptyis, and due evaluation with a contrast-enhanced computed tomography (CECT) scan of the chest and abdomen was done to identify the biliobronchial communication. CECT chest (Fig. 1) revealed the presence of a biliobronchial fistula to right latero-basal and antero-basal bronchus through a diaphragmatic rent, likely from the previous PCD insertion path, with associated consolidation of basal segments of the right lung. Routine blood investigations on admission revealed an elevated total white blood cell (WBC) count with tachycardia, fever, and suboptimal blood pressure suggestive of systemic inflammatory response syndrome (SIRS)—bilious sepsis.

Fig. 1.

Contrast enhanced computed tomography of chest depicting the biliopulomary fistulous tract

Our protocol for managing this case involved routine blood work, initiation of appropriate supportive care, imaging with CECT thorax to diagnose and delineate the fistulous tract, and an intra-operative bronchoscopy-guided segmentectomy with fistulous tract excision.

Given the high output nature of the fistula with frank sepsis and contamination of lung parenchyma with bile, a definitive urgent surgical management was considered to be prudent. After obtaining informed consent, the patient was taken up for surgery the next morning.

The patient was operated on with elective single lung ventilation using a left-sided double lumen endotracheal tube (DLT). Fiber optic bronchoscopy was done prior to skin incision, which showed golden yellow biliary fluid bubbling through the right lower lobe bronchus. A posterolateral thoracotomy was done through the 6^th intercostal space. The incision was made in a fish-mouth fashion around the previous PCD scar site, which contained the outer opening of the sinus tract. A probe was inserted through the sinus to delineate the tract. A thick-walled cavity with fluid was noted to have adhered to the liver (through the diaphragmatic rent) and to the middle lobe of the right lung (Fig. 2). Two fistula tracts were identified on careful evaluation and confirmed with cannulation of the tract with an infant feeding tube and instillation of saline and demonstration of the backflow of the fluid with on-table fiber optic bronchoscopy (Fig. 3). The fistula tracts were excised along with the involved segment of the middle and lower lobes of the right lung (Fig. 4). Diaphragmatic rent was repaired with non-absorbable sutures, which sealed off the pleural cavity from the peritoneal cavity. After securing two drains in the right hemithorax, standard thoracotomy incision closure was done.

Fig. 2.

Intra-operative image (a) displaying the fistulous tract from the diaphragm to the lung and schematic representation of the intra-operative image (b)

Fig. 3.

Operative photograph shows probe within the fistulous tract (a), schematic illustration of intra-operative image (b)

Fig. 4.

Excised fistulous tract

In the same sitting, ERCP + Stenting was done to relieve obstruction to the bilious drainage from the liver for source control of bile leak.

Post-operative course

The patient had immediate relief of bilioptysis after surgery. The patient was mobilized by post operative day (POD) 1 and was discharged from the hospital in stable condition on POD- 4 with one intercostal drain (ICD) in situ connected to a negative suction device. The patient has been asked to follow-up with the gastro-intestinal surgery team to plan definitive treatment for intra-hepatic biliary collection at a later setting to prevent recurrence of the symptoms.

Discussion

Bilioptysis is a startling clinical manifestation of a rare condition called biliobronchial fistula. Although this condition was first described as early as 1850 by Peacock [3], its etiologic profile has evolved significantly over time. Historically, biliobronchial fistulas were most commonly associated with complications of hydatid cysts. However, in the present era, primary and metastatic hepatic tumors have emerged as the leading cause, followed closely by biliary obstruction due to cholangiolithiasis and stenosis. Notably, the incidence of hepatic hydatidosis as an etiology has significantly declined in recent years. A meta-analysis by Liao et al. shows that only eight cases have been reported to be due to hepatic hydatidosis in the last 30 years [4]. Our case is unique in that it presents a multifactorial etiology—a surgically treated hydatid cyst in conjunction with a bacterial subphrenic abscess and biliary obstruction—that, to our knowledge, has not been previously reported. (PubMed Library was searched for MeSH word “post operative biliobronchial fistula” which retrieved two case reports where either the fistula was detected during the primary surgery for hydatid cyst or had only one of the three etiologies mentioned in ours.)

While surgical management has long been the cornerstone of treatment for this condition, several authors have underscored the utility of endoscopic procedures such as ERCP and stenting with or without sphincterotomy in both diagnosing and managing such fistulas [5–7]. ERCP can be employed both diagnostically and therapeutically, with placement of fully covered self‐expandable metal stents offering temporary control by diverting bile flow away from the fistula. However, these less invasive options often end up with prolonged hospital stays and multiple re-interventions [8], as also seen in our case. Owing to the physiology of negative pressure created by the thoracic cavity, admixed with the erosive nature of hydatoid and bilious subdiaphragmatic collections, biliary tree drainage at present can be used as an adjunct and not the sole modality of choice for treating such complicated fistulae.

Conclusion

Biliobronchial fistula, when suspected clinically, should be evaluated at the earliest and surgical management offers the best chance for salvaging the lung. Combining surgical with endoscopic intervention, like in our case, could ensure a greater post-operative success.

Author contributions

Conception and Design: Robin B Poovattil, Abhishek Mohan, Shyam Rengan. Acquisition of data: Robin B Poovattil, Abhishek Mohan, Shyam Rengan. Analysis and Interpretation: S Bal, Roman Dutta, Rohit Rathi, Robin B Poovattil, Abhishek, Shyam Rengan. Drafting of article: Robin B Poovattil. Revision: S Bal, Roman Dutta, Rohit Rathi. Final Approval of completed article: Robin B Poovattil, Abhishek, Shyam Rengan, Rohit Rathi, Roman Dutta, S Bal.

Funding

Nil.

Data Availability

All relevant data pertaining to the case report are available within the manuscript.

Declarations

Conflict of Interest:

The authors declare that they have no competing interests.

Informed consent:

Informed consent was obtained from the patient.

Ethics Committee Approval

N/A.

Statement of Human and Animal Rights

Not Applicable.