Surgical management of polycystic liver disease
Robert T Russell1, C Wright Pinson
1Vanderbilt University Medical Center, Department of Hepatobiliary Surgery and Liver Transplantation, 1301 22nd Avenue South, Nashville, TN 37232-5545, United States.
Insights
Polycystic liver disease (PCLD) is an inherited condition causing liver cysts. Treatment for symptomatic PCLD focuses on surgical interventions tailored to individual patient needs.
Area of Science:
- Hepatology
- Genetics
- Surgical Gastroenterology
Background:
- Adult polycystic liver disease (PCLD) is an autosomal dominant condition.
- PCLD can occur independently of autosomal dominant polycystic kidney disease (ADPKD).
- Hepatic cysts in PCLD originate from biliary microhamartomas or von Meyenburg complexes.
Purpose of the Study:
- To review the pathogenesis, clinical manifestations, and management of PCLD.
- To summarize surgical treatment options, outcomes, and complication rates for PCLD.
Main Methods:
- Review of the surgical literature on PCLD treatment.
- Analysis of techniques, outcomes, and complication rates.
Main Results:
- PCLD presents with a wide spectrum of symptoms, from asymptomatic to severe hepatomegaly.
- Complications include cyst rupture, infection, intracranial aneurysms, and valvular heart disease.
- Surgical therapy is the mainstay for symptomatic PCLD, with options including aspiration, fenestration, resection, and transplantation.
Conclusions:
- Early recognition of PCLD and associated conditions is crucial.
- Treatment for symptomatic PCLD should be individualized based on disease extent.
- Surgical interventions offer various management strategies for PCLD patients.
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.
