A334 CHRONIC MIRIZZI SYNDROME CAUSING SECONDARY SCLEROSING CHOLANGITIS AND CIRRHOSIS: A CASE REPORT.

Mirizzi syndrome (MS) is an uncommon presentation in which a gallstone becomes impacted in the cystic duct or neck of the gallbladder causing compression of the common bile duct (CBD) or common hepatic duct (CHD), with resultant biliary obstruction. Most common symptoms include abdominal pain, jaundice and fever. Patients can present with concurrent cholangitis and acute cholecystitis. Diagnosis can be confirmed by imaging modalities including ultrasound, MRCP, EUS or ERCP. Initial management of MS includes supportive care, and relief of biliary obstruction via ERCP. Surgery is the definitive treatment for MS. Secondary Sclerosing Cholangitis (SSC) refers to a chronic and progressive liver disease characterized by inflammation, fibrosis and obstruction of the intra and extrahepatic biliary tree. The etiology is varied but includes chronic biliary obstruction. MS has not been described as a potential etiology for SCC as it is typically an acute presentation requiring emergent treatment. To date there have been no documented reports of chronic MS or MS causing SSC. We present a case of chronic MS leading to SSC and cirrhosis. A literature search was performed between July 1997 and July 2017 in the PubMed and Google Scholar databases using the terms “Mirizzi syndrome”, “chronic Mirizzi syndrome”, “secondary sclerosing cholangitis”, either individually or in combination. Citations among the identified publications were also reviewed. A previously healthy 44-year-old female presented to the emergency department with a 15-month history of intermittent right upper quadrant pain and fevers. Her labs at the time of presentation revealed an elevated WBC of 13.8, ALT 234, AST 234, Total Bilirubin 535, ALP 1389, GGT 290 and albumin 30. MRCP imaging showed intrahepatic and CBD dilation concerning for biliary obstruction. She was referred to Gastroenterology for an ERCP which demonstrated markedly dilated intrahepatic biliary system with a 2-cm stone in the cystic duct causing external compression of the CBD consistent with MS (image1). She was then referred to hepatobiliary surgery where the diagnosis of MS was further confirmed surgically. Intraoperatively, note was made of severe intrahepatic and peri-portal inflammation with fibrosis, suspicious for SSC and portal hypertension. An intraoperative liver biopsy was consistent with the diagnosis of secondary sclerosing cholangitis. Screening workup for other causes of liver disease was negative and liver enzymes were normal prior to her presentation. MS is an uncommon and invariably acute complication of gallstone disease. This is the first reported case of MS as a chronic presentation since patients typically require emergent treatment. Consequently, this is also the first case of chronic MS as a cause of SSC and portal hypertension in the available literature. Image 1 None

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