Mirizzi syndrome is one of complicated situation that could encountered from a symptomatic gallstone disease. It was first described by Pablo Luis Mirizzi as the hepatic duct syndrome. This syndrome occurs when the impacted gallstone blocks the bile duct that compromise biliary flow. The incidence of this syndrome is quite rare, with data showed the less than 1% per year in patients with the gallstone. The impacted stone in the infundibulum of the gallbladder can obliterate the cystic duct and also causing other anomaly structures such as fistula formation around the biliary tree anatomy [1-3]. Biliopleural fistula is a rare condition which can arise in these circumstances [4]. This biliopleural fistula will cause accumulation of bile in pleural cavity, as we called as biliothorax. Biliothorax itself first introduced by Graham as broncho-biliary fistula. The presence of bile in the pleural cavity is damaging and can lead to empyema and even acute respiratory distress syndrome if not treated adequately. Most of the cases are found as right-sided pleural effusion and usually only unilateral because of the proximity to the liver and biliary system. However, bilateral involvement of pleural effusion due to this condition has also been reported, but extremely rare [5-7]. Biliary pleural effusion requires immediate attention, as it has a high potential to be associated with empyema that could lead to worse morbidities of the patients. Conservative non‐surgical treatment and adequate biliary drainage may be appropriate in these patients. Rapid diagnostic and optimal surgical strategies are essential for a successful outcome. Some approaches in complicated condition are still in debate and need further evaluation [8,9].

A 60-year-old female visited our emergency department with shortness of breath. The complaint was getting worse since about a week prior emergency department admission. She also had been experiencing intermittent right upper abdominal quadrant pain and fever since 3 months before admission. History of jaundice was denied. Chest radiograph study showed right pleural effusion. Mini chest tube thoracostomy was inserted to the right pleural cavity to treat the symptom and prevent further respiratory distress. During pleural chest tube insertion, about initial 600 cc of bilious liquid had been drainaged. After the respiratory problem was resolved, more diagnostic work up were scheduled to evaluate the possibility of biliary tract disease involvement in the patient. Abdominal magnetic resonance imaging revealed cystic lesion at the edge of right diaphragm that is connected to the fundus of gallbladder and suggested of impacted stone at the level of cystic duct (Fig. 1). Bilirubin total level was 1.62 mg/dL with predominance of direct bilirubin. At this point the diagnosis of Mirizzi syndrome was established and patient was prepared for elective cholecystectomy and abdominal exploration.

During the laparotomy, there was a lot of adhesions surrounding the hepatocystic area. After adhesiolysis, biliary tree anatomy was able to be identified. Fistula tract was found originated from the fundus of gallbladder that had a connection through the right diaphragm. There was also an impacted stone in cystic duct (Fig. 2). The fistula connection was then ligated, the diaphragm was closed using mattress sutured, and cholecystectomy was done (Fig. 3). The biliary flow was ensured to be patent and that there were no sign of common bile duct injury during the abdominal evaluation.

Patient was observed in the high care unit after the surgery. The vital signs remain stable during perioperative period. No respiratory distress was encountered, and chest tube production gradually decreased days after surgery and then safely removed after daily production declared normal by the pulmonologist. No jaundice and bilirubin were progressively back to normal level after the surgery. The patient was able to tolerate normal diet and then discharged after adequate mobilization. No other complications were documented.

Mirizzi syndrome is caused by impacted gallstone obstruction of the common bile duct or common hepatic duct. It usually causes bile stasis because when the stone causes external compression from infundibulum or Hartman’s pouch, there will be a blockage of the bile flow into the duodenum [1,2,8]. In 1948, Mirizzi proposed the term “hepatic duct syndrome” in the context of cholecystitis and cholelithiasis, described it as a mechanical obstruction of the gallbladder and inflammation of the infundibulum resulted in the contraction of a “muscular sphincter” in the common hepatic duct. In modern era, now it is known that there is no sphincter in the hepatic duct. The bile stasis in this syndrome is now believed to be the cause of external compression due to impacted stone of the adjacent bile duct [9,10].

There were a lot of changes in Mirizzi classification. At first, Beltrán [1] in 1982 classified the Mirizzi syndrome into two types based on endoscopir retrograde cholangio pancreaticography (ERCP) findings [10-12]. If the condition involves the external compression of the bile duct or cystic duct in the Hartmann’s pouch, it was categorized to type I. Type Ⅱ consists of a proper cholecystobiliary fistula in ERCP findings that is caused by a gallstone. The type II condition also included the stones that have eroded the bile duct. Beltrán [1] then revised the previous classification and weight the modification based on fistula condition and bile duct involvement. They classified the syndrome into four types. In type 1, the external compression cause bile stasis in the level of infundibulum or cystic duct, but no fistula was found. Type 2 consist of a fistula with less than one-third of bile duct circumference. Type 3 was classified if the circumference of the fistula reach up to two-third of the bile duct and type 4 is concluded when the cholecystobiliary fistula circumference is more than two-third until the complete destruction of the bile duct wall. Such condition will give devastating difficulties for the surgeon to recognize the dissecting plane of biliary anatomy.

