Seminar about vascular disorders of the liver, their classification, causes, diagnosis and treatment.

1. Vascular Disorders of the Liver
By
Ahmed Abudeif Abdelaal
Assistant Lecturer of Tropical Medicine & Gastroenterology
Sohag Faculty of Medicine
December, 2017

2. Introduction
- All liver disorders related to a primary injury to hepatic
vessels qualify as rare diseases, affecting less than 5/10000
patients.
- Their recognition requires a high degree of suspicion, as
well as awareness and expertise in the interpretation of
vascular imaging and liver histopathology.

3. Introduction
- Many patients with primarily vascular disorders of the liver
will be misdiagnosed as having common liver diseases,
particularly cirrhosis.

4. Classification of vascular disorders of the liver
A) Disorders of the hepatic arteries:
1. Hepatic artery occlusion.
2. Hepatic artery aneurysm.
3. Arterioportal and arteriovenous fistulae.
B) Disorders of the hepatic veins and IVC:
• Budd-Chiari syndrome (BCS).

5. Classification of vascular disorders of the liver
C) Disorders of extrahepatic portal vein:
1. Portal vein thrombosis (PVT).
2. Congenital portohepatic shunts.
3. Portal vein aneurysm.
D) Disorders of intrahepatic portal venous system:
1. Obliterative portal venopathy.
2. Portohepatic fistulae.

6. Classification of vascular disorders of the liver
E) Disorders of sinusoids:
1. Sinusoidal obstruction syndrome (SOS).
2. Sinusoidal dilatation and peliosis hepatis.
3. Sinusoidal fibrosis.

7. Disorders of the hepatic arteries
A) Hepatic artery occlusion:
- Very rare condition.
 Causes:
1) Polyarteritis nodose (PAN).
2) Giant cell arteritis.
3) Embolism as in infective endocarditis.
4) Tied during surgery e.g. cholecystectomy.
5) Trauma as in laparoscopic cholecystectomy.

8. Disorders of the hepatic arteries
A) Hepatic artery occlusion:
 Clinical picture: until now the condition is highly fatal.
- Features of the cause.
- Sudden pain in the right upper abdomen, followed by
collapse and hypotension.
- RUQ tenderness.
- The condition progresses to liver cell failure.

9. Disorders of the hepatic arteries
A) Hepatic artery occlusion:
 Diagnosis:
1) Hepatic angiography: the obstruction will be shown.
2) Scanning: show infarcts and maybe the occluded vessels.
 Treatment:
Treatment of the cause, and LCF if present.

10. Hepatic angiogram showing
occluded hepatic artery
CT abdomen show abscess formation
secondary to liver infarction after
hepatic artery thrombosis

11. Hepatic artery thrombosis, results in
hilar infarction involves the major bile
ducts, and extends into the corpus of the
liver.

12. Disorders of the hepatic arteries
B) Hepatic artery aneurysm:
- Rare, but make up to 1/5 of all visceral aneurysms.
 Causes:
1) Congenital.
2) Blunt or penetrating trauma (including liver biopsy).
3) PAN.
4) Infective endocarditis.
5) Atherosclerosis.

13. Disorders of the hepatic arteries
B) Hepatic artery aneurysm:
 Clinical picture:
- Abdominal pain is frequent.
- The classical triad of jaundice, abdominal pain, haemobilia is
present in only 1/3 of the cases.
- The majority of patients present for the first time with
rupture resulting in haemoperitoneum, haemobilia or
haematemesis.

14. Disorders of the hepatic arteries
B) Hepatic artery aneurysm:
 Diagnosis:
- The diagnosis is suggested by sonography and confirmed
by hepatic arteriography and a CT scan after enhancement.
 Treatment:
- Angiographic embolization or surgical ligation.

15. Selective hepatic artery angiogram
showing aneurysm of the common
hepatic artery

16. Multiplanar reformation CT
angiography image showing
aneurysm of the right branch of
the hepatic artery with normal
common hepatic artery

17. Disorders of the hepatic arteries
C) Arterioportal and arteriovenous fistulae:
 Causes:
1) Blunt or penetrating trauma (including liver biopsy).
2) Neoplasm usually HCC.
3) Rupture of hepatic artery aneurysm.
4) Part of hereditary haemorrhagic telangiectasia.

