Biliary Cast - Complication of Cholangitis and Pancreatitis in a Pancreas Divisum Patient

We report a rare cause of "biliary cast" secondary to cholangitis and pancreatitis, in a 60-year-old female patient with pancreas divisum. She was admitted in our hospital with an acute pancreatitis (alcoholic etiology was excluded) complicated with pancreatic abscess and obstructive jaundice. The patient had undergone a complex surgical intervention: cholecystectomy, choledocotomy with extraction of the biliary thrombus, external biliary drainage through a T tube, evacuation of the pancreatic abscess, sequestrectomy, peritoneal lavage and multiple drainages. In spite of the surgical and intensive care support, the biliary drainage through the T tube had ceased and the obstructive jaundice had reappeared in a more accentuated fashion. Endoscopic retrograde cholangiography showed complete pancreas divisum and diffuse multiple stenosis alternating with dilatation of the intrahepatic biliary tree (a pattern of sclerosing cholangitis). An endoscopic prosthesis was placed inside the right hepatic bile duct. Despite the use of the combined endoscopic plus UDCA (ursodeoxycholic acid) treatment for the management of the biliary cast syndrome, the evolution was unfavorable with hepatic coma, septic shock and finally death. The necropsy revealed an extensive biliary cast in the entire biliary tree and pyogenic cholangitis. The patient had a fatal outcome despite all the surgical, endoscopic and conservative efforts, with development of intraductal biliary obstruction and secondary pyogenic cholangitis. Biliary cast syndrome is a rare but very aggressive entity and its management is often difficult despite the advances in surgery and endoscopy treatments.