IS case 509: Polycystic liver disease

Jeremy Sykes, MD

University of Rochester


Imaging Sciences URMC 2010
Publication Date: 2010-09-10

History

Patient is an 84-year-old female with history of flank pain and clinical concern for renal calculus.

Findings

See figures

Diagnosis

Polycystic liver disease

Discussion

Polycystic liver disease is a rare autosomal dominant disorder often found in association with renal polycystic disease. Although hepatic cysts are found in 40% of cases of autosomal dominant polycystic disease involving the kidneys, they may also be seen without renal involvement. The condition is usually asymptomatic and results in liver dysfunction only sporadically, although advanced disease can result in hepatomegaly, liver failure, or Budd-Chiari syndrome. Percutaneous alcohol ablation can be used as an alternative to resection or transplantation in severe cases.

Polycystic liver disease typically appears as multiple to innumerable homogeneous and hypoattenuating cystic lesions with a regular outline on non-enhanced CT scans with no wall or content enhancement on contrast-enhanced images. Cyst contents are often greater than water density due to hemorrhage, or less commonly infection. Calcification of cyst walls is often seen due to old hemorrhage. The cysts neither communicate with each other nor with the biliary tree. Although the diagnosis of polycystic liver disease is easily made with both CT and MR imaging, MR imaging is more sensitive for the detection of complicated cysts.

References

  1. Mortelé KJ, Ros PR. Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics. 2001 Jul-Aug;21(4):895-910. PMID: 11452064
  2. Qian Q, Li A, King BF, Kamath PS, et al. Clinical profile of autosomal dominant polycystic liver disease. Hepatology. 2003 Jan;37(1):164-71. PMID: 12500201

2 images