eMedicine Specialties > Gastroenterology > Liver
Budd-Chiari Syndrome: Treatment & Medication
Updated: Sep 9, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
Medical therapy can be instituted for short-term, symptomatic benefit. Medical therapy alone is associated with a high 2-year mortality rate (80-85%).
- Management of ascites: See Ascites for more information.
- Anticoagulation
- Antithrombolytic therapy: This therapy has been used in a few cases. Agents include streptokinase, urokinase, recombinant tissue plasminogen activator, and other modalities.
- Angioplasty: This can help relieve obstruction caused by membranous webs.
Surgical Care
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 procedure can be performed.
Liver transplantation should be offered if decompensated liver cirrhosis is present (see Liver Transplantation for more information).
Consultations
- Gastroenterologist
- Hematologist
- Surgeon
- Radiologist
Diet
A low-sodium diet is recommended for the control of ascites.
Medication
Anticoagulation is needed in some patients, especially those with underlying hematological disorders as the cause of the syndrome.
Anticoagulants
Prevent recurrent or ongoing thromboembolic occlusion.
Warfarin (Coumadin)
Interferes with hepatic synthesis of vitamin K–dependent coagulation factors. Used for prophylaxis and treatment of venous thrombosis, pulmonary embolism, and thromboembolic disorders. Tailor dose to maintain an INR in the range of 2-3.
Adult
5-15 mg/d PO qd for 2-5 d; adjust dose according to desired INR
Pediatric
0.05-0.34 mg/kg/d PO; adjust dose according to desired INR
Drugs that may decrease anticoagulant effects include griseofulvin, carbamazepine, glutethimide, estrogens, nafcillin, phenytoin, rifampin, barbiturates, cholestyramine, colestipol, vitamin K, spironolactone, oral contraceptives, and sucralfate
Medications that may increase anticoagulant effects include oral antibiotics, phenylbutazone, salicylates, sulfonamides, chloral hydrate, clofibrate, diazoxide, anabolic steroids, ketoconazole, ethacrynic acid, miconazole, nalidixic acid, sulfonylureas, allopurinol, chloramphenicol, cimetidine, disulfiram, metronidazole, phenylbutazone, phenytoin, propoxyphene, sulfonamides, gemfibrozil, acetaminophen, and sulindac
Documented hypersensitivity; severe liver or kidney disease; open wounds; GI ulcers
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Do not switch brands after achieving therapeutic response; caution in active tuberculosis or diabetes; patients who have protein C or S deficiency are at risk of developing skin necrosis
Fibrinolytic agents
Used to dissolve a pathologic intraluminal thrombus or embolus that has not been dissolved by the endogenous fibrinolytic system. Also used for the prevention of recurrent thrombus formation and for the rapid restoration of hemodynamic disturbances.
Streptokinase (Kabikinase, Streptase)
Acts with plasminogen to convert plasminogen to plasmin. Plasmin degrades fibrin clots, fibrinogen, and other plasma proteins. Increase in fibrinolytic activity that degrades fibrinogen levels for 24-36 h occurs with intravenous infusion.
Adult
Not established; can be administered locally via catheter or IV
Local: 7500 U/h
IV: 100,000 U/h after loading dose of 250,000 U bolus
Pediatric
Administer as in adults
Antifibrinolytic agents may decrease effects; heparin, warfarin, and aspirin may increase risk of bleeding
Documented hypersensitivity; active internal bleeding, intracranial neoplasm, aneurysm, diathesis, or severe uncontrolled arterial hypertension
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in severe hypertension, IM administration of medications, or trauma or surgery in the previous 10 d; measure hematocrit, platelet count, aPTT, TT, PT, or fibrinogen levels before therapy is implemented; either TT or aPTT should be less than twice the normal control value following infusion of streptokinase and before instituting or reinstituting heparin; do not take blood pressure in lower extremities because may dislodge possible deep vein thrombi; PT, aPTT, TT, or fibrinogen should be monitored 4 h after initiation of therapy
Urokinase (Abbokinase)
Direct plasminogen activator that acts on the endogenous fibrinolytic system and converts plasminogen to the enzyme plasmin, which, in turn, degrades fibrin clots, fibrinogen, and other plasma proteins. Most often used for local fibrinolysis of thrombosed catheters and superficial vessels. Advantage is that agent is nonantigenic. However, more expensive than streptokinase and thus limits use. When used for local fibrinolysis, urokinase is given as local infusion directly into area of thrombus and with no bolus given. Dose should be adjusted to achieve clot lysis or patency of affected vessel.
Adult
Can be given locally or systemically
Loading dose: 4400 U/kg IV over 10 min and increase to 6000 U/kg/h
Maintenance dose: 4400-6000 U/kg/h IV
Pediatric
Administer as in adults
Thrombolytic enzymes, alone or in combination with anticoagulants and antiplatelets, may increase risk of bleeding complications
Documented hypersensitivity; internal bleeding, recent trauma, history of intracranial or intraspinal surgery or trauma, cerebrovascular accident, and intracranial neoplasm
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in patients receiving IM administration of medications, severe hypertension, and trauma or surgery in previous 10 d; to avoid dislodging possible deep vein thrombi, do not measure blood pressure in lower extremities; monitor therapy by measuring PT, aPTT, TT, or fibrinogen level approximately 4 h after initiation of therapy
Alteplase (Activase)
Tissue plasminogen activator used in management of acute myocardial infarction, acute ischemic stroke, and pulmonary embolism. Safety and efficacy with concomitant administration of heparin or aspirin during first 24 h after symptom onset have not been investigated.
Adult
0.25-0.50 mg/kg IV over 60 min
Pediatric
Administer as in adults
Anticoagulants and antiplatelets may increase risk of bleeding; may give heparin with and after alteplase infusions to reduce risk of rethrombosis; either heparin or alteplase may cause bleeding complications
Documented hypersensitivity; active internal bleeding, cerebrovascular accident or stroke within last 2 mo, intracranial or intraspinal surgery or trauma, intracranial hemorrhage upon pretreatment evaluation, possible subarachnoid hemorrhage, intracranial neoplasm, arteriovenous malformation or aneurysm, bleeding diathesis, or severe uncontrolled hypertension
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Monitor for bleeding, especially at arterial puncture sites, with coadministration of vitamin K antagonists; control and monitor blood pressure frequently during and following administration (when managing acute ischemic stroke); do not use >0.9 mg/kg to manage acute ischemic stroke; doses >0.9 mg/kg may cause ICH
More on Budd-Chiari Syndrome |
| Overview: Budd-Chiari Syndrome |
| Differential Diagnoses & Workup: Budd-Chiari Syndrome |
 Treatment & Medication: Budd-Chiari Syndrome |
| Follow-up: Budd-Chiari Syndrome |
| References |
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Further Reading
Keywords
Budd-Chiari syndrome, hepatic vein occlusion, hepatic vein obstruction, hepatic obstruction, liver obstruction, liver disease, hepatic disease, thrombotic hepatic vein obstruction, nonthrombotic hepatic vein obstruction, non-thrombotic hepatic vein obstruction, obliterating hepatic vein endophlebitis, hepatomegaly, ascites, thrombotic diathesis, congestive hepatopathy, hepatic congestion, liver congestion, membranous webs, acute liver disease, subacute liver disease, fulminant liver disease, liver failure
