Polycystic Liver Disease: Experience at a Teaching Hospital

Abstract

Most patients in this highly selected cohort were asymptomatic with normal hepatic function. Pain was the most common symptom. The natural history is variable however, with some patients developing complications including portal hypertension. Minimally invasive interventions are appropriate initially, with hepatic resection and liver transplantation reserved for those with severe symptoms or life-threatening complications.

Imaging in ductal plate malformations

“…[10] These cysts are a result of malformation of the embryonic ductal plate, with formation of von Meyenburg complexes lined with functional biliary epithelium. [11] Two types of cysts may be seen in the liver in hereditary PCLD, namely, intrahepatic and peribiliary cysts. [12] The presence of more than 20 liver cysts is considered to be hereditary polycystic liver and replace over 50% of the hepatic parenchyma [Figure 7].…”

Medical and surgical treatment options for polycystic liver disease1

“…2 The elevated AP and gGT levels probably reflect activation of cholangiocytes. 9,[23][24][25][26] Serum transaminases are normal or only mildly elevated. 2 Bilirubin is rarely elevated but in advanced cases jaundice may arise due to compression of the common bile duct secondary to a strategically located cyst.…”

Isolated Polycystic Liver Disease

“…Morphological studies of individual liver cysts reveal that liver cysts originate from biliary microhamartomas, also termed von Meyenburg’s complexes(14) that arise by proliferation of biliary ductules, and from peribiliary glands(2, 15, 16). As the cysts enlarge, they typically become detached from their origins.…”