Mirizzi syndrome could make things difficult to surgeon during surgery. Data showed that the incidence of bile duct injuries will increase in patients operated with Mirizzi syndrome without preoperative diagnosis, reaching as high as 17% [2,13]. Mirizzi usually associated with gallbladder inflammation and severe adhesion. This condition will lead to difficulties in identification of the biliary tract anatomy and therefore complications such as bile duct injury is more likely to occur [8,14]. Unfortunately, not all preoperative diagnosis of Mirizzi syndrome can be made. Sometimes the diagnosis has been made intraoperatively even though some imaging modalities has been provided before the surgery. Data showed that diagnosis of Mirizzi syndrome is difficult and can be made in only 9% to 60% of patients [9,10]. The diagnosis should be suspected based on the clinical characteristics, surgeon’s experience, and might be augmented by radiological imaging or ERCP [1]. Magnetic resonance cholangiopancreatography (MRCP) is also a useful modality to identify extrinsic compression of the bile duct and to determine if a fistula is present or not. MRCP could also able to rule out choledocholithiasis and other causes of bile tract obstruction, such as stenosis or even malignancy. Classic typical features of Mirizzi syndrome can be shown by MRCP such as the narrowing of the common hepatic duct due to extrinsic compression, gallstones in the cystic duct, diameter enlargement of the intrahepatic and common hepatic ducts, and normal bile duct. If the bile duct dilatation is present, we have to rule out other cause of the obstructive jaundice. MRCP sometimes can also predict the extent of the inflammatory process surrounding the gallbladder and therefore it has the advantage of avoiding the complications associated with endoscopic procedure. If the preoperative diagnosis is not made, we have to recognize the possibility of the syndrome intraoperatively, and proper management is very essential. Inadequate recognition of this condition will lead to high perioperative morbidity and mortality.

In the presented case, the diagnosis of the Mirizzi syndrome had been made in the preoperative setting. Patient was brought to the emergency department due the respiratory problem, so it was necessary to initially stabilize the respiratory distress. The chest tube drainage was needed to put the intrapleural pressure back to its natural negative pressure condition. History of right upper abdominal quadrant and elevated level of bilirubin suggested the disease of gallstone and bile flow obstruction, which confirmed by MRCP study that showed filling defect in the neck of infundibulum and also a connection from the gallbladder fundus through the right pleural cavity. We provided open surgery in this patient because we had predicted that intraabdominal adhesion will limit our identification of adjacent area due to severe inflammation. The history of respiratory distress in this patient, although had been resolved by the chest tube insertion, was also a factor to not prefer laparoscopic approach in this patient due to risk of greater pulmonological burden.

Biliothorax is the presence of bile fluid found in the pleural cavity. In 1897, this condition was first reported by Graham and known as broncho-biliary fistula, and some call it as cholethorax. The condition is a devastating entity if not recognized early. The presence of bile material in the pleural cavity is very damaging and also could lead to worse condition such as empyema and acute respiratory distress syndrome [4]. Various causes have been reported for biliothorax such as infection, thoracoabdominal trauma, neoplasm, and iatrogenic procedure such as percutaneous hepatic intervention. Post surgical biliopleural fistula formation have also been reported before, some of which were related to complicated hepatobiliary surgery. The cause was believed due to diaphragm defect that cause any bile leak into the pleural cavity.

Within physiologic condition, normal pleural cavity may contain a small amount of fluid, usually less than 20 cc and has negative pressure. If there is any defect in the pleural cavity, the negative pressure will be disrupted. Biliopleural fitula is one of a rare cause of pleural effusion. Most of the fistula are associated with biliary malignancy or benign biliary obstruction that cause biliothorax [6]. Due to anatomical proximity, pleural effusion related biliopleural fistula usually encountered in the right side, but bile fluid can also drainage naturally through the oesophageal and aortic hiatuses and involve the left pleural cavity. The most specific diagnostic criteria for this condition is pleural fluid analysis. The ratio of total pleural fluid bilirubin to total serum bilirubin greater than 1 is confirmation of the diagnosis of biliothorax [4,5]. Biliothorax due to biliopleural fistula should be suspected in patients who have undergone hepatobiliary procedures and they who have complicated biliary disease. Contrast spillage through the biliary tree into the pleura during ERCP is noted and is confirmatory of the diagnosis.

The most optimal treatment of biliothorax until present day is surgical intervention, with closing the fistula give us a high rate of success. Until now, there is still no guidelines for the treatment of this rare entity. Some also reports the importance of conservative approach to appropriately drainage the pleural effusion using the chest tube. Intra-abdominal drainage may also be required to drainage the bile externally instead of towards the pleura [5,6].

In this case, since there was a biliary fistula through the pleura, we think that surgical approach is mandatory to close the connection between those two adjacent organs. Cholecystectomy (total/subtotal) and sometimes biliodigestive bypass might be needed in some circumstances.

Biliopleural fistula is a rare complication of biliary tract obstruction that requires multidisciplinary attention. The best treatment strategy for complicated biliopleural fistula should be weigh on patient’s condition.