BUDD-CHIARI SYNDROME Nader Saad PGY2 Vascular

DEFINITION

•BCS is characterized by hepatic venous outflow obstruction at any level from the small
hepatic veins to the atrio-caval junction, regardless of the cause of obstruction.

•These articles review discusses the presentation, management and prognosis of BCS
with particular attention focused on the interventional and surgical aspects of treatment.
PATHOPHYSIOLOGY

•Obstruction is usually caused by a thrombus, but may result from extrinsic

compression (tumor, abscess, cysts), membranous webs within the inferior vena cava
(IVC) or postoperative complications following liver transplantation.

•Post-hepatic obstruction leads to increased sinusoidal pressure, sinusoidal

congestion, hepatomegaly, hepatic pain, portal hypertension and ascites.

•Without venous outflow, the elevated sinusoidal pressure leads to perisinusoidal

necrosis of hepatocytes and eventually to liver failure.
PATHOPHYSIOL
OGY
CLASSICFICATION
1. Primary BCS :
 Originates from within the lumen of the veins or venules.
 Results from thrombus, webs or endophlebitis.

2. Secondary BCS :
 Results from an extra-luminal lesion such as tumor, abscess or cyst, which can
invade the lumen or cause extrinsic compression of the hepatic venous outflow tract.
PRESENTATION

The majority of patients with BCS present with a triad:

1. Hepatomegaly
2. Right upper quadrant pain
3. Abdominal ascites

Lower extremity edema is another common finding on physical exam.

Young children (10 years of age) account for 1–7% of all cases of BCS. This group often
presents late in the course of disease, by which time irreversible pathology may be present .
PRESENTATION

•The most common underlying disorders in patients with BCS are myeloproliferative disorders
(53%).

•Other common etiologies: Factor V Leiden (25%) and Factor II gene mutation (5%).

•Common other associations: Oral contraceptive use, protein C and S deficiencies,

Antiphospholipid syndrome, Antithrombin III deficiency, PNH, Cancer, Bechet’s syndrome and
Trauma.

•Twenty percent of cases of BCS are idiopathic.

CLINICAL PRESENTATION
•The clinical presentation is driven by:
 The extent and swiftness of hepatic outflow obstruction.
 The body’s ability to decompress the liver via development of collateral blood flow.

•Patients can be divided into the following categories:

Fulminant, Acute, Subacute, or Chronic
The subacute form is the most common presentation and is characterized by slow
accumulation of ascites. Hepatocellular damage is minimal due to the development of
hepatic and portal venous collaterals.
DIAGNOSTIC EVALUATION

•Radiographic analysis should attempt to determine the patency of the hepatic veins, IVC and portal vein.

•Doppler ultrasound:
Often the first study obtained.
Has a sensitivity and specificity as high as 85%.

•CT scans:
Allow for the evaluation of ascites, hepatic vein patency, vena cava patency and caudate lobe hypertrophy.

•MRI:
May differentiate chronic from acute disease
Provide further delineation of vascular anatomy.
DIAGNOSTIC EVALUATION
•Other investigations that may aid in management include:
Infra-hepatic and supra-hepatic caval pressures.
Hepatic venography and liver biopsy.

•Underlying hypercoagulable states should be investigated in all patients.

MEDICAL MANAGEMENT

1. Anticoagulation

2. Sodium restriction

3. Diuretic therapy

4. Paracentesis
MANAGEMENT
STRATEGIES

Treatment of BCS generally follows

a least invasive to most invasive
strategy.
INTERVENTIONAL TECHNIQUES

1. TIPS:
Has become the first option when a shunting procedure is indicated.

2. Thrombolytic therapy:
A 10 patient case series concluded that systemic thrombolytic therapy is of little value and that catheter-directed thrombolysis is
more efficacious for acute thrombus that is not completely occlusive.

3. Angioplasty:
Often suffers from high re-occlusion rates  placement of stents in the IVC or hepatic veins has been recommended.
A reported case series of 115 in which stents were placed in the IVC and hepatic veins showed success rates of 94% and 87%
respectively. Patency reached 90% after a mean follow-up of more than 45 months.

Unfortunately, most patients do not present acutely and their disease is often not amenable to thrombolytics, angioplasty or
stenting.
TRANS-JUGULAR
INTRAHEPATIC
PORTOSYSTEMIC SHUNT
TIPS has become an attractive option in the elective and emergent situations
since:

1. It effectively decompresses the portal system and serves as a bridge to transplant.

2. It may be employed in the acute or chronic setting (For the patients who present
weeks to months after formation of hepatic vein thrombus)
3. Most institutions are more likely to possess the capability to perform TIPS than to
employ a surgeon with the expertise to perform a mesenteric-systemic shunt
TRANS-JUGULAR
INTRAHEPATIC
PORTOSYSTEMIC
SHUNT
SURGICAL SHUNTS

•The role of surgical shunts in the treatment of BCS is a controversial topic.

