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Liver Diseases pp 211-217 | Cite as

Polycystic Liver Diseases

  • Carmen Fierbinteanu-Braticevici
  • Alexandru Constantin Moldoveanu
Chapter

Abstract

Polycystic liver disease is a rare disease, manifesting with progressive bile duct enlargement and development of multiple cysts of various sizes in the liver. While it can be regarded as a hereditary disease, with either autosomal dominant transmission or autosomal recessive transmission, only about 20% of patients have been identified to have genetic mutations. Prevalence of isolated polycystic liver disease is less than 0.01%, much lower compared to polycystic disease of both the liver and kidney where it is closer to 0.2% of the population. Most patients with uncomplicated cysts are asymptomatic. In symptomatic patients, the symptoms usually result from cyst compression on adjacent structures. Isolated polycystic liver disease can be evaluated and diagnosed using the Reynolds criteria, while liver and kidney disease can be diagnosed using Unified Ravine criteria and Pei criteria. The differential diagnosis includes other pathologies that generate cysts or cyst-like structures and includes simple cysts, echinococcosis, cystadenoma, cystadenocarcinoma. The natural history of the disease describes a continuous growth of the liver size of 0.9–3.2% on average per year. Complications include cyst infection, rupture and hemorrhaging. Commonly used classifications have a role in determining therapy and include the Schnelldorfer’s Classification and Gigot’s Classification. Treatment options include cyst aspiration, cyst fenestration, cyst enucleation, hepatic resection and hepatic transplant. Non-surgical options include somatostatin analogues, everolimus and transarterial embolization.

Keywords

Polycystic liver Polycystic kidney Rare disease 

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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Carmen Fierbinteanu-Braticevici
    • 1
    • 2
  • Alexandru Constantin Moldoveanu
    • 1
    • 2
  1. 1.Department of Internal Medicine II and Gastroenterology - University Hospital Bucharest“Carol Davila” University of Medicine and Pharmacy BucharestBucharestRomania
  2. 2.Department of GastroenterologyUniversity Hospital BucharestBucharestRomania

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