Challenges in the diagnosis and management of spontaneous bile duct perforation: A case report and review of literature.

We report a case of 14-year-old male, who presented to us with complaints of severe pain in abdomen, vomiting, and inability to pass feces and flatus. He was diagnosed as a case of peritonitis after careful history, examination, and investigations. The exact cause of peritonitis was not known. Exploratory laparotomy was done, and it was found that there was perforation of the right hepatic duct about 1 cm proximal to its confluence with the left hepatic duct. Perforation was closed around the T-tube. Postoperative T-tube cholangiogram was done after 3 weeks which confirmed the free passage of dye into the duodenum, and there was no leakage of dye. T-Tube was removed 4 weeks after the operation, and the patient was discharged in satisfactory condition. Postoperative follow-up was done for 3 months, and it was uneventful.

INTRODUCTION

Spontaneous perforation of the biliary tract and the resulting bile peritonitis, though rare, are known to affect the extrahepatic biliary tract.[1] The majority of these perforations occur in the gall bladder and are because of gallstones.[2] Because of the rarity of the condition and lack of supportive investigations, preoperative diagnosis is usually not possible in most of the cases and diagnosis is only made at the time of laparotomy.[3] We present a case with biliary peritonitis due to spontaneous rupture of the right hepatic duct in which the diagnosis was made on exploratory laparotomy.

CASE REPORT

A 14-year-old boy with no any previous medical history presented to emergency and accident department with complain of severe pain in right upper abdomen, abdominal distension, low-grade fever, nausea, and nonbilious vomiting for last 2 days. On clinical examination, vitals at the time of presentation were pulse rate 112/min, blood pressure 100/60 mmHg. On per abdominal examination, there was tenderness along with guarding and rigidity all over the abdomen but there was no masking of liver dullness. The preliminary investigations were unremarkable except for raised total leukocyte count (14 × 109/L). The blood investigations showed total bilirubin 1.2 mg/dl (≤1.2 mg/dl), direct bilirubin 0.6 mg/dl, indirect bilirubin was 0.6 mg/dl, alkaline phosphatase 151 U/L (38-126U/L), alanine transaminase 40 U/L (9-52 IU/L), aspartate transaminase 29 U/L (15-46 IU/L) (Figures in brackets indicate normal range).

Plain X-ray of abdomen did not show any evidence of pneumoperitoneum. Ultrasonography revealed a large amount of fluid collection with low-level internal echoes, septations, and loculations on the right side of the peritoneal cavity. The gall bladder was distended, grossly edematous and had moderate pericholecystic fluid. The intra-hepatic biliary radicles were normal, and the common bile duct (CBD) was obscured by bowel gas. Ultrasound-guided abdominal paracentesis was done, and it revealed bilious collection in the peritoneal cavity. Provisional diagnosis of peritonitis was made, and emergency exploratory laparotomy was planned and done.

Per operatively, 2 l of free intraperitoneal bile was found. Whole of viscera was stained with bile. Stomach, small bowel, large bowel up to the rectum was normal. Gallbladder was also found to be normal, and there were no stones inside its lumen. A perforation was found on the anterior aspect of the right hepatic duct approximately 1 cm proximal to its confluence with the left hepatic duct. The perforation involved about one-third of the circumference of right hepatic duct. The diameter of CBD was normal; there was no congenital abnormality and neither there was any calculus present in it. Furthermore, no calculus was found free in the peritoneal cavity.

Right hepatic duct was drained through perforation by T-Tube [Figure 1] after checking the patency of ampulla. The right subhepatic space and pelvic region were drained. Postoperatively, recovery of the patient was good, and he was allowed orally on 2nd day. On the 5th postoperative day, bile started leaking in the abdominal drain. T-tube cholangiography was done to confirm the position of limbs inside the hepatic duct, and it was found to be well in place, but there was leakage of bile alongside the T-tube. The quantity of bile gradually decreased, and it stopped in about 3 weeks after the operation. T-tube was removed 4 weeks after the operation when cholangiogram was repeated and free passage of contrast inside the duodenum was confirmed [Figure 2].

Figure 1

Peroperative image of the patient showing T-tube drainage of the right hepatic duct through the perforation

Figure 2

Postoperative T-tube cholangiogram of the patient showing T-tube in right hepatic duct and free passage of contrast into the duodenum

DISCUSSION

First series of spontaneous perforation of biliary tract was reported by McWilliams in 1912.[4] In his series, he reported 114 cases of spontaneous perforation of the biliary tract out of which majority of the patients were having gallbladder perforation, four patients were having CBD perforation and only one patient was having hepatic duct perforation. Lochan and Joypaul in 2005 reported a case with perforation of the intrahepatic portion of left hepatic duct.[5] Khanna et al. in 2010 reported a patient with choledocholithiasis who presented with acute abdomen due to perforation of the supraduodenal part of the CBD.[6] Mizutani et al. in 2011 reported a case of perforation of left hepatic duct secondary to stones in which he closed the perforation around the T-tube which was removed after 25 days.[7] In our patient also, the perforation was closed around the T-tube. In none of the above-mentioned case reports, condition was diagnosed preoperatively. Diagnosis was confirmed only upon doing exploratory laparotomy.

Perforation of the hepatic duct in the absence of the iatrogenic injury or severe trauma is extremely rare cause of bile peritonitis.[3] Several possible mechanisms of spontaneous perforation of the biliary system have been proposed, which include:

Increased intraductal pressure due to either mechanical blockage by stones or reflux spasm of the sphincter of Oddi.

Erosion of the duct wall due to pressure by an impacted stone.

Intramural infection which weakens the duct wall.

Thrombosis of a mural vessel leading to necrosis of the affected part of the bile duct wall.

Diverticulum of the CBD.[8] In our case, the possible cause of perforation could be intramural infection leading to weakening of the wall.

Various approaches for the management of bile duct perforation have been described. They can be used according to the general condition of the patient, extent of the peritonitis, and the imaging findings. Patients presenting with generalized peritonitis require surgical exploration, thorough lavage and drainage of the peritoneal cavity, suture closure of the perforation if possible and treatment of associated biliary pathology. Most of these patients have associated choledocholithiasis which may require either choledocholithotomy with T-tube drainage. Choledochoduodenostomy and Roux-en-Y choledochojejunostomy have been described in the literature but preferably be avoided because general condition of the patient is usually low and also because of inflammation which can result in an anastomotic leak. Endoscopic retrograde cholangiopancreatography with sphincterotomy and stenting has also proven useful in the absence of generalized peritonitis. If the site of perforation can be localized, T-tube decompression is an effective procedure that is safe and reliable.

CONCLUSION

Spontaneous perforation of the bile duct is a rare and potentially fatal disorder. The condition presents a diagnostic dilemma. It is emphasized that spontaneous perforation of the bile duct should always be considered when there is peritonitis without pneumoperitoneum. The symptoms warrant abdominal paracentesis to assist in the diagnosis. An early and effective surgical management is associated with good prognosis; however, a delay in the correct diagnosis may result in bacterial contamination of the bile with an unfavorable outcome. Simple procedure like T-tube decompression should be preferred over more complex procedures.