A man in his 40s w/ no PMHx presented with abdominal pain, fever, and mildly elevated bilirubin, ultimately found to have cholangitis secondary to severely dilated biliary ducts from Caroli Disease.
*Take home points: Two key management principles for cholangitis; recognize Caroli disease as a rare cause of severe non-obstructive biliary ductal dilation*
Management of cholangitis:
1) SOURCE CONTROL! Source control, source control. If imaging shows an impacted stone or abscess or cyst or anything that can be opened/drained, get GI, surgery, and/or IR involved immediately!
2) Antibiotics: a) BLOOD CULTURES are key! They will help you narrow and are often positive in cholangitis! As Ryan pointed out, make sure you’re asking for these upfront when the ED calls so they can be done before antibiotics. b) Abx choice: broadly cover GNRs and anaerobes. Good options include ampicillin-sulbactam (unasyn), piperacillin-tazobactam (zosyn), or ceftriaxone + metronidazole.
Caroli disease: a rare, congenital disorder with multifocal dilation of biliary ducts and no other liver abnormalities (its counterpart, caroli syndrome, is more common and accompanied by congenital hepatic fibrosis so seen more by our pediatric colleagues)
*Usually, biliary dilation results from obstruction (stones, acute or chronic infection)
*In Caroli disease, infection results from dilation
Kaiser SF Internal Medicine Residency Blog 