Review
Copyright ©2007 Baishideng Publishing Group Co., Limited. All rights reserved.
World J Gastroenterol. Oct 14, 2007; 13(38): 5052-5059
Published online Oct 14, 2007. doi: 10.3748/wjg.v13.i38.5052
Surgical management of polycystic liver disease
Robert T Russell, C Wright Pinson
Robert T Russell, C Wright Pinson, Vanderbilt University Medical Center, Department of Hepatobiliary Surgery and Liver Transplantation, 1301 22nd Avenue South, Nashville, TN 37232-5545, United States
Author contributions: All authors contributed equally to the work.
Supported by an educational grant from Novartis Pharma-ceuticals
Correspondence to: C Wright Pinson, MD, MBA, Vanderbilt University Medical Center, Department of Hepatobiliary Surgery and Liver Transplantation, 1301 22nd Avenue South, Nashville, TN 37232-5545, United States. wright.pinson@vanderbilt.edu
Telephone: +1-615-3439324 Fax: +1-615-3436478
Received: July 13, 2007
Revised: August 2, 2007
Accepted: August 26, 2007
Published online: October 14, 2007
Abstract

Adult polycystic liver disease (PCLD) is an autosomal dominant condition commonly associated with autosomal dominant polycystic kidney disease (ADPKD). However in the last decade, it has been recognized that there is a distinct form of autosomal dominant PCLD that arises without concomitant ADPKD. Early knowledge of the pathogenesis was gained from the study of hepatic cysts in patients with ADPKD. Bile duct overgrowth after embryogenesis results in cystic hepatic dilatations that are known as biliary microhamartomas or von Meyenburg complexes. Further dilatation arises from cellular proliferation and fluid secretion into these cysts. There is a variable, broad spectrum of manifestations of PCLD. Although PCLD is most often asymptomatic, massive hepatomegaly can lead to disabling symptoms of abdominal pain, early satiety, persistent nausea, dyspnea, ascites, biliary obstruction, and lower body edema. Complications of PCLD include cyst rupture and cyst infection. Also, there are associated medical problems, especially intracranial aneurysms and valvular heart disease, which clinicians need to be aware of and evaluate in patients with PCLD. In asymptomatic patients, no treatment is indicated for PCLD. In the symptomatic patient, surgical therapy is the mainstay of treatment tailored to the extent of disease for each patient. Management options include cyst aspiration and sclerosis, open or laparoscopic fenestration, liver resection with fenestration, and liver transplantation. The surgical literature discussing treatment of PCLD, including techniques, outcomes, and complication rates, are summarized in this review.

Keywords: Polycystic liver disease; Fenestration; Laparoscopy; Liver resection; Liver transplantation