Budd-Chiari Syndrome Treatment & Management
- Author: Praveen K Roy, MD, AGAF; Chief Editor: BS Anand, MD more...
In patients with Budd-Chiari syndrome, aggressively seek specific therapy aimed at correcting or alleviating the obstruction. Also treat the underlying conditions aggressively.
Although medical therapy can be instituted for short-term, symptomatic benefit, the use of such treatment alone is associated with a high 2-year mortality rate (80-85%).
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.
This therapy has been used in a few cases. Agents include streptokinase, urokinase, recombinant tissue-type plasminogen activator (rt-PA), and other modalities.
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, 31, 32]
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.
A low-sodium diet is recommended for the control of ascites.
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.
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, 26, 33, 34, 35, 36]
Liver transplantation should be offered if decompensated liver cirrhosis is present.[4, 37]
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.
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.
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, 40]
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). The authors reported successful PTBA (performed after hepatovenography and with or without stenting) in 92 of the study’s patients, with all of the successful procedures resulting in significant symptom improvement.
Complications reported in the above study, all of which were managed nonsurgically, included acute hepatic vein thrombosis, occurring during or after the operation (3 patients); sustained intraperitoneal bleeding from the transhepatic puncture track (2 patients); pulmonary embolism, which occurred during the procedure (1 patient); and intrahepatic hematoma (1 patient). Primary patency rates at 6-, 12-, and 24-month follow-up were 84%, 78%, and 76%, respectively (although a number of patients did not return for follow-up). Secondary patency rates for these same follow-up periods 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.
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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