Introduction: Oriental cholangiohepatitis is commonly seen in Asian with prevalence of < 1% in Western countries. It is thought to be secondary to parasitic infection of bile system with concomitant biliary infection that leads to production of beta-glucuronidase that play key roles in bilirubin precipitation, stone and stricture formation. Case: A 73 year-old caucasian female with history of diabetes mellitus presented with nausea, vomiting, epigastric abdominal pain, diarrhea for 1 week. Reported 70-lb weight loss over past year. CT scan was concerning for possible pancreatic mass, omental metastasis, numerous tumor nodules in mesentery and possible thrombus in portal vein. Labwork showed ALP 230, albumin 1.6, CA 19/9 45.2. US Liver showed moderate intrahepatic biliary dilation, dilated proximal common duct measuring 1.4cm. MRCP showed moderate intra and extrahepatic biliary ductal dilatation with multiple large filling defects within both intrahepatic and extrahepatic bile ducts, consistent with stones (Fig 1). EUS was done which showed normal visual exam, diffuse pancreas with mixed echogenicity. FNA of pancreatic head/body negative for malignancy. ERCP showed CBD of 1.8 cm with atleast 4 (˜1cm stones) with largest being ˜1.2cm (Fig 2). Biliary sphincterotomy was performed followed by mechanical lithotripsy, balloon extraction. A 10 Fr 9cm plastic biliary stent was placed in CBD proximal to retained stones. Repeat ERCP was done in OR with laser lithotripsy and residual stone removal in 2-3 weeks.Figure 1Figure 2Discussion: It is a serious condition owing to its intractability, frequent recurrence and predisposition of cholangiocarcinoma. Presenting symptoms include abdominal pain, jaundice, cholangitis and can lead to production of strictures and liver abscesses. US liver is first diagnostic modality as it can clearly show intrahepatic stones and biliary dilation. MRCP is the best non-invasive imaging that shows intrahepatic stones, biliary strictures and hepatic abscess. Direct cholangiography remains the gold standard with sensitivity of almost 100% in the detection of obstruction. The goals of treatment include treating cholangitis (fluids/antibiotics), preventing recurrence, clearance of stones, correction of strictures, restoration of biliary drainage and to stop the progression of the disease. There is no definitive treatment owing to the complicated nature of the disease. Urgent endoscopic biliary decompression prevents patient from going into shock. Hepatic resection has shown to reduce the risk of recurrence of stones, since it removes not only intrahepatic stones but also the associated pathological bile ducts damaged by strictures. Liver transplantation is usually the only treatment option in cases of diffuse liver parenchyma involvement and development of cirrhosis.Table 1: Grading the severity of hepatolithiasis
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