Vascular Liver Diseases Flashcards

1
Q

What are the 3 categories of obstruction of the venous system of the liver

A

Veno-occlusive disease
Budd-Chiari syndrome
Congestive hepatopathy

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2
Q

Where does the liver arise from embryologically

A

A diverticulum on the ventral surface gut

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3
Q

What are the 3 vessels of the liver

A

Hepatic Artery
Hepatic Vein
Hepatic Portal Vein

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4
Q

What is the function of the hepatic veins

A

To convey the blood away from the liver

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5
Q

What do the hepatic veins converge to form

A

Three large trunks draining into the inferior vena cava

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6
Q

What is the substance of the liver composed of

A

Lobules

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7
Q

What does each lobule consist of

A

A mass of hepatic cells, arranged in irregular radiating columns between which are the blood channels (sinusoids

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8
Q

What is the function of the sinusoids

A

T convey the blood from the circumference to the centre of the lobule ending in the intralobular vein

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9
Q

What does the intralobular vein drain into

A

The sublobular vein

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10
Q

What does the intralobular vein drain into

A

The sublobular vein

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11
Q

What is Budd-Chiari sundrome

A

An uncommon and potentially life-threatening condition

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12
Q

What causes Budd-Chiari syndrome

A

The obstruction of hepatic venous outflow at any level from the small hepatic veins to the junction of the IVC with the right atrium

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13
Q

BCS is more common in women. True or False

A

True

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14
Q

What is the commonest cause of of BCS

A

Intraluminal thrombosis

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15
Q

What are some of the other causes of BCS

A

Malignancy
Parasites
Abscess or cyst
vascular webs

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16
Q

how do the pathological features of BCS result

A

From the increased sinusoidal pressure that occurs with hepatic venous obstruction

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17
Q

Reduced venous perfusion and congestion result in what

A

Hypoxic damage to the liver parenchymal cells, releasing free radicals

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18
Q

What happens if the sinusoidal pressure is reduced

A

The liver function improves

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19
Q

What are the clinical features of BCS

A

Ascites
Hepatomegaly
abdominal pain

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20
Q

What has resulted in a decrease in mortality for BCS patients

A

Introduction of anticoagulation and earlier recognition of asymptomatic disease

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21
Q

What might also be present if IVC compression or thrombosis causes the disease

A

Leg oedema or venous collateral over the trunk and back

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22
Q

What is the diagnosis of BCS dependent on

A

Imaging:
Doppler US - shows hepatic vein obstruction or abnormal flow in large intrahepatic or subcapsular venous collaterals
Contrast CT allows assessment for parenchymal disease, ascites and splenomegaly

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23
Q

What is seen pathologically in BCS

A

A variable degree of parenchymal damage dependent on the location and extent of venous congestion

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24
Q

What is characteristic in the perivenular areas in BCS

A

Ischaemic necrosis and fibrosis

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25
Q

What are the 3 main aims of managing BCS

A

Alleviate the obstruction
Prevent extension of thrombosis
Preserve hepatic function by decreasing centrilobular congestion

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26
Q

What is involved in the medical management of BCS

A

Control the ascites (low sodium diet, diuretics or paracentesis
Prevent thrombosis extension (anticoagulation)
Treat complications
Investigate and treat underlying cause

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27
Q

How might restoration of hepatic blood flow be achieved by

A

Thrombolytic therapy (fresh thrombus)
Percutaneous angioplasty
Transjugular intrahepatic portosystemic shunt (TIPS)
Portosystemic shunt surgery

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28
Q

What does TIPS do

A

Provides an alternative venous outflow tract to decompress the liver
Useful as a bridge to liver transplantation and in acute situations

29
Q

What does TIPS do

A

Provides an alternative venous outflow tract to decompress the liver
Useful as a bridge to liver transplantation and in acute situations

30
Q

Why is a liver biopsy not accurate in determining the prognosis of BCS

A

There is an uneven distribution of hepatic lesions in BCS

31
Q

Why is a liver biopsy not accurate in determining the prognosis of BCS

A

There is an uneven distribution of hepatic lesions in BCS

32
Q

What is Hepatic Veno-occlusive disease

A

It typically occurs after heamatopoietic stem cell transplantation but also after ingestion of pyrrolizidine alkaloids

33
Q

What are the histological features of Veno-occlusive disease

A

Loss of sinusoidal endothelial cell
appearance of gaps in the SEC barrier
Narrowing of the sublobular and venous lumena become obliterated

34
Q

What are some of the clinical features of Veno-occlusive disease

A
signs are symptoms develop within 3 weeks of exposure in the case of HSCT and primary those of 
tender hepatomegaly, 
fluids retention
ascites
jaundice 
RUQ pain
35
Q

What are the risk factors for the development of VOD

A

Advanced age
Presence of liver injury prior to HSCT
Clotting cascade mutations

36
Q

What is the fold standard investigation for VOD

A

Liver biopsy

37
Q

What is the preventative measures against VOD

A

modifying the conditioning regimen in patients at increased risk of VOD
Use of ursodeoxycholic acid as prophylaxis
Heparin infusion may be useful but risk of bleeding is high

