Polycystic Liver Disease

Embryonic ductal malformation of the intrahepatic tree; numerous cysts throughout liver parenchyma

Aetiology

  • Inherited disorder

Pathophysiology

Von Meyenburg complexes (VMC)

  • Benign cystic nodules throughout the liver, originating from the peripheral biliary tree
  • Remnants develop into small hepatic cysts and usually remain silent during life - incidental finding

Polycystic liver disease

  • Liver function preserved, renal failure rare
  • PCLD genes - PRKCSH and SEC63

Autosomal dominant polycystic kidney disease

  • Renal failure due to polycystic kidneys
  • ADPKD genes - PKD1 and PKD2

Clinical presentation

  • Abdominal pain
  • Abdominal distension
  • Atypical symptoms due to compression of tissue or failure of affected organ

Investigations

  • USS

Management

  • Conservative treatment is recommended to halt cyst growth
  • Some patients with PCLC/ADPKD/liver failure may require defenestration, aspiration or a liver transplant
  • Somatostatin analogues - symptom relief, reduce liver volume