EASL Guidelines on Cystic Liver Disease
In June 2022, the European Association for the Study of the Liver (EASL) published guidelines on management of cystic liver disease. [18] Recommendations included the following.
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 ultrasonography (US) as the first imaging modality for diagnosing simple hepatic cysts and polycystic liver disease (PCLD), with additional imaging for hepatic cysts with complex features such as atypical cyst wall or content, either solitary or in patients with PCLD. For diagnosis of biliary hamartomas, use magnetic resonance imaging (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 PCLD) 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 computed tomography (CT) or MRI, tenderness near the liver, and increased C-reactive protein (CRP).
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 PCLD.
Treatment for PCLD should be given to symptomatic patients only.
Before pregnancy, patients with PCLD should be counseled about the risk of giving PCLD 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.
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Histology demonstrating biliary epithelium lining simple cyst.
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Ultrasonographic appearance of large simple hepatic cyst.
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Computed tomography (CT) scan appearance of large hepatic cyst.
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Computed tomography (CT) scan of polycystic liver disease curiously limited to right liver.
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Hepatic cysts. Sagittal magnetic resonance imaging (MRI) reconstruction in patient with large echinococcal cyst; note daughter cysts in interior.
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Computed tomography (CT) appearance of biliary cystadenoma.
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Resection of involved liver in polycystic liver disease.
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Laparoscopic view of initial hepatic cyst puncture, before unroofing. Lesion is located high in right liver near the diaphragm.
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Laparoscopic view of beginning of unroofing of large simple hepatic cyst near dome of right liver.
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Drawing of final result of laparoscopic unroofing of a large simple hepatic cyst in right liver.
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Initial penetration of hepatic cyst with drainage of cyst fluid.
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Unroofing of hepatic cyst.
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Omentum sutured to excised margin.
Tables
First Author (Year) |
Institution |
Number of Patients |
Success Rate, Simple Cysts |
Success Rate, Polycystic Liver Disease |
Morino (1994) [7] |
University of Torino, Italy |
17 |
100% |
40% |
Krahenbuhl (1996) |
University of Bern, Switzerland |
8 |
100% |
N/A |
Hansen (1997) [8] |
University of California, San Francisco, United States |
19 |
94% |
0% |
Emmerman (1997) |
Eppendorf University, Hamburg, Germany |
18 |
89% |
N/A |
Fabiani (1997) [9] |
University of Nice, France |
10 |
100% |
N/A |
Martin (1998) [10] |
Royal Infirmary, Edinburgh, Scotland |
38 |
92% |
39% |
Katkhouda (1999) [11] |
University of Southern California, United States |
25 |
100% |
89% |
Zacherl (2000) [12] |
University Clinic of Surgery, Vienna, Austria |
11 |
86% |
N/A |
Fiamingo (2003) [13] |
University Hospital, Via Giustiniani, Italy |
15 |
89% |
50% |
Robinson (2004) [14] |
University of Colorado, United States |
11 |
N/A |
45% |
Konstadoulakis (2005) [15] |
Athens University, Greece |
9 |
N/A |
78% |
Fabiani (2005) [16] |
University Nice, France |
38 |
96%* |
N/A |
*Clinicoradiologic recurrence |
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