Secondary Sclerosing Cholangitis in the Setting of Elective Bariatric Surgery

Abstract

Secondary sclerosing cholangitis (SSC) is a rare, chronic, progressive condition of biliary tree fibrosis after an insult. It is a rare cause of preventable liver cirrhosis. Of 31 reported cases in the past decades most presented after surgical trauma, other causes include chronic cholestatis, cholangitis or ischemia.1 A 45-year-old female with a history of idiopathic pulmonary fibrosis presented with prolonged hyperbilirubinemia after gastric sleeve bypass, cholecystectomy, and hiatal hernia repair three months prior to admission. She presented with jaundice and elevated total bilirubin, direct bilirubin and alkaline phosphatase at 3.6 mg/dL, 3.0 mg/dL, and 217 U/L, respectively. MRCP revealed a stricture near the level of the cystic duct. ERCP showed a focal stricture at the distal common hepatic duct (CHD) requiring stent (Image 1). Due to worsened hyperbilirubinemia, a repeat ERCP was completed which revealed a highgrade stricture at the CHD that required dilation and another stent. Liver biopsy was also performed and showed canalicular cholestatis without intra-hepatic granulomas or ductal lesions, suggesting extrahepatic ductal obstruction (Image 2). She then underwent hepaticojejunostomy with surgical pathology of the common bile duct revealing a post-cholecystectomy traumatic neuroma. She was discharged after HIDA scan revealed no evidence of biliary leak, but returned to clinic 14 days later with progressive jaundice, new ascites, bilateral lower extremity edema, and acute kidney injury (AKI) with elevated creatinine of 1.76 mg/dL (baseline 0.4 mg/dL). A third liver biopsy was performed which showed findings consistent with portal edema, mixed inflammation, cholestasis and fibrosis (Image 3). Her bilirubin slowly improved without further intervention, but remained elevated at 14.2 mg/dL and she ultimately required dialysis. This patient was diagnosed with SSC in the setting of biliary tract fibrosis subsequent to traumatic injury of the biliary tract after cholecystectomy. Our presentation is unique as SSC was complicated by hemodialysis dependent renal dysfunction with microscopic evidence of acute tubular necrosis. This case is consistent with the common cause, histological characteristics and chronic nature of SSC. Future research is required to elucidate other organ complications that management of this disease may be optimized. 1- Ruemmele et. al. Secondary sclerosing cholangitis. Nat. Rev. Gastroenterol. Hepatol. 6, 287-295 (2009).Figure: ERCP showing common hepatic duct stricture.Figure: Cholestasis and edema.Figure: Portal tract with edema and fibrosis.