Clinical manifestations, diagnosis, and treatment of the Budd-Chiari syndrome

thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava

237 patients( in US, the Netherlands, and France) between 1984 and 2001:

• The median age was 35 (range 13 to 76)
• 67 % were female
• An overt myeloproliferative disorder 23%(the majority of whom had polycythemia vera)
•  ascites (84 %) and hepatomegaly (76 %); 11 patients (5%) were asymptomatic
•  hepatic veins (62 %) inferior vena cava (7 %) or both (31 %); 34 patients (14 %) had associated portal vein thrombosis

• Acute – 20 % (five with fulminant hepatic failure), pregnancy , present with severe right upper quadrant pain,jaundice, ascites, variceal bleed, enceph
•  high levels of ALT (≥5 times the upper limit of normal) reflected acute, severe but potentially reversible ischemic injury, a slow decline in ALT levels (<50 percent of starting concentration within three days) predicted a poor outcome
• Subacute < six months and no evidence of liver cirrhosis) – 40%
• Chronic (> six months with evidence of portal hypertension and cirrhosis) – 40%, may be associated with hypertrophy of the caudate lobe of the liver, compressing inferior vena cava
• Jaundice is rare

  ultrasonography with Doppler studies, CT or MRA (MRI angiography).

 The portal and splenic circulation should also be evaluated. Occlusion of the portal vein in patients with Budd-Chiari syndrome limits therapeutic options and has a poor prognosis

 Splenomegaly is uncommon, 30%, and venous collaterals may be seen on the anterior abdominal wall,  on the back suggest occlusion of the vena cava. Signs of RHF suggest an underlying cardiac cause of fluid accumulation

SAAG >1.1

Venography — The gold standard , which is performed by accessing the hepatic venous circulation percutaneously, either via the internal jugular vein, cephalic vein, or femoral vein.

 Arteriography —  when planning a surgical approach to decompress the congested liver


  
Liver biopsy —AASLD

• There is confusion regarding the diagnosis (an uncommon situation given the multitude of available imaging tests).
• In patients with subacute presentations- if show significant fibrosis/cirrhosis may provide an impetus for transplant evaluation. if centrizonal congestion may benefit from shunt surgery.

The goals of therapy are to:

• Prevent the propagation of the clot
• Restore patency of thrombosed veins
• Decompress the congested liver
• Address and prevent complications related to fluid retention, malnutrition, and portal hypertension

Medical- AASLD
- treat fluid retention like in cirrosis, compression stocking, shunt
- improve nutrition status
-treat underlying condition-hypercoagulability- warfarin; myeloproliferative- aspirin + hydroxyurea

 237 patients treated at four centers 1984- 2001 72 % received anticoagulation.
worse prognosis included the presence of encephalopathy, ascites, an INR >2.3, and higher bilirubin concentrations
a benefit of anticoagulation on survival (RR 0.14, 95% CI 0.02 to 1.21) could only be demonstrated in patients with class I disease (ie, less severe disease).

Addition -prognosis was worse in patients who presented with clinical features suggesting chronic disease.

Recommendation:  chronic or subacute Budd-Chiari syndrome with well compensated liver disease at the time of presentation. However, additional measures to decompress the liver should be considered

Surgical portosystemic shunting had no apparent benefit on survival.

radiologic procedures (such as angioplasty, transjugular intrahepatic portosystemic shunt [TIPS] placement, and stenting) and surgical intervention (including shunting procedures and liver transplantation).
TIPS- used for patients who do not respond to dilation of a hepatic venous outflow stricture or in whom a dilatable lesion cannot be found. is
- temporizing measure prior to liver transplantation in patients with acute, fulminant Budd-Chiari syndrome.
-Placement of TIPS in these patients, if not performed properly, can result in complication such as portal vein thrombosis

Monitor closely for disease progression LFT. OGDS looking for varices and liver biopsies annually, tapering 2-3years depending upon the overall health status of the patient. Bleeding complications appear to be common in patients maintained on anticoagulation,

Thrombolytic therapy — Systemic and locally administered - no study,  the bleeding risk is similar.
Not for chronic, but for subacute and acute.
use in: clot well defined in venography, recent, no CI
not in: who have extensive clot involving the intrahepatic vena cava and hepatic veins or a clot of unknown age.


Liver transplantation —for who are not candidates for radiologic or surgical decompression or who have decompensated cirrhosis. Patients who developed the Budd-Chiari syndrome as a result of protein S, protein C, or antithrombin III deficiency may also be cured of their clotting tendency by liver transplantation, since the transplanted liver produces normal amounts of these enzymes.

AASLD GUIDELINE 2009

• Correct  risk factor(s)
• Initiate anticoagulation therapy immediately. Use LMWH targeting anti-Xa activity to 0.5-0.8 int. unit/mL. Change to oral anticoagulation agent INR 2-3
• Maintain permanent anticoagulation therapy unless a major contraindication is present or complication occurs.
• Treat complications of portal hypertension as recommended for other types of liver disease.
• Check for a venous obstruction amenable to percutaneous angioplasty/stenting in all symptomatic patients and treat accordingly.
• In patients without ongoing improvement on anticoagulation therapy (with or without angioplasty), consider transjugular intrahepatic portosystemic shunt (TIPS).
• Consider liver transplantation if TIPS insertion fails or does not improve the patient's condition and in those with fulminant hepatic failure.
• Coordinate care with a transplant center.
• Monitor patients with longstanding, well controlled Budd-Chiari syndrome for the late development of hepatocellular cancer and transformation of underlying myeloproliferative disease