38
Q

What is the treatment for VOD

A

Simply diuretics and sodium restriction

Repeated paracentesis may be required

39
Q

What should be avoided in VOD

A

Hepatotoxic drugs

40
Q

What is portal vein thrombosis

A

Occurs most commonly as a complication of cirrhosiss, particularly in decompensated disease

41
Q

What are the 4 anatomical categories for PVT

A

Thrombus confined to the portal vein beyond confluence with the SMV
Extension into the SMV but patent mesenteric vessels
Diffuse splanchnic venous involvement with large collaterals
Splanchnic involvement but extensive fine collaterals

42
Q

What are the 4 anatomical categories for PVT

A

Thrombus confined to the portal vein beyond confluence with the SMV
Extension into the SMV but patent mesenteric vessels
Diffuse splanchnic venous involvement with large collaterals
Splanchnic involvement but extensive fine collaterals

43
Q

What does Portal cavernoma formation or cavernous transformation of the portal vein result from

A

The development of multiple small vessels in and around the recanalising or occluded main portal vein

44
Q

What are some of the clinical features of a portal vein thrombosis

A

Acute: onset is suggested by the absence of clinical, endoscopic and radiological evidence of portal hypertension, typically thrombosis occurring

45
Q

What is the most common complication of PVT

A

Variceal bleeding

46
Q

What are the investigations required in PVT

A

Doppler ultrasound

Contrast CT

47
Q

What is the management in PVT

A

Reserve or prevent the advancement of thrombosis and to treat the complications

48
Q

How is variceal bleeding managed

A

Endoscopic therapy / medical therapy including non-selective B blockers and nitrates

49
Q

What might be of benefit where splenomegaly causes hypersplenism

A

Splenectomy

50
Q

What are the 5 main causes of congestive hepatopathy and cardiac cirrhosis

A
Ischaemic heart disease 
Cardiomyopathy 
Valvular heart disease 
Restrictive lung disease 
Pericardial disease
51
Q

What is the classical pathological description of congestive hepatopathy and cardiac cirrhosis

A

Nutmeg liver

52
Q

What are the clinical features of congestive hepatopathy and cardiac cirrhosis

A

Liver dysfunction is mild and asymptomatic

mild jaundice and RUQ pain in more severe congestion

53
Q

What are the clinical features of congestive hepatopathy and cardiac cirrhosis

A

Liver dysfunction is mild and asymptomatic

mild jaundice and RUQ pain in more severe congestion

54
Q

What are the symptoms of congestive hepatopathy and cardiac cirrhosis

A

Exertional dyspnoea
orthopnoea
angina

55
Q

Presence of what murmur may make the liver pulsatile

A

Tricuspid regurgitation

56
Q

What investigations are carried out for congestive hepatopathy and cardiac cirrhosis

A

Liver biochemistry:
Hyperbilirubinaemia is mostly unconjugated
Alkaline phosphatase is only mildly elevated
Viral hepatitis serology
Abdominal US with Doppler studies of the liver
Liver biopsy
ECG and Echo

57
Q

What is the management for congestive hepatopathy and cardiac cirrhosis

A

Treating the underlying heart disease

Diuresis for jaundice and ascites

58
Q

Liver cell injury resulting from subcritical supply of oxygen to hepatocytes is traditionally classified as what 2 things

A
Inadequate blood supply due to reduced hepatic arterial flow and or passive venous congestion (heart failure) termed ischaemic hepatitis 
Hypoxic insult (respiratory failure) termed hypxoc hepatitis
59
Q

What is the final common pathway in ischaemic/ hypoxic hepatitis

A

Hepatocellular dysfunction secondary to critically low levels of oxygen for metabolic processes

60
Q

Hypoxic injury to the liver is not reversible. True or false.

A

False- it is reversible

61
Q

What are the 5 main aetiologies for hypoxic hepatitis

A
Primary heart disease
Congestive heart failure 
Acute MI 
Chronic respiratory failure 
Circulatory shock and sepsis
62
Q

What results in increased oxygen consumption or decreased oxygen availability

A

Hypoperfusion

63
Q

What are some of the histological findings in hypoxic hepatitis

A
central hepatic vein congestion with centrilobular hepatic necrosis 
fragmentation of liver bulks 
polymorphonuclear cell infiltration
abnormal hepatocyte complexes
pyknosis
disintegration of hypatocyte nuclei
64
Q

What are absent which are present in other forms of hepatitis

A

Hyperplasia
Inflammation
regeneration

65
Q

What are the clinical features of hypoxic hepatitis

A

Nausea and vomiting

Tender and enlarged liver

66
Q

What are risk factors for hypoxic hepatitis

A
Acute and chronic heart failure 
respiratory failure
sepsis 
prolonged hypotension
toxin ingestion
Heat stroke
67
Q

What are the investigations involved for Hypoxic hepatitis

A

Diagnosis is based on clinical and biochemical criteria generally without the need for procedural intervention
Transient serum enzyme elevation in conjunction with abnormal renal function and abnormalities in PT and APTT activity

68
Q

What is the management for patients with Hypoxic hepatitis

A

Purely supportive and focuses on correcting the underlying conditions leading to hypotension and hypoxic and hepatic hypoperfusion