Table 1

Main characteristics of patients with peribiliary cysts

CharacteristicsAvailable valuesResults
patients% or median (range)
Age, years12263 (4–88)
Males12298/122 (80%)
Japanese patients13588/135 (65%)
Associated conditions/diseases*
Cirrhosis (of all aetiologies)13552/135 (38%)
Portal hypertension11740/117 (34%)
Portal vein thrombosis11712/117 (10%)
Hepatic, biliary or pancreatic neoplasms (%)13523/135 (17%)
ADPKD13515/133 (11%)
Liver transplantation1355/135 (3%)
Primary sclerosing cholangitis1352/135 (1.5%)
No associates conditions/diseases13521/135 (15%)
Clinical manifestations
Asymptomatic patients10431/104 (30%)
Symptomatic patients10473/104 (70%)
Symptoms related to associated conditions/diseases7338/73 (52%)
Symptoms related to peribiliary cyst(s)7321/73 (29%)
Symptoms related to associated diseases and PBC(s)7314/73 (19%)
Complications of peribilary cyst(s)
Dilated biliary tract (slight or severe)12647/126 (36%)
Obstructive jaundice12618/126 (14%)
Cholangitis10413/104 (12.5%)
Surgical interventions
Surgical interventions13561/133 (45%)
Therapeutic misadventures related to incorrect diagnosis6120/61 (33%)
Duration of follow-up, months4018 (1–92)
Increase in number and/or size of cysts4015/40 (37.5%)
Overall mortality12229/122 (24%)
Death related to decompensated cirrhosis2117/21 (81%)
  • *Some patients have more than one associated disease.

  • ADPKD, autosomal dominant polycystic kidney disease.