Spontaneous common bile duct perforation presenting as extrahepatic bilioma: a rare clinical presentation of gall stone disease

Spontaneous common bile duct perforation presenting as extrahepatic bilioma: a rare clinical presentation of gall stone disease

Princy Gupta
Maulana Azad Medical College, New Delhi, India

A 45 years old female presented to surgery emergency with 5 days history of pain right upper abdomen with high grade fever, chills and rigors associated with yellowish discoloration of eyes and skin with generalized itching all over body. On examination, patient was febrile (101°F), tachypneic with tachycardia of 108/min, dehydrated and icteric. Abdomen was soft with tenderness present at right hypochondrium and right lumbar region. Murphy’s sign negative. Laboratory investigation revealed TLC of 9700 cells/mm3 with total bilirubin of 5.68, direct bilirubin of 3.49, and normal liver enzymes with elevated alkaline phosphatase (219). Ultrasound revealed dilated CBD, 12 mm at porta, distal CBD obscured with bilobar IHBRD and a contracted gall bladder. A diagnosis of acute cholangitis was made and patient was managed conservatively and was improving clinically as well as biochemically. On 3rd day of admission, patient developed abdominal distention, examination revealed a swelling of around 15×15 cm involving whole of the right abdomen, which was non tender. Her laboratory studies also demonstrated a marked increase in alkaline phosphatase level to 3529 from 219. Ultrasound revealed reduced distention of CBD to 7 mm with large thick walled collection with multiple internal echoes and dependent echogenic debri within in right lumbar region. Magnetic resonance cholangiopancreaticography revealed cholelithiasis with choledocholithiasis with resultant upstream bilobar IHBRD and a large thick walled hyperintense collection measuring 7.8x19x10 cm in subhepatic region, reaching upto right iliac fossa. The collection is reaching upto proximal CBD proximal to calculus, likely extra hepatic bilioma. Ultrasound guided pigtail catheter drainage was done which revealed stat output of 2000 mL of bilious fluid. Patient was taken up for exploratory laparotomy, which revealed a 2×2 cm perforation present over lateral edge of CBD with single large stone present just beneath the perforation site. Intrahepatic, normal, distended gall bladder with calculi, dense adhesions between liver, gall bladder, omentum and bowel and 10×10 cm well localized collection present in right lumbar region surrounded by omentum with pigtail in situ. Peritoneal lavage was done using warm normal saline. Cholecystectomy was done and CBD explored. CBD calculi removed and CBD closed over T tube, inserted through the perforation site. Patient improved symptomatically and biochemically. T Tube cholangiogram done on POD5 revealed a filling defect. T tube was clamped and repeat T tube cholangiogram revealed no abnormality. T tube was removed on POD15, and patient discharged on POD17. She was doing well on her first and subsequent visits for next 3 months.

Non traumatic perforation of extrahepatic bile ducts is a clinically rare entity seen more commonly in infants than adults. In infants, it is secondary to congenital anomalies like choledochal cyst, anomalous union of pancreatico-biliary ductal system, bile duct stenosis or atresia that weakens the common bile duct wall. Although the pathogenesis of spontaneous biliary perforation is poorly understood, the commonest cause for perforation was a stone in adults with the mechanism being impaction of stone followed by erosion. Other causes reported are tumor obstructing the lumen and increasing canalicular pressure, spasm of sphincter, intramural infection, mural necrosis secondary to mural vessel infarction, connective tissue defect, ischemic compromise or previous biliary tract surgeries. Kang et al. reviewed 70 cases of spontaneous bile duct perforations in adults. Among these, 42 patients had perforation in common bile duct, followed by hepatic duct in 28 cases. In our case, we attribute the perforation to the impacted stone within the CBD leading to ischemic compromise and eventually erosion of the duct. The presentation of CBD perforation may be acute or insidious, with the latter type being more common and characterized by progressive jaundice, painless abdominal distension and clay colored stools. The acute form is less common and presents with fever, vomiting and signs of fulminant peritonitis. Ultrasound findings in a case of extrahepatic biliary duct perforation reveals free fluid in peritoneal cavity with normal extra and intrahepatic biliary radicles. Failure to see distended gall bladder points towards the decompression of biliary system secondary to perforation. MRCP reveals the same finding with a collection communicating with the extra hepatic biliary ductal system, as in our case. In doubtful cases, radio isotope scanning can be done. Recommended standard of care in a case of spontaneous perforation of CBD is exploration of CBD with retrieval of stone and closure of CBD over T tube placed from the perforation site. If distal obstruction is anticipated, biliary enteric bypass can be done. Primary repair of CBD over CBD stent is hazardous in acute presentation due to local inflammation, especially if distal patency is not known.

Keywords: Non-traumatic perforation, extrahepatic bile ducts