Cystic Liver Disease Clinical Practice Guidelines (EASL, 2022)

European Association for the Study of the Liver

These are some of the highlights of the guidelines without analysis or commentary. For more information, go directly to the guidelines by clicking the link in the reference.

August 10, 2022

Guidelines on cystic liver disease were published in June 2022 by the European Association for the Study of the Liver in The Journal of Hepatology.[1]

Hepatic Cysts

The number of lesions (solitary vs multiple) and architecture (simple vs complex cyst) are essential in the characterization of hepatic cyst(s).

Use ultrasound as the first imaging modality for diagnosing simple hepatic cysts and polycystic liver disease (PLD), with additional imaging for hepatic cysts with complex features such as atypical cyst wall or content, either alone or in patients with PLD. For diagnosis of biliary hamartomas, use MRI with heavily T2-weighted sequences and MR cholangiography sequences.

Do not use the tumor markers carcinoembryonic antigen (CEA) or carbohydrate antigen 19-9 (CA19-9) in blood or cyst fluid to distinguish between hepatic cysts (solitary or in PLD) and mucinous cystic neoplasms (MCNs) of the liver.

Patients with symptomatic simple hepatic cysts without biliary communication should receive the best volume-reducing therapy available locally.

Hepatic cyst infection should be viewed as definite when accompanied by neutrophil debris and/or microorganisms in cyst aspirate with evidence of infection, and it should be viewed as likely when accompanied by features including fever for more than 3 days with no other detectable source, gas in a cyst found on CT or MRI, tenderness near the liver, and increased C-reactive protein.

Fluoroquinolones and third generation cephalosporins should be used for empiric first-line antibiotic therapy for hepatic cyst infection; antibiotics should be given for 4-6 weeks.

CT should not be used for diagnosis of cyst hemorrhage.

Temporarily stop anticoagulants in patients with hepatic cyst hemorrhage.

Polycystic Liver Disease

Stop exogenous estrogen in female patients with PLD.

Treatment for PLD should be given to symptomatic patients only.

Before pregnancy, patients with PLD should be counseled about the risk of giving PLD to the newborn but should not be advised against pregnancy.

Caroli Disease

Patients with multiple segmental cystic or saccular dilatations of bile ducts should be evaluated for congenital hepatic fibrosis to distinguish Caroli disease from Caroli syndrome.

Consider referral for liver transplantation in patients with Caroli disease or Caroli syndrome who have recurrent cholangitis and either bilobar involvement or monolobar involvement with liver fibrosis or portal hypertension when liver resection is not possible.

For more information, please go to Hepatic Cysts.


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