[1] Liver Cysts Flashcards

1
Q

Are cystic diseases of the liver common?

A

Relatively

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2
Q

How are cystic diseases of the liver most commonly identified?

A

Incidentally on routine imaging

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3
Q

Are cystic diseases of the liver serious?

A

In rare cases they can be complicated and life-threatening

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4
Q

What are simple liver cysts?

A

Simple, fluid-filled epithelial-lined sacs within the liver

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5
Q

Where do simple liver cysts most commonly occur?

A

In the right lobe

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6
Q

What is the prevalence of simple liver cysts?

A

2.5-18%

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7
Q

What happens to the incidence of simple liver cysts with age?

A

Increases

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8
Q

What are simple liver cysts due to?

A

Thought to be due to congenitally malformed bile ducts, failing to connect the extrahepatic ducts

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9
Q

What does the failure of hepatic bile ducts to connect with extrahepatic ducts lead to?

A

Local dilation filled with bile-like fluid

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10
Q

How are most simple liver cysts found?

A

Incidentally on imaging

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11
Q

What % of patients with simple liver cysts experience symptoms

A

10-15%

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12
Q

What symptoms may be produced by simple liver cysts?

A

Abdominal pain
Nausea
Early satiety

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13
Q

What typically causes the symptoms of simple liver cysts?

A

Mass effect on the surrounding structures

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14
Q

Is there a correlation between increased size of simple liver cysts and increased incidence of complications?

A

Yes, there seems to be

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15
Q

What investigations are done in suspected simple liver cysts?

A

Bloods

Ultrasound

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16
Q

What may be found on bloods in simple liver cysts?

A

LFTs are typically normal, although a small number of patients may have raised GGT
Tumour markers CEA and CA19-9 may be elevated

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17
Q

How do simple liver cysts appear on ultrasound?

A

They are characteristically anechoic, well-defined, thin-walled (often imperceptible), oval/spherical lesions with no septations, and strong posterior wall acoustic enhancement

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18
Q

Do simple liver cysts require intervention?

A

Most require no intervention

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19
Q

What is recommended for simple liver cysts ?>4cm in size?

A

Follow-up ultrasound scans at 3, 6, and 12 months post-detection to check for growth

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20
Q

What is done if the size of simple liver cysts remains unchanged after 2-3 years of ultrasound follow up?

A

No further scans are required unless the patient becomes symptomatic

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21
Q

When may further intervention be warranted in simple liver cysts?

A

If the patient is symptomatic or the diagnosis is uncertain

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22
Q

What are the options for intervention in simple liver cysts?

A

Ultrasound-guided aspiration

Laparoscopic de-roofing

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23
Q

What is characteristic of simple liver cysts on laparoscopy?

A

A ‘blue hue’

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24
Q

What is the advantage of laparoscopy over aspiration in the management of simple liver cysts?

A

Has lower rates of failure and recurrence

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25
Q

What is polycystic liver disease characterised by?

A

The presence of 20 or more cysts within the liver parenchyma, each of which are 1cm or more in size

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26
Q

What is polycystic liver disease caused by?

A

Autosomal dominant polycystic kidney disease or autosomal dominant polycystic liver disease

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27
Q

What is autosomal dominant polycystic kidney disease caused by?

A

Mutations in the PKD1 and PKD2 genes

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28
Q

Where is the PKD1 gene found?

A

Chromosome 16

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29
Q

Where is the PKD2 gene found?

A

Chromosome 4

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30
Q

What % of patients with autosomal dominant polycystic kidney disease will also develop liver cysts?

A

10-60%

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31
Q

What causes autosomal dominant polycystic liver disease?

A

Mutations in the PRKCSH or SEC63 genes

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32
Q

Where is the PRKCSH gene found?

A

Chromosome 19

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33
Q

Where is the SEC63 gene found?

A

Chromosome 6

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34
Q

Will patients with autosomal dominant polycystic liver disease have renal involvement?

