Review
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World J Gastroenterol. Sep 21, 2013; 19(35): 5775-5786
Published online Sep 21, 2013. doi: 10.3748/wjg.v19.i35.5775
Pathophysiology, epidemiology, classification and treatment options for polycystic liver diseases
Bassam Abu-Wasel, Caolan Walsh, Valerie Keough, Michele Molinari
Bassam Abu-Wasel, Caolan Walsh, Michele Molinari, Department of Surgery, Dalhousie University, Halifax, Nova Scotia B3H 2Y9, Canada
Valerie Keough, Department of Radiolgoy, Dalhousie University, Halifax, Nova Scotia B3H 2Y9, Canada
Michele Molinari, Department of Surgery and Community Health and Epidemiology, Dalhousie University, Halifax, Nova Scotia B3H 2Y9, Canada
Author contributions: Abu-Wasel B performed the review of the literature, wrote the paper and created the diagrams used in this review; Walsh C performed the review of the literature and edited the final draft; Keough V performed the selection of cross sectional studies, reviewed the literature on radiological classification of polycystic liver disease and edited the final draft; Molinari M ideated the paper, performed the review of the literature, edited the paper and selected some of the relevant radiological images.
Correspondence to: Michele Molinari, MD, MSc, Associate Professor, Department of Surgery and Community Health and Epidemiology, Dalhousie University, Room 6-302 Victoria Building, 1276 South Park Street, Halifax, Nova Scotia B3H 2Y9, Canada. michele.molinari@cdha.nshealth.ca
Telephone: +1-902-4737624 Fax: +1-902-4737639
Received: March 5, 2013
Revised: May 21, 2013
Accepted: July 9, 2013
Published online: September 21, 2013
Abstract

Polycystic liver diseases (PLD) represent a group of genetic disorders in which cysts occur in the liver (autosomal dominant polycystic liver disease) or in combination with cysts in the kidneys (autosomal dominant polycystic kidney disease). Regardless of the genetic mutations, the natural history of these disorders is alike. The natural history of PLD is characterized by a continuous increase in the volume and the number of cysts. Both genders are affected; however, women have a higher prevalence. Most patients with PLD are asymptomatic and can be managed conservatively. Severe symptoms can affect 20% of patients who develop massive hepatomegaly with compression of the surrounding organs. Rrarely, patients with PLD suffer from acute complications caused by the torsion of hepatic cysts, intraluminal cystic hemorrhage and infections. The most common methods for the diagnosis of PLD are cross sectional imaging studies. Abdominal ultrasound and computerized tomography are the two most frequently used investigations. Magnetic resonance imaging is more sensitive and specific, and it is a valuable test for patients with intravenous contrast allergies or renal dysfunction. Different treatment modalities are available to physicians caring for these patients. Medical treatment has been ineffective. Percutaneous sclerotherapy, trans-arterial embolization, cyst fenestration, hepatic resection and liver transplantation are indicated to specific groups of patients and have to be tailored according to the extent of disease. This review outlines the current knowledge of the pathophysiology, clinical course, diagnosis and treatment strategies of PLD.

Keywords: Polycystic liver disease, Hepatic, Epidemiolgy, Classification, Therapy, Genetic

Core tip: The management of patients with symptomatic polycystic liver disease is challenging. Among several treatments options, the most common interventions are: percutaneous cyst aspiration, fenestration, hepatic resection and liver transplantation. There is no consensus on the best treatment options and the optimal timing for interventions in symptomatic patients. In vision of these limitations, we reviewed the most recent literature and present a comprehensive article on this topic.