A 40 year old southeast Asian presents to your clinic with yet another episode of right upper quadrant abdominal pain, fever, and chills. Reviewing the chart, you note that he's had these same symptoms many times in the past along with a few episodes of pancreatitis, two liver abscesses that required drainage, and cirrhosis. In social history, you find out that he is poor and lives in a rural area. He does not drink. Physical exam shows jaundice, right upper quadrant tenderness, and hepatomegaly.
A RUQ ultrasound shows ductal dilatation, stones, and hepatic abscesses. CT and cholangiogram are shown below.
You treat the patient with fluids, antibiotics, and biliary drainage, but so many stones keep coming out; they're mostly pigmented. Nevertheless, there is no evidence of hemolysis. Endoscopy is limited because of the strictures. Finally, you get surgery to do a cholecystectomy with common bile duct exploration and T-tube drainage.Unfortunately, the most common causes of death in this disease are sepsis, liver failure, and complications from cirrhosis. There is an increased risk of cholangiocarcinoma.
Challenge: What is this bizarrely named disease?
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2 comments:
I Had Never Heard of This Disease
This is oriental cholangiohepatitis, characterized by intrahepatic pigment stone formation resulting in biliary obstruction with recurrent cholangitis, dilatation, and stricturing of the biliary tree. The CT and ERCP cholangiogram shows massively dilated left hepatic system and numerous stones (arrow in CT, round lucencies in ERCP).
Source: UpToDate, original images courtesy of Hannah Lee.
it is not uncommon to see such cases in our setting. this condition is difficult to treat bt easy to diagnose. most of them progress to cirrhosis and require trnasplantation
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