A

No

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35
Q

How do the mutations in the genes causing autosomal dominant polycystic liver and kidney disease cause liver cysts?

A

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

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36
Q

Is polycystic liver disease symptomatic?

A

The majority of patients are asymptomatic

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37
Q

What may symptoms result from in polycystic liver disease?

A

Localised compression or complications

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38
Q

How do patients with symptomatic polycystic liver disease present?

A

Abdominal pain present as the cysts grow in size, and hepatomegaly being present on examination

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39
Q

How might concurrent renal disease present in polycystic liver disease?

A

Additional urinary tract symptoms

40
Q

What will significant disease eventually cause in polycystic liver disease?

A

Liver cirrhosis and portal hypertension

41
Q

What may bloods show in polycystic liver disease?

A

Patient will have normal LFTs (ALP can become raised in a small proportion)
Renal function may be affected in those who also have renal cysts

42
Q

How is a definitive diagnosis of polycystic liver disease made?

A

Ultrasound imaging

43
Q

What is found on ultrasound imaging in polycystic liver disease?

A

Multiple cysts, usually 20 or more, which have the same sonographic characteristics as simple cysts

44
Q

How can asymptomatic polycystic liver disease be managed?

A

Patients can be left alone and monitored

45
Q

Will patients with asymptomatic polycystic liver disease eventually need some form of intervention?

A

Many patients will, due to the progressive nature of the disease

46
Q

What medical management may be of use in polycystic liver disease?

A

Some trials have demonstrated the short-term benefit for somatostatin analogues in symptomatic relief

47
Q

How do somatostatin analogues work in providing symptomatic relief for polycystic liver disease?

A

They reduce cyst volume

48
Q

What are the indications for surgery for cystic liver lesions?

A

Intractable symptoms
Inability to rule out malignancy on imaging alone
Prevention of malignancy

49
Q

What are the surgical options for the treatment of polycystic liver disease?

A

US-guided aspiration

Laparoscopic de-roofing of cysts

50
Q

What is the use of US-guided aspiration in polycystic liver disease?

A

May provide temporary relief in patients experiencing pain due to cyst size

51
Q

Why is US-guided aspiration not used routinely in polycystic liver disease?

A

Due to fluid re-accumulation

52
Q

Where is laparoscopic de-roofing of cysts the preferred technique in polycystic liver disease?

A

In those experiencing symptoms, or for those whom there is evidence of compression of surrounding structures

53
Q

What treatment option can be employed where particular liver segments are grossly affected by polycystic liver disease?

A

Resection

54
Q

What treatment options may be warranted in extreme cases of polycystic liver disease?

A

Transplantation

55
Q

What % of liver cysts are true cystic neoplasms of the liver?

A

<5%

56
Q

What is the most common subtype of cystic neoplasms of the liver?

A

Cystadenoma

57
Q

What are cystadenomas?

A

Non-invasive mucinous cystic neoplasms

58
Q

Are cystadenomas malignant?

A

No, they are pre-malignant lesions

59
Q

What causes the development of cystadenomas?

A

Abnormal proliferation of the biliary epithelium

60
Q

What can cystadenomas transform into?

A

Can undergo malignant transformation into cystadenocarcinomas in around 10% cases

61
Q

Are cystic neoplasms of the liver always symptomatic?

A

No, patients are commonly asymptomatic

62
Q

What is the rate of growth of cystic neoplasms of the liver?

A

Slow, typically 1-2mm per year

63
Q

What is the result of the slow growth of cystic neoplasms of the liver?

A

Symptoms may develop insidiously

64
Q

What are the common symptoms of cystic neoplasms of the liver in symptomatic individuals?

A
Abdominal pain
Anorexia
Nausea
Fullness
Bloating
65
Q

What may the blood shows in cystic neoplasms of the liver?

A

LFTs are often normal, although ALP, CEA< and CA19-9 can become mildly elevated

66
Q

What can be used to differentiate between simple cysts and more complicated cystic lesions in the liver?

