Polycystic liver disease(PLD)is a genetic disorder[1]that is di-agnosed by presence of multiple hepatic cysts.Clinical manifesta-tions of PLD are related to the size of hepatic cysts and associated complications.In th...Polycystic liver disease(PLD)is a genetic disorder[1]that is di-agnosed by presence of multiple hepatic cysts.Clinical manifesta-tions of PLD are related to the size of hepatic cysts and associated complications.In the early stage,PLD rarely causes any symptoms.However,patients with advanced stage PLD have a variety of clin-ical manifestations and complications due to severe hepatomegaly.They include abdominal pain,abdominal distension,dyspepsia,as-cites and many others that may result from compression of ad-jacent tissue or organ failure[2,3].Surgical treatment of PLD in-cludes:aspiration with or without sclerotherapy,cyst fenestration,hepatectomy and liver transplantation.Despite some researchers have concluded that cyst fenestration or hepatectomy is effective in treatment of PLD[4–6],the only curative surgical option in se-vere patients is liver transplantation[7,8].All other types of surg-eries present a great challenge to a surgeon,having little or no ef-fect when cysts are diffuse,or even contraindicated due to possi-bility of life-threatening complications arising during or after the procedure.Herein,we report a case of 51-year-old male diagnosed with massive PLD complicated by abdominal adhesion,liver cirrho-sis,splanchnic varices and ascites that successfully underwent liver transplantation.展开更多
基金supported by grants from the National S&T Major Project(2017ZX10203205)the Medical Science and Technology Project of Zhejiang Province(2014KYA082)+1 种基金the Fundamental Research Funds for the Central Universities(2018FZA7002)the Shulan Talent Foundation.
文摘Polycystic liver disease(PLD)is a genetic disorder[1]that is di-agnosed by presence of multiple hepatic cysts.Clinical manifesta-tions of PLD are related to the size of hepatic cysts and associated complications.In the early stage,PLD rarely causes any symptoms.However,patients with advanced stage PLD have a variety of clin-ical manifestations and complications due to severe hepatomegaly.They include abdominal pain,abdominal distension,dyspepsia,as-cites and many others that may result from compression of ad-jacent tissue or organ failure[2,3].Surgical treatment of PLD in-cludes:aspiration with or without sclerotherapy,cyst fenestration,hepatectomy and liver transplantation.Despite some researchers have concluded that cyst fenestration or hepatectomy is effective in treatment of PLD[4–6],the only curative surgical option in se-vere patients is liver transplantation[7,8].All other types of surg-eries present a great challenge to a surgeon,having little or no ef-fect when cysts are diffuse,or even contraindicated due to possi-bility of life-threatening complications arising during or after the procedure.Herein,we report a case of 51-year-old male diagnosed with massive PLD complicated by abdominal adhesion,liver cirrho-sis,splanchnic varices and ascites that successfully underwent liver transplantation.
