[1] Liver Cysts Flashcards
Are cystic diseases of the liver common?
Relatively
How are cystic diseases of the liver most commonly identified?
Incidentally on routine imaging
Are cystic diseases of the liver serious?
In rare cases they can be complicated and life-threatening
What are simple liver cysts?
Simple, fluid-filled epithelial-lined sacs within the liver
Where do simple liver cysts most commonly occur?
In the right lobe
What is the prevalence of simple liver cysts?
2.5-18%
What happens to the incidence of simple liver cysts with age?
Increases
What are simple liver cysts due to?
Thought to be due to congenitally malformed bile ducts, failing to connect the extrahepatic ducts
What does the failure of hepatic bile ducts to connect with extrahepatic ducts lead to?
Local dilation filled with bile-like fluid
How are most simple liver cysts found?
Incidentally on imaging
What % of patients with simple liver cysts experience symptoms
10-15%
What symptoms may be produced by simple liver cysts?
Abdominal pain
Nausea
Early satiety
What typically causes the symptoms of simple liver cysts?
Mass effect on the surrounding structures
Is there a correlation between increased size of simple liver cysts and increased incidence of complications?
Yes, there seems to be
What investigations are done in suspected simple liver cysts?
Bloods
Ultrasound
What may be found on bloods in simple liver cysts?
LFTs are typically normal, although a small number of patients may have raised GGT
Tumour markers CEA and CA19-9 may be elevated
How do simple liver cysts appear on ultrasound?
They are characteristically anechoic, well-defined, thin-walled (often imperceptible), oval/spherical lesions with no septations, and strong posterior wall acoustic enhancement
Do simple liver cysts require intervention?
Most require no intervention
What is recommended for simple liver cysts ?>4cm in size?
Follow-up ultrasound scans at 3, 6, and 12 months post-detection to check for growth
What is done if the size of simple liver cysts remains unchanged after 2-3 years of ultrasound follow up?
No further scans are required unless the patient becomes symptomatic
When may further intervention be warranted in simple liver cysts?
If the patient is symptomatic or the diagnosis is uncertain
What are the options for intervention in simple liver cysts?
Ultrasound-guided aspiration
Laparoscopic de-roofing
What is characteristic of simple liver cysts on laparoscopy?
A ‘blue hue’
What is the advantage of laparoscopy over aspiration in the management of simple liver cysts?
Has lower rates of failure and recurrence
What is polycystic liver disease characterised by?
The presence of 20 or more cysts within the liver parenchyma, each of which are 1cm or more in size
What is polycystic liver disease caused by?
Autosomal dominant polycystic kidney disease or autosomal dominant polycystic liver disease
What is autosomal dominant polycystic kidney disease caused by?
Mutations in the PKD1 and PKD2 genes
Where is the PKD1 gene found?
Chromosome 16
Where is the PKD2 gene found?
Chromosome 4
What % of patients with autosomal dominant polycystic kidney disease will also develop liver cysts?
10-60%
What causes autosomal dominant polycystic liver disease?
Mutations in the PRKCSH or SEC63 genes
Where is the PRKCSH gene found?
Chromosome 19
Where is the SEC63 gene found?
Chromosome 6
Will patients with autosomal dominant polycystic liver disease have renal involvement?
No
How do the mutations in the genes causing autosomal dominant polycystic liver and kidney disease cause liver cysts?
They result in aberrant ductal plate configurations during liver embryogenesis, which are not connected to the intrahepatic bile ducts and so do not drain, leading to dilation and eventual cyst formation as they progressively fill with bile-like fluid
Is polycystic liver disease symptomatic?
The majority of patients are asymptomatic
What may symptoms result from in polycystic liver disease?
Localised compression or complications
How do patients with symptomatic polycystic liver disease present?
Abdominal pain present as the cysts grow in size, and hepatomegaly being present on examination