Hepatic vein thrombosis

1. Budd Chiari Syndrome
Dr Asif Raza Zaidi
MBBS FCPS
Gastroenterology & Hepatology

2. Road map for today
• Introdution
• Epidemiology
• Etiology
• Pathogenesis
• Clinical presentation
• Diagnosis
• Management

3. INTRODUCTION
• Obstruction of hepatic venous outflow anywhere from small
hepatic veins to the supra hepatic inferior vena cava
• Obstruction due to cardiac or pericardial disease and sinusoidal
obstruction syndrome is not included in BCS
Journal of the College of Physicians and Surgeons Pakistan 2017,
Vol. 27 (5): 301-304

4. INTRODUCTION
• First described by British physician, William Budd, in 1845.
• The description of the clinical features of hepatic vein outflow
obstruction is generally attributed to a pathologist, Hans Chiari.
• In 1899, Chiari described an "obliterating endophlebitis of the
hepatic veins" and its association with hepatomegaly, ascites and
abdominal pain.
Expert Rev Gastroenterol Hepatol. 2012;6(6):731-744

5. EPIDEMIOLOGY
• It is a rare disorder
• Incidence varies in different parts of the world
• US --------- 1 in 2.5 million person/year
• Japan ------- 0.13 cases/million/year
• Nepal -------10 times more common
• 17% of all hospital admission due to liver disease

6. EPIDEMIOLOGY

7. ETIOLOGY
• Primary BCS
• Underlying thrombophilic disorder
• Secondary BCS
• Having hepatic venous compression or invasion from an external source,
such as neoplasm
• In a study from 2009, one risk factor was found in 84% of patients
and two or more risk factors in 48%
• A local risk factor (e.g., venous anomaly) was found in only 5% of
patients
Budd-Chiari syndrome as an initial presentation of hepatocellular carcinoma: a case report. Med Ultrason 2014
Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med 2009

8. ETIOLOGY

9. ETIOLOGY

10. ETIOLOGY

11. PATHOGENESIS

12. CLINICAL PRESENTATION
• Fulminant BCS
• 5%
• Develop within a few days
• Acute liver failure
• Increase ALT, AST, Bilirubin
• Coagulopathy, encephalopathy, ascites, AKI, enlarged liver
• Needs urgent liver transplantation
• 15% of patients with BCS may be asymptomatic
Intractable & Rare Diseases Research. 2015; 4(1):24-32

13. CLINICAL PRESENTATION
• Acute BCS
• 20%
• Within one month
• Intractable ascites
• Abdominal pain
• Liver enlargement
• Renal failure
• Subacute and chronic BCS
• 60%
• Most common form in prothrombotic patients
• Portal hypertension
Intractable & Rare Diseases Research. 2015; 4(1):24-32

14. CLINICAL PRESENTATION
EASL Clinical Practice Guidelines: Vascular diseases of the liver. J Hepatol (2015)
Clinical feature Percentage
Ascites 83%
Hepatomegaly 67%
Abdominal pain 61%
Esophageal varices 58%
GI bleeding 5% HepatomegalyAscites
Abdominal pain

15. DIAGNOSIS
• Doppler USG abdomen
• CT abdomen
• MRI abdomen
• Venography
• Liver biopsy
• Investigation of underlying conditions
• Prognostic markers

16. DIAGNOSIS
• Doppler USG and MRI are the gold standard for diagnosis
• Contrast enhanced triphasic CT can be used when MRI is not
available
• Direct signs:
• blockage or compression of hepatic veins/IVC
• Venous collaterals
• Indirect signs:
• Morphological changes in the liver
• Caudate lobe hypertrophy
• Delayed nodule formation

17. DOPPLER USG ABDOMEN
• Initial investigation of choice
• Sensitivity 87.5%
Diagnosis of Budd–Chiari syndrome by pulsed Doppler ultrasound. Gastroenterology (1991)

18. DOPPLER USG ABDOMEN

19. CT SCAN ABDOMEN

20. CT SCAN ABDOMEN
Computed tomography showing
ascites, splenomegaly and typical
patchy reduced perfusion of the
liver.Theportalvein ispatent.

21. MRI ABDOMEN

22. Liver biopsy
• High-grade venous congestion and
centrilobular liver cell atrophy
• Possibly,thrombi within the terminal
hepatic venules.
• The extent of fibrosis can be
determined based on biopsy
findings.
• The most severe findings can include
fulminant hepatic failure with
massive centrilobular necrosis ...

23. MANAGEMENT
• Remove the cause
• Prevent the thrombus extension
• Relieve the high pressure and congestion of liver
• Prevent and treat complications of portal hypertension
• Ascites
• GI bleeding
• HCC

24. TREATMENT
• Medical therapy
• Thrombolytic therapy
• Angioplasty/stenting
• TIPS
• Surgical shunts
• Liver transplantation

25. WHICH ONE IS BETTER????????????
No randomized control trials

26. MEDICAL THERAPY
• Anticoagulation:
• Treat all BCS patients with anticoagulation, in the absence of major
contraindications
• LMWH followed by warfarin
• Bleeding complications occured in up to 17% of patients
• MPD:
• Aspirin
• Hydroxyurea
• PNH:
• Eculizumab
EASL Clinical Practice Guidelines: Vascular diseases of the
liver. J Hepatol (2015)

27. THROMBOLYTIC THERAPY
• Catheter directed thrombolytic therapy may be effective in acute BCS
• Delivered just proximal to or within the thrombus
• Overall success rate is low
• Risk of bleeding is high
Sharma et al, J Hepatol 2004

