Polycystic liver disease

Polycystic liver disease

  • Not the same as PKD, genetically they are different.
  • ADPLD is linked to a mutation in a protein kinase C substrate, appropriately called hepatocystin, which causes abnormal biliary cell proliferation and configuration.
  • The majority of patients are asymptomatic, and the condition is discovered incidentally.
    – Liver function is generally maintained.
    – Portal hypertension secondary to ADPLD is rare and must prompt investigation of vascular thrombosis.
  • Ultrasound demonstrates multiple smooth-walled, round, anechoic structures with distal echo enhancement.
  • CT demonstrates multiple round cysts with distinct margins, which may be large, with fluid attenuation and no contrast enhancement.
  • Irregular borders, septations, rapid growth -> consider infection, neoplasm or hemorrhage.
  • Ddx: multiple mets, VHL disease, ADPKD with liver cysts
  • Mx:
    i) Surgical: Single cyst – aspiration & sclerotherapy; multiple cysts: fenestration (aspiration and deroofing)
    – There are risks of recurrence of the cysts as the underlying pathophysiology is not addressed.
    – Liver transplantation as last option.

    ii) Medical
    – Octreotide: inhibit cAMP which is responsible cholangiocyte proliferation and biliary epithelium fluid secretion.

Reference: radiopaedia.org, auntminnie.com

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