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Cholangitis

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Fibrocystic Diseases of the Liver

Part of the book series: Clinical Gastroenterology ((CG))

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Summary

Acute cholangitis is a common complication in certain of the fibrocystic disorders especially Caroli’s disease and choledochal cysts, with recurrent episodes potentially leading to progressive liver failure. It is a clinical syndrome characterized by fever, jaundice, and abdominal pain (Charcot’s triad) that develops as a result of stasis and an infection of the biliary tree. The additional symptoms of hypotension and a change in mental status (Reynolds’ pentad) can occur in some patients with suppurative cholangitis and is still associated with a high morbidity and mortality rate. The differential diagnosis includes hepatic abscess, cholecystitis, hepatitis, right lower lobe pneumonia, and other less common disorders. Hepatic abscess and renal failure are complications associated with cholangitis that convey a poor prognosis. Diagnosis is made on clinical grounds, routine blood work, and imaging studies of the biliary tree. Magnetic resonance cholangiopancreatography (MRCP) is especially useful in the fibrocystic diseases, in that it provides a useful anatomic map prior to undertaking any definitive therapy. Blood cultures and bile cultures should always be obtained.

Antibiotics, and if possible, appropriate biliary drainage are the two arms used to treat this condition. Eighty percent of patients will respond to initial empiric antibiotics and conservative management with only a few requiring emergent biliary decompression. If, however, after 6–12 h of conservative management, there is decline in clinical status with increased pain, fever, hypotension, or confusion, emergent drainage should be performed. Quinolones, beta-lactam/beta-lactamase inhibitors, third-generation cephalosporins, or carbepenams are acceptable initial choices for antibiotics, with changes made according to the biliary and/or blood culture data.

Biliary drainage can be achieved in order of preference with endoscopic, percutaneous, or very rarely surgical intervention. Endoscopic stent placement is probably as effective as nasobiliary drain placement without the associated discomfort of the nasobiliary drain or its accidental dislodgement. Cholangioscopy can have a role in identifying and treating some of the causes (biliary stones) and other complications of fibrocystic diseases, including indeterminate strictures. Occasionally, recurrent bouts of cholangitis can lead to progressive liver failure necessitating liver transplantation as the only remaining management option.

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Authors and Affiliations

  1. The Digestive Disease Institute at Virginia Mason Medical Center, Seattle, WA, USA

    Shayan Irani MD

  2. Division of Gastroenterology, Department of Medicine, The Digestive Disease Institute at Virginia Mason Medical Center, Seattle, WA, USA

    Richard Kozarek MD

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  1. Shayan Irani MD
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Editors and Affiliations

  1. School of Medicine, Children's Hospital & Regional, University of Washington, Sand Point Way NE. 4800, Seattle, 98105, USA

    Karen F. Murray

  2. St. Paul University Hospital, Harry Hines Blvd. 5939, Dallas, 75390-9258, USA

    Anne M. Larson

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Irani, S., Kozarek, R. (2010). Cholangitis. In: Murray, K., Larson, A. (eds) Fibrocystic Diseases of the Liver. Clinical Gastroenterology. Humana Press. https://doi.org/10.1007/978-1-60327-524-8_21

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  • DOI: https://doi.org/10.1007/978-1-60327-524-8_21

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