Budd-Chiari Syndrome Treatment & Management

Updated: Jan 24, 2017
  • Author: Praveen K Roy, MD, AGAF; Chief Editor: BS Anand, MD  more...
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Treatment

Approach Considerations

In patients with Budd-Chiari syndrome, aggressively seek specific therapy aimed at correcting or alleviating the obstruction. Also treat the underlying conditions aggressively. [30]

Although medical therapy can be instituted for short-term, symptomatic benefit, [31] the use of such treatment alone is associated with a high 2-year mortality rate (80-85%).

Anticoagulant therapy

Anticoagulation is needed in some patients, especially those with underlying hematologic disorders as the cause of Budd-Chiari syndrome.

Prothrombin time and activated partial thromboplastin time should be monitored once anticoagulation is started and should be maintained within the therapeutic range.

Thrombolytic therapy

This therapy has been used in a few cases. Agents include streptokinase, urokinase, recombinant tissue-type plasminogen activator (rt-PA), and other modalities.

Radiologic intervention

Systemic thrombolysis can be a high-risk endeavor; local thrombolysis performed by an interventional radiologist is preferable.

Other available radiologic interventions include balloon angioplasty, as well as placement of a stent or a transjugular intrahepatic portacaval shunt (TIPS). [18, 32, 33]

In a single-center retrospective study (1996-2012), Tripathi et al reported good long-term outcomes in 67 patients with Budd-Chiari syndrome following successful transjugular intrahepatic portosystemic stent-shunt (TIPSS) using either polytertrafluoroethane (PTFE)-covered (n=40) or bare (n=27) stents. [34] At a mean follow-up of 82 months, 15% of patients experienced post-TIPSS encephalopathy; 2 patients underwent transplantion, 2 patients developed hepatocellular cancer, and 6 patients had liver-related deaths. The PTFE-covered stents had significantly better primary patency (76%) and shunt reinterventions (22%) compared to the bare stents (27% and 100%, respectively). Survival at 6 and 12 months was at 92% or above; that at 24 and 60 months was 80% or above; and 120-month survival was 72%. The investigators indicated that in symptomatic patients in whom hepatic vein patency cannot be restored, TIPSS should be considered first-line therapy. [34]

In another single center retrospective study (2008-2014) of 190 patients with Budd-Chiari syndrome who underwent endovascular procedures (hepatic vein, collateral vein or inferior vena cava [IVC] plasty with or without stenting, or TIPSS), venous recanalization and TIPPS were safe and effective: 153 patients (80.5%) experienced treatment response, with 19 patients (10.0%) requiring repeat interventions and 9 patients (4.7%) with complications. [35] Of the 190 patients, 147 had hepatic vein obstruction, 40 had IVC obstruction, and 3 had both. Thirty-eight patients underwent hepatic vein/stenting; 3, collateral vein stenting; 40, IVC plasty/stenting; 3, hepatic vein and IVC stenting; and 106, TIPSS. [35]

Tripathi et al reported similar findings for venous recanalization and TIPPS in 122 patients. [36]

Variceal treatment

Gastroscopy should be performed to help rule out the presence of esophageal and gastric varices. If present, they may be obliterated with banding or sclerotherapy. Nonselective beta blockers (eg, propranolol, nadolol) can be administered for primary prophylaxis against variceal bleeding.

Diet

A low-sodium diet is recommended for the control of ascites.

Paracentesis

Symptomatic treatment for Budd-Chiari syndrome includes diuretics and therapeutic paracentesis, when necessary, although paracentesis can be associated with catastrophic complications, such as bacterial peritonitis. Consequently, the benefits of therapeutic paracentesis must be carefully weighed against its risks.

Portal decompression

Decompression of the hepatic vasculature should be offered if portal hypertension is the cause of the symptoms. Either surgery or a transjugular intrahepatic portosystemic shunt (TIPS) procedure can be performed. [2, 6, 27, 37, 38, 39, 40]

Liver transplantation

Liver transplantation should be offered if decompensated liver cirrhosis is present. [4, 41]

In a Polish retrospective study (2000-2009), the long-term clinical outcomes (eg, patient and graft survival) following liver transplantation and anticoagulation maintenance for Budd-Chiari syndrome were good in 25 patients with myeloproliferative disease and recurrent thrombosis. [42]

Similar findings were reported in an Indian retrospective study (2011-2015) of 9 patients with Budd-Chiari syndrome and chronic liver disease who underwent living donor liver transplantation. [43] The investigators noted that prevention of recurrent thrombosis was dependent on "meticulous surgical technique, perfect and wide outflow anastomoses, and a strict anticoagulation protocol. Moreover, the use of synthetic (PTFE) graft for inferior vena cava interposition was safe, feasible, and provided good reconstruction results. [43]

Consultations

Early involvement of a hepatologist can help to establish the direction of workup and therapy. Consultation with interventional radiologists, hematologists, oncologists, gastroenterologists, and general surgeons may be required, depending on the situation. [44]

Follow-up and monitoring

Patients with lesions that are amenable to balloon dilatation or stents require follow-up catheterizations and, frequently, repeat dilatations or stent replacement. In addition, patients should have routine surveillance for hepatocellular carcinoma (HCC). [18, 45]

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Angioplasty

This procedure can help relieve obstruction caused by membranous webs. In a study of 101 patients with Budd-Chiari syndrome, Li et al concluded that the condition can be safely and effectively treated with percutaneous transhepatic balloon angioplasty (PTBA). [46] The authors reported successful PTBA (performed after hepatovenography, with or without stenting) in 92 of the study’s patients, with all of the successful procedures resulting in significant symptom improvement.

Complications included acute hepatic vein thrombosis, occurring during or after the operation (n=3); sustained intraperitoneal bleeding from the transhepatic puncture track (n=2); pulmonary embolism, which occurred during the procedure (n=1); and intrahepatic hematoma (n=1). [46] All were managed nonsurgically. Primary patency rates at 6-, 12-, and 24-month follow-up were 84%, 78%, and 76%, respectively (with several patients lost to follow-up); secondary patency rates were 95%, 92%, and 84%, respectively. Despite these satisfactory midterm patient outcomes, the authors cautioned that long-term outcomes in patients treated with PTBA for Budd-Chiari syndrome require investigation. [46]

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Diuretic Therapy

Patients with liver failure and ascites have total body sodium overload, despite typically low serum sodium concentrations. Inducing negative sodium balance can reduce the amount of ascites. Take special care when using diuretics, to avoid inducing hepatorenal syndrome or creating electrolyte and fluid disturbances through overly aggressive diuresis. Electrolyte levels should be monitored closely.

Secondary hyperaldosteronism is a part of this clinical picture, making spironolactone typically the first-line diuretic. Chlorothiazide or furosemide is often added, which can provide synergy and avoid hyperkalemia.

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