A Rare Cause of Mirizziʼs Syndrome: Cholangiocarcinoma

Abstract

Introduction: Elevated liver function tests are the most common reason for consultation to a hepatology service, the etiologies of which are varied but most commonly are due to viral hepatitis, toxic, and ischemic injuries. Obstruction due to biliary stones are also a common etiology as well, but we present a more rare case of biliary obstruction that resulted in elevated liver function tests and has a higher incidence of carcinoma. Case Report: A 48-year-old female with no past medical history presents with 1-month history of gradual yellowing of her eyes and skin. Also noted decreased appetite, increasing pruritis, and some subjective weight loss. She denied any abdominal pain, nausea, vomiting, signs of gastrointestinal bleeding, fevers, or chills. She has never experienced this prior. On exam, her vital signs were normal but she had overt jaundice as well as scleral icterus. She had mild tenderness to palpation in the right upper quadrant but negative Murphy's sign. Admission labs revealed a total bilirubin of 14.1 with a direct bilirubin of 10.3. Her AST and ALT were elevated to 112 and 78, respectively, as well as an alkaline phosphatase elevation to 308. Her amylase and lipase were mildly elevated to 146 and 168, respectively. Workup included negative hepatitis serologies and autoimmune panel among other tests. An MRCP was performed which showed a large obstructing cystic duct stone compressing against the common bile duct (CBD) with subsequent biliary tree dilation. An ERCP was performed which confirmed these findings and a CBD stent was placed with effective biliary drainage. She subsequently went for a cholecystectomy. However, 1 month later she presented again with weight loss and jaundice, and was subsequently diagnosed with cholangiocarcinoma after ERCP and further surgical evaluation. Discussion: Mirizzi's syndrome is a rare disorder for which the indication for cholecystectomy is <1%. It is characterized by two different classifications (Csendes or McSherry) based on the presence of and the extent of a cholecystocholedochal fistula. The diagnosis can be made preoperatively with MRCP or ERCP, but often they are diagnosed intraoperatively. The ultimate treatment is surgical cholecystectomy but an ERCP with stent placement can be a temporizing measure. One lesser known association of Mirizzi's syndrome is that there is a higher incidence of gallbladder carcinoma; in one study up to 27%. Thus it is imperative to send the specimen for pathology to evaluate for malignancy and to also expedite any cases of Mirizzi's syndrome for surgical resection. To date, we report the only case of Mirizzi's syndrome associated with cholangiocarcinoma.