18. Disorders of the hepatic arteries
C) Arterioportal and arteriovenous fistulae:
 Clinical picture:
- Arterioportal shunts may present with portal hypertension,
heart failure, while arteriovenous shunts may present with
heart failure.
- Large shunts cause a bruit in the RUQ.

19. Disorders of the hepatic arteries
C) Arterioportal and arteriovenous fistulae:
 Diagnosis:
- Confirmed by hepatic angiography.
 Treatment:
- Embolization with particles and/or placement of occluding
devices.

20. Arterial Phase Coronal CT shows a
fistula between the right hepatic artery
and right portal vein with resulting
severe dilatation of the main portal
vein. Yellow Arrow: Right Hepatic
Artery. Blue Arrow: Right Portal Vein

21. Disorders of the hepatic veins and IVC
- Obstruction of the hepatic veins and terminal IVC
corresponds to Budd-Chiari syndrome (BCS).
Budd-Chiari syndrome:
 Definition:
- obstruction of hepatic venous outflow that can be located
from the small hepatic venules up to the entrance of the IVC
into the right atrium.

22. Disorders of the hepatic veins and IVC
Budd-Chiari syndrome:
 Definition:
- Hepatic outflow obstruction related to cardiac disease,
pericardial disease or sinusoidal obstruction syndrome
(SOS) are excluded from this definition.

23. Disorders of the hepatic veins and IVC
Budd-Chiari syndrome:
 Classification:
- BCS can be classified into:
A) Primary: caused by thrombosis in the absence of
compression by space occupying lesions, or invasion by
malignancy or parasites.
B) Secondary: otherwise.

24. Prothrombotic conditions and
risk factors that have been
associated with BCS, PVT, or
obliterative portal venopathy

25. Disorders of the hepatic veins and IVC
Budd-Chiari syndrome:
 Clinical picture:
- Ranges from asymptomatic to fulminant LCF.
- Acute form: picture of acute LCF.
- Chronic form: the patient may present with triad of
abdominal pain, hepatomegaly, ascites. Negative
hepatojugular reflux. Finally LCF and portal hypertension
occurs.

26. Disorders of the hepatic veins and IVC
Budd-Chiari syndrome:
 Clinical picture:
- Asymptomatic BCS: account for up to 15% of cases. The
condition is diagnosed accidentally, either by imaging or by
the investigation of abnormal liver function tests.

27. Disorders of the hepatic veins and IVC
Budd-Chiari syndrome:
 Diagnosis:
- Early diagnosis depends on doppler US and contrast
enhanced MRI, where abnormalities in the direction of flow
in the hepatic vein and IVC, is revealed.
- CT and/or angiography can be used.

28. Hepatic venogram in a patient with
BCS. Note the lace-like spider-web
pattern.
MRI in a patient with the BCS showing a liver
(L) which is dyshomogeneous, the aorta (A)
and the inferior vena cava (V). The side-to-
side narrowing of the inferior vena cava
(arrows) is due to the enlarged caudate lobe.

29. Contrast-enhanced CT demonstrates lack of
enhancement in the right hepatic vein (arrowhead)

30. Disorders of the hepatic veins and IVC
Budd-Chiari syndrome:
 Treatment:
- Early treatment of the underlying cause.
- Long term anticoagulation is given to all patients.
- Angioplasty/stenting should be considered in patients with
short segment hepatic vein stenosis, IVC stenosis or to
dilate webs.

31. Disorders of the hepatic veins and IVC
Budd-Chiari syndrome:
 Treatment:
- TIPS, should be considered if anticoagulation and/or
angioplasty/stenting failed.
- Surgical portosystemic shunts are indicated only if TIPS is
not available or cannot be fashioned.
- Liver transplantation is indicated when TIPS/surgical
shunting have failed.

32. Disorders of extrahepatic portal vein
A) Portal vein thrombosis:
- This is the most common of the vascular disorders of the
liver.
- Portal vein thrombosis (PVT) may be:
1. Acute.
2. Chronic.