•A wide range of perioperative mortality has been reported (0–50%). However, long-
term survival rates (5–14 years) after various shunting procedures have been
reported to be as high as 90%.
SURGICAL SHUNTS
Limitations:

Studies that compare surgical shunts to medical or minimally invasive techniques are
often difficult to interpret because they are usually:
1. Retrospective.
2. May have significant time bias.
3. The sickest patients are usually managed non- operatively.
SURGICAL SHUNTS
Indications:

•Extensive IVC thrombus may make TIPS difficult and favor placement of a surgical
shunt.
•For patients without IVC obstruction  Portocaval or Meso-caval shunts are
reasonable options.
•IVC obstruction and/or an infra- hepatic to right atrium pressure gradient of
>20mmHg  Meso-caval and Portocaval shunts may not effectively decompress the
liver  Many authors consider Meso-atrial or Cavo-atrial shunts in these cases.
From 1972 to 1999, the authors conducted prospective studies of the treatment of 60
patients with BCS who were divided into three groups:

1. Occlusion confined to the hepatic veins treated by Direct side-to-side Portacaval

shunt (SSPCS) N=32
2. Occlusion involving the inferior vena cava (IVC) treated by a Portal
decompressive procedure that bypassed the obstructed IVC N=18
3. Advanced cirrhosis and hepatic decompensation, required Orthotopic liver
transplantation. N=10
RESULTS OF SSPCS FOR HEPATIC VEIN
OCCLUSION

•One surgical death (3%).

•The other 30 patients were alive at the time of writing for an overall survival rate of 94%.

•None of the patients had portosystemic encephalopathy.

•Hepatosplenomegaly disappeared in all patients.

•Angiography demonstrated patency of the SSPCS and IVC, and pressure measurements showed
a wide-open anastomosis.
RESULTS OF SSPCS FOR
HEPATIC VEIN OCCLUSION
Two patients with polycythemia rubra vera developed thrombosis of the SSPCS
1 week and 3 months after surgery

Both patients underwent immediate surgery, the shunt was taken down, fresh
thrombus was removed from the portal vein, and the portacaval anastomosis was
reconstructed.
Subsequently, both patients received anticoagulant therapy with warfarin.
One patient remained well for 19 years since revision of the shunt. The other patient
developed recurrent shunt thrombosis and required liver transplantation; he was well
9.5 years after transplantation.
DIRECT SIDE-TO-
SIDE PORTACAVAL
SHUNT
RESULTS OF PORTAL DECOMPRESSION
FOR IVC OCCLUSION

Meso-atrial Shunt
•Meso-atrial shunt was performed in eight patients with BCS caused by thrombosis of both the IVC and
hepatic veins.
• Five of the eight (63%) subsequently developed thrombosis of the graft and died of liver failure.
•Three of the deaths occurred during the first postoperative year, one occurred in the second year, and one
occurred in the fifth year.
•The three long-term survivors of meso-atrial shunt (38%) were followed up for 19, 17, and 17 years.
Angiography demonstrated patency of their grafts. They were free of ascites, had normal liver function,
and did not require diuretics.
•The high thrombosis rate of the meso-atrial shunt may be due to the fact that it is a relatively low-flow
synthetic graft placed in the venous system.
•It does not decompress the obstructed IVC.
MESOATRIAL
SHUNT
COMBINED SSPCS AND CAS

•To overcome these shortcomings, we worked in the experimental laboratory to devise a surgical
procedure for this form of BCS consisting of combined SSPCS and CAS through a Gor-Tex.
•Combined shunt was aimed at decompressing the IVC and shunting the entire systemic venous flow
from the lower two thirds of the body, as well as the entire portal venous flow, through the graft.
•We treated 10 seriously ill patients with BCS
•All 10 patients survived the combined shunt procedure and were alive as of this writing, 4 to 16
years after surgery.
•In our program, combined SSPCS and CAS has replaced meso-atrial shunt as the preferred
treatment for BCS caused by IVC obstruction.
•Doppler ultrasonography demonstrated patency and good function of both the SSPCS and CAS in
all patients. Permanent anticoagulation with warfarin and low-dose acetylsalicylic acid was used in
all patients.
COMBINED
SSPCS AND CAS
ORTHOTOPIC LIVER
TRANSPLANTATION
•Orthotopic liver transplantation (OLT) has been useful in advanced, decompensated liver disease
when it is too late to use portal decompression procedures.
•The indications for OLT that we have adhered to are:
Cirrhosis with progressive liver failure that has reached the point of permitting a reasonable
prediction that the patient will die within a year.
Failure of SSPCS, usually because of thrombosis, with persistence or recurrence of symptoms and
signs of BCS.
Unshuntable portal hypertension resulting from thrombosis of the portal vein, splenic vein, and
much of the superior mesenteric vein: rare indication that applies if patent blood vessels are available
to vascularize the liver allograft.
Acute fulminant hepatic failure(rare indication)
ORTHOTOPIC LIVER
TRANSPLANTATION
CONCLUSION
Budd–Chiari syndrome is a complex disease with a wide spectrum of etiologies and
presentations.

Medical therapy: Consists of treatment of underlying disease, Anticoagulation

and symptom control.

Minimally invasive procedures: Percutaneous catheter-directed thrombolysis,

Angioplasty, Stenting and TIPS.
CONCLUSION
Surgical shunting :
Best reserved to institutions with significant operative experience
SSPCS appears to be the most effective treatment of BCS confined to the hepatic veins.
Obstruction or occlusion of the IVC is a contra- indication to SSPCS.
When BCS involves thrombosis or occlusion of the IVC:
1. Meso-atrial shunt Short-term limited success + High incidence of thrombosis of
the synthetic bypass graft.
2. Combined shunt procedure Preferred treatment for BCS caused by IVC
occlusion.
CONCLUSION
Liver transplantation:
Treatment of choice for patients with fulminant hepatic failure or those with chronic
cirrhosis and poor synthetic function.
REFERENCES
1. A 27-Year Experience With Surgical Treatment of Budd-Chiari Syndrome
Orloff et al. - Annals of Surgery – 2000
2. Budd-Chiari syndrome: illustrated review of current management
Horton et al. - Liver International - 2008
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