para>Overly aggressive diuresis can induce prerenal acute kidney injury, encephalopathy, and hyponatremia. Monitor creatinine and electrolytes closely. Serum creatinine >2 mg/dL or serum sodium <120 mmol/L should prompt withdrawal of diuretics.
ISSUES FOR REFERRAL
Consider referral for liver transplant in patients with decompensated liver disease. Liver transplant is the definitive treatment for portal hypertension (HTN).
COMPLEMENTARY & ALTERNATIVE MEDICINE
Avoid herbal and other supplements due to risk of drug interactions and hepatotoxicity.
ONGOING CARE
PROGNOSIS
- Without intervention, 5-year survival is <10%.
- With intervention, the 5-year survival rate is ~70%.
- Unfavorable prognostic factors include the following:
- Advanced age
- Elevated creatinine serum levels
- Prolonged partial thromboplastin time
- Ascites
- Encephalopathy
- Elevated total bilirubin levels
- Elevated INR levels
- Accompanying portal vein thrombosis
- The Rotterdam score predicts 3-month mortality based on (5):
- Encephalopathy
- Ascites
- Partial thromboplastin time
- Bilirubin levels
COMPLICATIONS
- Hepatocellular carcinoma (HCC) is more frequent with BCS; routine surveillance is recommended (8)[A].
- Hepatopulmonary disease
- Hepatic vein thrombosis following liver transplantation
- Recurrent BCS occurs, on average, ~9 months to several years following liver transplantation without sufficient thrombolytic therapy. Recurrence within hours to days with proper anticoagulation initiation has occurred.
- Hepatorenal syndrome: Diagnosed when possible causes of acute renal failure are excluded, at least 2 days of diuretic withdrawal, and after maximal intravascular volume expansion with albumin; typically has evidence of worsening renal function: urine volume <500 mL/day, rising BUN, and creatinine >1.5 mg/dL, fractional excretion of sodium <1%, and decreased urine sodium excretion (<20 mEq/L, indicates significant sodium avidity):
- Stop diuretics and begin intravascular volume expansion with crystalloid and colloid (5 to 20 g IV albumin/day) (4)[B].
- Consider vasoactive drugs, such as octreotide 200 μg SC TID and midodrine 12.5 mg PO TID, either alone or together, in addition to colloid volume expanders rapidly progressive (type I) renal failure (7)[B].
- Liver transplant is definitive treatment.
- May need hemodialysis to control azotemia and correct electrolyte imbalance while awaiting transplant
REFERENCES
11 Valla DC. Hepatic venous outflow tract obstruction etiopathogenesis: Asia versus the West. J Gastroenterol Hepatol. 2004;19:S204-S211.22 Valla DC. The diagnosis and management of the Budd-Chiari syndrome: consensus and controversies. Hepatology. 2003;38(4):793-803.33 Seijo S, Plessier A, Hoekstra J, et al. Good long-term outcome of Budd-Chiari syndrome with a step-wise management. Hepatology. 2013;57(5):1962-1968.44 Orloff MJ, Isenberg JI, Wheeler HO, et al. Budd-Chiari syndrome revisited: 38 years' experience with surgical portal decompression. J Gastrointest Surg. 2012;16(2):286-300.55 Montano-Loza AJ, Tandon P, Kneteman N, et al. Rotterdam score predicts early mortality in Budd-Chiari syndrome, and surgical shunting prolongs transplant-free survival. Aliment Pharmacol Ther. 2009;30(10):1060-1069.66 Plessier A, Rautou PE, Valla DC. Management of hepatic vascular diseases. J Hepatol. 2012;56(Suppl 1):S25-S38.77 Garcia-Pag ¡n JC, Heydtmann M, Raffa S, et al. TIPS for Budd-Chiari syndrome: long-term results and prognostics factors in 124 patients. Gastroenterology. 2008;135(3):808-815.88 Ren W, Qi X, Yang Z, et al. Prevalence and risk factors of hepatocellular carcinoma in Budd-Chiari syndrome: a systematic review. Eur J Gastroenterol Hepatol. 2013;25(7):830-841.
ADDITIONAL READING
- Burton JRJr, Rosen HR. Diagnosis and management of allograft failure. Clin Liver Dis. 2006;10(2):407-435.
- Horton JD, San Miguel FL, Membreno F, et al. Budd-Chiari syndrome: illustrated review of current management. Liver Int. 2008;28(4):455-466.
- Jayanthi V, Udayakumar N. Budd-Chiari syndrome. Changing epidemiology and clinical presentation. Minerva Gastroenterol Dietol. 2010;56(1):71-80.
SEE ALSO
- Ascites; Pregnancy, Cholestasis
- Algorithm: Hepatomegaly; AST Elevation
CODES
ICD10
I82.0 Budd-Chiari syndrome
ICD9
453.0 Budd-chiari syndrome
SNOMED
Budd-Chiari syndrome (disorder)
CLINICAL PEARLS
- Consider BCS in young female patients with sudden onset of RUQ pain, hepatomegaly, and ascites.
- BCS is commonly mistaken for right heart failure.