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  • Review Article
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Diagnosis and management of polycystic liver disease

Nature Reviews Gastroenterology & Hepatology volume 10, pages 101–108 (2013)Cite this article

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Abstract

Polycystic liver disease (PLD) is arbitrarily defined as a liver that contains >20 cysts. The condition is associated with two genetically distinct diseases: as a primary phenotype in isolated polycystic liver disease (PCLD) and as an extrarenal manifestation in autosomal dominant polycystic kidney disease (ADPKD). Processes involved in hepatic cystogenesis include ductal plate malformation with concomitant abnormal fluid secretion, altered cell–matrix interaction and cholangiocyte hyperproliferation. PLD is usually a benign disease, but can cause debilitating abdominal symptoms in some patients. The main risk factors for growth of liver cysts are female sex, exogenous oestrogen use and multiple pregnancies. Ultrasonography is very useful for achieving a correct diagnosis of a polycystic liver and to differentiate between ADPKD and PCLD. Current radiological and surgical therapies for symptomatic patients include aspiration–sclerotherapy, fenestration, segmental hepatic resection and liver transplantation. Medical therapies that interact with regulatory mechanisms controlling expansion and growth of liver cysts are under investigation. Somatostatin analogues are promising; several clinical trials have shown that these drugs can reduce the volume of polycystic livers. The purpose of this Review is to provide an update on the diagnosis and management of PLD with a focus on literature published in the past 4 years.

Key Points

  • Polycystic liver disease (PLD) is the primary presentation in isolated polycystic liver disease (PCLD) and can present as an extrarenal manifestation in autosomal dominant polycystic kidney disease

  • Female sex, exogenous oestrogens and multiple pregnancies are risk factors for the development of polycystic livers

  • Ultrasonography is the first step in diagnosing PLD

  • Screening for intracranial aneurysms is not recommended for patients with PCLD

  • Surgical treatment is indicated in symptomatic patients; the choice of treatment depends on total liver volume and the size and location of the liver cysts

  • Somatostatin analogues decrease the volume of polycystic livers, are well-tolerated and improve patients' perception of their health

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Figure 1: Embryonic development of the ductal plate.
Figure 2: Gigot type I–III livers.
Figure 3: Percentage changes in liver volume during somatostatin analogue therapy or after withdrawal, presented as medians (IQR) or means (SD).
Figure 4: Flow diagram for treatment strategy in patients with PLD.

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Acknowledgements

The authors would like to acknowledge the support of the Institute of Genetic and Metabolic Diseases of the Radboud University Nijmegen Medical Centre, The Netherlands. The authors thank the following persons from the Department of Gastroenterology and Hepatology, Radboud University Nijmegen Medical Centre, The Netherlands: Frank Weimer for designing the structure of this paper and drafting the first version, Melissa Chrispijn for expert advice and Marten Lantinga for proofreading the article before submission.

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  1. Department of Gastroenterology and Hepatology, Radboud University Nijmegen Medical Centre, Nijmegen, P. O. Box 9101, Code 455, Nijmegen, 6500 HB, The Netherlands

    Tom J. G. Gevers & Joost P. H. Drenth

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T. J. G. Gevers researched data for the article. Both authors contributed to discussion of the content and wrote the article. J. P. H. Drenth reviewed/edited the manuscript before submission.

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J. P. H. Drenth has received grant support from Ipsen and Novartis.

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Gevers, T., Drenth, J. Diagnosis and management of polycystic liver disease. Nat Rev Gastroenterol Hepatol 10, 101–108 (2013). https://doi.org/10.1038/nrgastro.2012.254

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