This uncommissioned systematic review was subject to full peer-review.
Systematic review: the pathophysiology and management of polycystic liver disease
Article first published online: 26 JUL 2011
© 2011 Blackwell Publishing Ltd
Alimentary Pharmacology & Therapeutics
Volume 34, Issue 7, pages 702–713, October 2011
How to Cite
Temmerman, F., Missiaen, L., Bammens, B., Laleman, W., Cassiman, D., Verslype, C., van Pelt, J. and Nevens, F. (2011), Systematic review: the pathophysiology and management of polycystic liver disease. Alimentary Pharmacology & Therapeutics, 34: 702–713. doi: 10.1111/j.1365-2036.2011.04783.x
- Issue published online: 6 SEP 2011
- Article first published online: 26 JUL 2011
- Publication data Submitted 3 May 2011 First decision 29 May 2011 Resubmitted 21 June 2011 Accepted 1 July 2011 EV Pub Online 26 July 2011
Aliment Pharmacol Ther 2011; 34: 702–713
Background Polycystic liver diseases (PCLD) represent a group of genetic disorders in which cysts occur solely in the liver, or together with renal cysts. Most of the patients with PCLD are asymptomatic, however, in some patients, expansion of liver cysts causes invalidating abdominal symptoms.
Aim To provide a systemic review on the pathophysiology and management of PCLD.
Methods A PubMed search was undertaken to identify relevant literature using search terms including polycystic liver disease, pathophysiology, surgical and medical management.
Results The most common complication in patients with PCLD is extensive hepatomegaly, which may lead to malnutrition and can be lethal. Conservative surgical approaches are only partially effective and do not change the natural course of the disease. Liver transplantation has been successfully performed in PCLD, however, in an era of organ shortage, medical management needs to be evaluated. A better understanding of the pathophysiology and the availability of animal models have already identified promising drugs. Abnormalities in cholangiocyte proliferation/apoptosis and enhanced fluid secretion are key factors in the pathophysiology. It has been demonstrated in rodents and in humans that somatostatin analogues diminish liver volume. The role of the inhibitors of the mammalian target of rapamycin (mTOR) in the management of PCLD is still under investigation.
Conclusions The exact pathophysiology of polycystic liver disease still remains unclear. In symptomatic patients, none of the currently available surgical options except liver transplantation have been shown to change the natural course of the disease. The use of somatostatin analogues has been shown to diminish liver volume.