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
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.
– Octreotide: inhibit cAMP which is responsible cholangiocyte proliferation and biliary epithelium fluid secretion.
Reference: radiopaedia.org, auntminnie.com