2. DR. TANVEER HUSSAIN FCPS(Medicine) FCPS (Gastroenterology)
BUDD CHIARI SYNDROME
DR. TANVEER HUSSAIN
FCPS(Medicine)
FCPS (Gastroenterology)

3. HISTORY Lambron in 1842 : First case report. George Budd (1845)
Internist, kings College.
Classic Triad of
Abdominal pain
Hepatomegaly
Ascites
Hans Chiari (1895)
Pathologist
Histopathology features. Obliterative endophlebitis of hepatic veins

4. HISTORY Okuda et al, hepatology , 1998 Rokitansikys disease.
Synonymous:
Hepatic venous outflow obstruction.
Obliterative hepatocavopathy.
Okuda et al, hepatology , 1998
Rokitansikys disease.
Von Rokitansikys disease.
Membranous obstruction of IVC.
Hepatic vena cava disease.
Budd Chiari syndrome with occlusion of hepatic vein or
Hepatic vein thrombosis
Shin N et al.BCS review
World J Hepatol 2016 June

5. DEFINITION Budd Chiari Syndrome. Hepatic Venous Outflow obstruction at
Hepatic venules.
Large hepatic veins.
IVC.
Right atrium.
Sinusoidal obstruction syndrome is excluded from this definition.
Shin N et al.BCS review
Worlds J Hepatol 2016 june.
European group for the study of hepatic vascular diseases

6. ETIOLOGICAL CLASSIFICATION
ORIGIN
EXAMPLES
PRIMARY
Endoluminal lesions
Thrombosis.
Webs.
Endophlebitis.
SECONDARY
Outside venous system.
Invasion or Extrinsic compression.
Tumor.
Abscess.
Cysts.
Rossle M et al, Surgery 2004
Okuda K et al, J Gastroenterol Hepatol 2002.
Janssen HL et al, Hepatol 2003.

7. CLASSIFICATION -VESSEL INVOLVEMENT
TYPE I
TYPE II
TYPE III
IVC with or without Hepatic veins occlusion.
Major Hepatic veins
Small Centrilobular Veins.

8. Categories: based on rapidity of onset and collateralization
Acute +/- fulminant:
20%, F>M, Severe RUQ pain,
Rapid development of jaundice, ascites,
AST/ALT > 5x ULN; ↑↑ bili; +/- encephalopathy
Sub-acute:
40%; variable presentation; typical is partial occlusion.
asymptomatic to mildly symptomatic.
Chronic:
40%; seen as cirrhosis

9. So then, what are the causes?
FIRST THINK
Hypercoagulable
myeloproliferative diseases
Multiple causes in one person
often see + JAK2 mutation

10. DEMOGRAPHIC PROFILE FEATURES WEST (Classical) EAST (HVC-BCS)
OBSTRUCTION HV
(classical BCS)
IVC
SEX
Females
Males
OBSTRUCTION OF IVC.
Thrombosis.
Membranous in Japan, Nepal.
IVC thrombosis in India.
PRESENTATION
Acute (60-85%)
Chronic (75-80%)
Association
OCP and MPD
Pregnancy and Infection

11. CLINICAL PRESENTATION
Fulminant
Rare(7%)
Acute
Sub acute
Most common
Chronic
Onset
< 2 months
Up to 6 months.
6 months- years
Months to years CF HE
Sudden tense ascites, abdominal pain hepatomegaly.
No collaterals.
Resistant ascites
Hepatic and portal collaterals
Features of CLD.
Transaminase levels.
> five times
Mild elevation.
Histopathology
No cirrhosis
Little necrosis
Severe fibrosis or
Cirrhosis

12. Signs and Symptoms Abdominal Pain Hepatomegaly Ascites Jaundice
Venous collateralization – varices, abdominal/flank collaterals
Absence of hepatojugular reflux
Laboratory evidence of liver dysfunction

13. Normal R hepatic vein
Normal liver
Centrilobular necrosis, dilated sinusoids filled with RBCs
No R hepatic v. Rich collaterals ‘Spiders web’

14. HOW TO DIAGNOSE?

15. DIAGNOSIS Clinical suspicion. LFTs Imaging
Infra and supra hepatic caval pressure.
Hypercoagulable profile.
Bone Marrow.
Liver biopsy.

16. CLINICAL SUSPICION Classic Triad present simultaneously.
FHF with hepatomegaly and ascites.
Liver disease in patients with prothrombotic disorders.
Patients with CLD, intractable ascites with mildly derranged LFTs.
Few cases of idiopathic CLD ..
KV menon, NEJM 2004

17. IMAGING Role of imaging Site of obstruction. Patency of HV , IVC, PV.
Details of vascular anatomy.
Status of liver.
Caudate lobe
Collaterals.
Differentiates forms of BCS.
Brancatelli G et al, AmJ Roentgenol 2007

18. IMAGING Doppler ultrasound (first step). CT scan MRI
Hepatic Venography.

19. LIVER ULTRASOUND WITH DOPPLER STUDIES
Normal right hepatic vein
Doppler study showing normal expected biphasic waveform across hepatic vein
No hepatic vein, no venous flow

20. CHARACTERISTIC FEATURES ON VENOGRAPHY AND CT
Caudate Hypertrophy and IVC Compression
Mottled parenchyma / lack of perfusion
Retroperitoneal Varices

22. MANAGEMENT
Underlying disorder can be found in 70% cases and multiple disorders can be present in 25% cases.
Early relief of obstruction may reverse parenchymal abnormalities and improve survival.
Remove the cause.
Prevention of thrombosis extension.
Relieve high pressure and congestion in liver.
Management of massive ascites.

23. TREATMENT: Longterm/indefinite anticoagulation and. . .

24. MEDICAL MANAGEMENT Remove cause of venous thrombosis.
Relieve high pressure and congestion with in liver.
Prevent extension of thrombosis.
Reverse massive ascites.

25. DECISION MAKING IN SHUNT SX
Documentation of ongoing necrosis.
Demonstration of intact lobular architecture
Status of infra hepatic IVC.
Status of PV.
Status of caudate lobe.
Damaged liver with no lobular collapse.
shunt sx.
Severe damaged liver with lobular collapse and fibrosis .
LT.

26. BCS– SURGICAL CONCLUSION
BCS due to HV occlusion, SSPCS is most effective therapy.
BCS due to IVC occlusion , combined PCS and CAS is very effective.
LT for BCS with progressive liver failure or in failed PCS.
SSPCS and LT are not competing forms of treatment.
Early and middle stages of BCS: SSPCS.
Fibrosis or Cirrhosis: LT

27. PROGNOSIS

28. THANK YOU