33. Disorders of extrahepatic portal vein
A) Portal vein thrombosis:
- Acute PVT is defined as a recent formation of a thrombus
within the portal vein and/or right or left branches.
- The thrombus may extend into the mesenteric or splenic
veins; occlusion may be complete or partial.

34. Disorders of extrahepatic portal vein
A) Portal vein thrombosis:
- Following acute thrombosis, in the absence of
recanalization, the portal venous lumen obliterates and
portoportal collaterals develop. This process is called
cavernomatous transformation, the result of which is the
portal cavernoma. Chronic PVT has been used to
designate the latter condition.

35. Disorders of extrahepatic portal vein
A) Portal vein thrombosis:
 Causes:
- May be due to local factors (e.g. infiltration by tumour or
trauma) and/or underlying prothrombotic conditions.
 Clinical picture:
- Features of the underlying cause.

36. Disorders of extrahepatic portal vein
A) Portal vein thrombosis:
 Clinical picture:
- Acute PVT:
 Acute abdominal pain in 90% of cases.
 SIRS is present in 85% of patients even in absence of an
inflammatory focus.
 Prolonged total obstruction leads to intestinal perforation,
peritonitis, shock and death.

37. Disorders of extrahepatic portal vein
A) Portal vein thrombosis:
 Clinical picture:
- Chronic PVT:
 There is manifestations of portal hypertension with bleeding varices
is the most common presentation.
 Portal cholangiopathy, a condition characterized by compression
and deformation of intra- and extrahepatic bile ducts by the
collateral veins constituting the cavernoma.

38. Disorders of extrahepatic portal vein
A) Portal vein thrombosis:
 Diagnosis:
1. Abdominal US, CT and MRI can show thrombus.
2. Doppler US show flow in collaterals with no PV signal.
3. EUS can detect small non-occluding thrombi.
4. Angiography.

39. Abdominal US reveal PVT

40. Contrast-enhanced CT demonstrates lack of enhancement in the portal vein

41. Cavernous transformation of the portal vein in a patient with chronic PVT

42. Disorders of extrahepatic portal vein
A) Portal vein thrombosis:
 Treatment:
- Treatment of underlying cause.
- Treatment of acute PVT:
 Anticoagulants should be given to all patients.
 Continue on long term anticoagulation in patients with permanent
thrombotic risk factors.

43. Disorders of extrahepatic portal vein
A) Portal vein thrombosis:
 Treatment:
- Treatment of chronic PVT:
 Screening for varices with treatment of active haemorrhage.
 Long term anticoagulation in patients without cirrhosis and with a
permanent thrombotic risk factors.
 In children consider portosystemic shunting.

44. Disorders of extrahepatic portal vein
B) Congenital portohepatic shunts:
- They represent persistence of the omphalomesenteric
venous system.
- These shunts divert portal blood away from the liver.
- Clinically there is portosystemic encephalopathy.
- They are diagnosed by ultrasound, CT, MRI and colour
Doppler imaging and confirmed by angiography.

45. Disorders of extrahepatic portal vein
C) Portal vein aneurysm:
- This abnormality is mostly encountered in patients with
portal hypertension and therefore represents a secondary
alteration.

46. PV aneurysm

47. Disorders of intrahepatic portal venous system
A) Obliterative portal venopathy:
- Obliteration of small portal veins is usually associated with
portal fibrosis, nodular regenerative changes, and sinusoidal
dilatation or fibrosis.
 Causes:
 Schistosomiasis.
 Chronic exposure to chemicals (arsenic, copper, vinyl chloride,
thorium dioxide).

48. Disorders of intrahepatic portal venous system
A) Obliterative portal venopathy:
 Causes:
 Cytotoxic drugs (azathioprine, 6-MP, methotrexate).
 Underlying prothrombotic conditions.
 Congenital hepatic fibrosis.
 Sarcoidosis.
 Congenital diseases as Turner’s syndrome.
 Idiopathic portal hypertension (hepatoportal sclerosis).

49. Disorders of intrahepatic portal venous system
A) Obliterative portal venopathy:
 Clinical picture:
- Manifestations of the underlying cause.
- Manifestations of portal hypertension.
 Treatment:
- Treatment of the cause.
- Treatment of portal hypertension, bleeding varices.