A

Ultrasound scanning

67
Q

What should be performed on all patients in whom a cystic neoplasm is suspected?

A

CT imaging with contrast

68
Q

Why should CT imaging with contrast be performed on all patients with suspected cystic neoplasms?

A

For further delineation and to check for evidence of metastasis

69
Q

Why should aspiration or biopsy be avoided if a cystic neoplasm is suspected?

A

It can result in potential peritoneal seeding of the malignancy

70
Q

What features can be seen on imaging for liver cysts that are suspicious for malignancy?

A

Septations
Wall enhancement
Nodularity

71
Q

What features can be seen on imaging for liver cysts that are suspicious for an abscess?

A

Debris within the lesions

Loculation

72
Q

What features can be seen on imaging that are suspicious for hydatid cysts?

A

Calcification

‘Daughter cysts’ around the main lesion

73
Q

What is the treatment of choice for cystadenomas and cystadenocarcinomas?

A

Liver lobe resection

74
Q

What happens following liver lobe resection in cystadenomas and cystadenocarcinomas?

A

They are sent for histopathology to confirm the diagnosis

75
Q

What does a hydatid cyst result from?

A

Infection with the tapeworm Echinococcus granulosus

76
Q

How is Echinococcus granulosus transmitted?

A

The eggs are passed by faeco-oral transmission

77
Q

What excretes the Echinococcus granulosus larvae?

A

Carnivores, commonly dogs

78
Q

What happens once the Echinococcus granulosus larve have entered the hosts GI tract?

A

They pass into the hepatic portal system into the liver, where they continue to grow and form cysts

79
Q

Where is echinococcal disease found?

A

Has a global distribution, though the highest prevalence is in South America, North Africa and Central Asia

80
Q

How quickly can hydatid cysts grow?

A

Many only grow at a rate of a couple of mm’s a year

81
Q

What is the result of hydatid cysts only growing at a rate of a couple of mm’s per year?

A

They can remain asymptomatic and undetected for many years

82
Q

What is the most common presenting symptom of hydatid cysts?

A

Vague abdominal pain

83
Q

What causes the abdominal pain in hydatid cysts?

A

Mass effect on the surrounding structures, or due to rupture

84
Q

What presentations can hydatid cysts result in?

A
Jaundice
Cholangitis 
Vomiting
Dyspepsia
Early satiety
Anaphylaxis
85
Q

When can hydatid cysts cause cholangitis?

A

If the biliary system is involved

86
Q

When can hydatid cysts cause anaphylaxis?

A

If the cyst ruptures into the thorax or intraperitoneally

87
Q

What may be found on bloods in a hydatid cyst?

A

LFTs are often abnormal, unless presenting with cholangitis picture
FBC can show eosinophilia

88
Q

What % of those with hydatid cysts have positive Echinococcal antibody titres?

A

80%

89
Q

What will be shown on ultrasound scanning with hydatid cysts?

A

Calcified, spherical lesion with multiple septations

May be anechoic or containing snowflake-like inclusions

90
Q

How can further imaging assessment of a hydatid cyst be performed?

A

CT imaging with contrast

91
Q

Why is aspiration not recommended in those with suspeted hydatid cysts?

A

It may rupture, which can cause an anaphylactic reaction

92
Q

How can hydatid cysts be managed if they are asymptomatic and inactive?

A

May be possible to monitor them

93
Q

What is the primary treatment for hydatid cysts?

A

Surgical cyst deroofing

94
Q

What are the options for the surgical management of hydatid cysts in specialist centres?

A

Radiological agents and injection of a scolecidal agent

95
Q

What is the use of medical management in hydatid cysts?

A

It is used as an adjunct to surgical therapy

96
Q

Who is medical management of hydatid cysts used for?

A

Those with widely disseminated hydatid disease, or in patients who are unfit for surgery

97
Q

What is given to patients with hydatid cysts whom are in need of active treatment?

A

Anti-microbial action varies, however a combination of albendazole, mebendazole, and/or praziquantel is normally given