Polycystic liver disease

Section: Hepatobiliary Sub-section: Liver Curriculum: Curriculum, page 85

Definition

• Multiple hepatic cysts.
• Similar to simple Liver cysts, it is a congenital malformation of biliary ducts.
• Three PLD entities are recognised in adults:
  1. Von Meyenburg complexes (biliary hamartoma; hepatic cystic hamartoma) with characteristic small, non-hereditary nodular cystic lesions;
  2. Isolated PLD with innumerable hepatic cysts (autosomal dominant; PCLD)
  3. Autosomal dominant polycystic kidney disease (ADPKD) with cysts in both kidneys and in many cases hepatic cysts.

Epidemiology

• Von Meyenburg complexes is estimated at 0.7–5.6%
• ADPKD 1/400 to 1/1000
• PCLD is 1/100,000 to 1/1,000,000.

Clinical

• The main difference between ADPKD and PCLD is the presence of polycystic kidneys.
• The prognosis of these diseases is different, as the majority of ADPKD patients develop enlarged kidneys and end-stage renal disease.
• In patients with PCLD, a few renal cysts may be present, but this does not result in renal failure.

Investigations

Imaging is similar to that for simple cysts

Several clinical classifications have been proposed to grade the severity of PLD, e.g. Gigot’s classification, Qian’s classification and Schnelldorfer’s classification. These classifications use factors such as symptoms, number and size of cysts to grade the severity of PLD

Management

• The primary aim of treatment is to
  • Reduce symptoms
• Treatment varies depending on the phenotype of PLD; patients with a few large cysts require a different approach to those with many small cysts.

Asymptomatic

• No therapy is warranted
• Women should be advised to stop oral contraceptives.
• Treatment with somatostatin analogues has shown a reduction in liver volume after 6 months of therapy, but only a modest improvement in quality of life. e

Large symptomatic dominant cysts

• Aspiration sclerotherapy
• Laparoscopic cyst deroofing.
• Recurrence and reintervention rates are high
• In selected cases, hepatic resection combined with fenestration may be an option.
• Liver transplantation for PLD is uncommon.
  • Indications for liver transplantation are progressive sarcopenia from inability to eat combined with pain and immobility due to a massive liver, and Budd–Chiari-like syndrome with the development of ascites.
  • In patients with both complicated PLD and kidney failure, a combined liver and kidney transplant is often performed.