28. ANGIOPLASTY AND STENTING
• Good results are seen in patients with short length stenosis
• Treatment option for acute and subacute BCS
• First step change when the medical treatment fails
• May be complicated by
• Bleeding
• Renal failure
• Allergic reaction to dye
• Restenosis
• Zhang et al. found that the hepatic venous stent patency rate was
90.9% after 15-78 months of follow-up
• Another study showed 1-year and 5-year survival of 94% and 87%
respectively
Interventional Radiology Advance Publication 2017
Gut 2006;55:878–884

29. TRANSJUGULAR INTRAHEPATIC PORTOSYSTEMIC
SHUNT TIPS
• TIPS is a percutaneous image-guided procedure that creates a portocaval
shunt that passes through the liver parenchyma
• When angioplasty fails or not feasible
• Or patient is not improved with medical therapy
• Contraindications:
• Primary prevention of variceal bleeding
• CCF
• Severe tricuspid regurgitation
• Sepsis
• Severe pulmonary hypertension
• Hepatic encephalopathy

30. TRANSJUGULAR INTRAHEPATIC PORTOSYSTEMIC
SHUNT TIPS
• Complications:
• TIPS dysfunction
• Transcapsular
puncture
• Fistulae

31. TREATMENT
• Both shunting and transplantation can result in a 5-year survival rate of at
least 75%
• Surgical procedures are rarely done due to high mortality and advanced
radiological procedures
• Liver transplantation is an option if patients don’t improve after medical
and radiological interventions
• Patients should be followed up regularly and post transplant
anticoagulation is needed
ANNALS OF SURGERY Vol. 233, No. 4, 522–527

32. United European Gastroenterology Journal 2015

33. OBJECTIVE:
To evaluate the outcomes of hepatic
venoplasty in the management of Budd
Chiarisyndrome.
STUDYDESIGN:
Case series
PLACE AND DURATION OF STUDY:
Gastroenterology Department, Shaikh
Zayed Hospital Lahore, from January
2017toDecember2017.
SINGLE CENTRE EXPERIENCE OFOUTCOMES OFHEPATIC VENOPLASTY INTHE
MANAGEMENT OFBUDD–CHIARI SYNDROME: ACASE SERIES
KASHIF MALIK,KHURAMSHAFIQ KHAN, SADIAJABBAR, MUHAMMAD USMANNAEEM
DEPARTMENT OF GASTROENTEROLOGY SHAIKH ZAYEDHOSPITALLAHORE
METHODOLOGY:
Patients of acute, subacute or chronic
Budd Chiari syndrome, who were
managed by hepatic Balloon angioplasty
with or without stenting were included
in study. Baseline characteristicsand
etiologicalfactorsnoted.Prevenoplasty
Child Pugh class, amount of ascites and
sizeof
varices noted. Patients followed up for
6months after venoplasty. The primary
outcome of the study was defined as
patency of the hepatic veins 6 months
post venoplasty. The secondary
outcomeswere toobserveimprovement
in ascites and size of esophageal varices.
The statistical analysis done using SPSS
version20.
RESULTS:
Out of 6 patients, 4 (66.7%) were males
and 2 (33.3%) were females. The age of
patientsranges
from 18 to 32 years with a mean of 22.8
± 4.9 years. Amongst the etiological
factors, 2(33.3%) patientshadIdiopathic
BCS, 2 (33.3%) had JAK II mutation, 1
(16.7%)hadProteinCandS
CONCLUSION:
Hepatic venoplasty is an effective
treatment option in carefully selected
patientsbefore
undergoing more invasive procedures
andlivertransplantation.
KEYWORDS:
Budd Chiari syndrome, Hepatic
venoplasty
4
2
GENDER
MALES FEMALES
Etiology Frequency Percentag
e
Protein C
and S
deficiency
01 16.7
JAK II
mutation
02 33.3
Idiopathic 02 33.3
Other 01 16.7
deficiency and 1 (16.7%) had
Ulcerative colitis. 4 (66.7%) were in
Child Pugh Class A and 2 (33.3%) were
in Child Pugh Class B. On
ultrasoundand CT scan abdomen, all 6
patients had obstruction of all hepatic
veins (RHV, MHV, LHV) and 2 (33.3%)
patients also had IVC involvement. 4
(66.7%) patients had smooth
echotexture of liver. Spleen size
ranges from 14 to 18.4 cm with a
meansizeof16.7±1.94cm.
Portal vein diameter ranges from 9 to
13 mm with a mean diameter of 10.8
± 1.6 cm. Hepatic venoplasty
performed via transhepatic approach
inall6patients.
Child Pugh class A B C
Frequency 2 4 ZER0
Vessel
patency
after 6
month
follow up
Frequency Percentage
Yes 5 83.3
No 1 16.7
Balloon dilatation done in all patients
and self expendable metal stent was
placed in 5 (83.3%) of patients.
Venoplasty was successful in terms of
primary outcome in 5 (83.3%) of
patients. These patients had
improvement in ascites and size of
esophageal varices after 6 months of
followup.
1 (16.7%) patient had her stent blocked
andreferredforlivertransplantation.
BACKGROUND:
BCSisanuncommondisorder,definedas
hepatic venous outflow obstruction
anywhere from small hepatic veins to
suprahepatic inferior vena cava. It is
divided into primary and secondary BCS
according to etiological factors. It is
diagnosed by Doppler USG and MRI.
There is lack of randomized control trials
as regards treatment of BCS is
concerned. Hepatic venoplasty is one of
the well established treatment modality
usedinpatientswithacuteandsubacute
BCS.

34. TAKE HOME MESSAGE
• Budd chiari syndrome should be suspected in any acute or chronic
liver disease
• Early diagnosis and treatment is key to good survival
• Underlying cause should always be looked for and treated