50. Disorders of intrahepatic portal venous system
B) Portohepatic fistulae:
- This rare entity is almost exclusively encountered in patients
with hereditary hemorrhagic telangiectasia.

51. Disorders of Sinusoids
A) Sinusoidal obstruction syndrome:
- Also known as hepatic veno-occlusive disease (VOD).
- SOS is related to toxic injury to sinusoidal endothelium.
- It is almost exclusively encountered in:
 After exposure to certain plants containing pyrrolizidine
alkaloids (chronic form). The alkaloids are ingested in the
form of herbal teas, hence the term Jamaican bush tea
disease. or

52. Disorders of Sinusoids
A) Sinusoidal obstruction syndrome:
 After BMT or haematopoietic stem cell transplantation due
to exposure to high dose cytoreductive therapy, with or
without hepatic irradiation. SOS also associated with several
chemotherapeutic agents used in treatment of solid cancer,
or immunosuppressors used after organ transplantation
(acute form).

53. Major causes of
acute form of SOS

54. Disorders of Sinusoids
A) Sinusoidal obstruction syndrome:
 Clinical picture:
A) SOS following BMT has been defined as the occurrence of
two or more of the following characteristics appearing within
20 days after transplantation (Seattle criteria):
 Painful hepatomegaly.

55. Disorders of Sinusoids
A) Sinusoidal obstruction syndrome:
 Clinical picture:
 Sudden weight gain of more than 2% of baseline body weight.
 Total serum bilirubin level greater than 2.0 mg/dL.
B) Chronic form of SOS have features similar to those of
hepatic vein occlusion and include tender hepatomegaly,
abdominal pain, ascites, and fatigue.

56. Disorders of Sinusoids
A) Sinusoidal obstruction syndrome:
 Diagnosis:
- Mainly clinical.
- Transjugular liver biopsy may be helpful.
 Treatment:
- Largely supportive with therapy of fluid retention, sepsis,
and organ failure.

57. Disorders of Sinusoids
A) Sinusoidal obstruction syndrome:
 Treatment:
- Defibrotide, may be of benefit in prophylaxis or treating
severe SOS, with complete remission in 30-60%.
- TIPS, portosystemic shunting and liver transplantation have
been used in individual cases.

59. Disorders of Sinusoids
B) Sinusoidal dilatation and peliosis hepatis:
- These are rare vascular conditions characterized by a
proliferation of the sinusoidal hepatic capillaries that results
in cystic blood-filled cavities distributed randomly
throughout the liver.

60. Disorders of Sinusoids
B) Sinusoidal dilatation and peliosis hepatis:
- Peliosis is characterized by destruction of the sinusoidal
wall, while in pure sinusoidal dilatation the endothelium
appears to be preserved.
 Causes:

61. Conditions associated
with sinusoidal dilatation
and peliosis hepatis

63. Disorders of Sinusoids
B) Sinusoidal dilatation and peliosis hepatis:
 Clinical picture:
- The condition usually asymptomatic, but when severe, it
manifest as jaundice, hepatomegaly, LCF,
haemoperitoneum.
 Diagnosis of peliosis hepatis:
- Diagnosis can established by CT, or MRI.

64. Disorders of Sinusoids
B) Sinusoidal dilatation and peliosis hepatis:
 Treatment:
- Treatment of the underlying cause.
- Rarely partial resection of the liver or transplantation may
be required.

65. CT picture of peliosis hepatis

66. MRI picture of peliosis hepatis

67. Disorders of Sinusoids
C) Sinusoidal fibrosis:
- When located in the perivenous area of the lobule, this
lesion is associated frequently with alcoholic or non
alcoholic steatohepatitis, or with longstanding hepatic
venous outflow block or heart failure.
- When randomly distributed, it can accompany obliterative
portal venopathy or sinusoidal dilatation.

68. Disorders of Sinusoids
C) Sinusoidal fibrosis:
- In rare patients, sinusoidal fibrosis is an isolated finding. A
typical cause for isolated sinusoidal fibrosis is chronic
vitamin A supplementation.