Budd-Chiari Syndrome

Budd-Chiari syndrome is a condition resulting from the interruption of the normal outflow of blood from the liver. The primary type arises from a venous process (affecting the hepatic veins or inferior vena cava) such as thrombosis, but can also be from a lesion compressing or invading the veins (secondary type). The patient typically presents with hepatomegaly, ascites, and abdominal discomfort. Onset is often subacute or chronic. Diagnosis is confirmed by Doppler ultrasound. Treatment involves addressing the underlying condition that caused the venous occlusion. Further management involves prevention of further clotting (anticoagulation), restoration of blood flow, and decompressing the liver. Liver transplantation is considered if initial treatment fails and/or the patient has decompensated liver cirrhosis.

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Overview

Definition

  • Hepatic venous outflow obstruction
  • Interruption of outflow may be in the hepatic veins, inferior vena cava (IVC), or both.
  • Types:
    • Primary Budd-Chiari syndrome: obstruction due to a venous process or endoluminal venous lesion (thrombosis or phlebitis)
    • Secondary Budd-Chiari syndrome: compression or invasion of the hepatic veins and/or the IVC by a lesion originating outside the vein

Epidemiology

  • In non-Asian countries:
    • Women > men
    • Hepatic vein occlusion is more often noted.
  • In Asian countries:
    • Affects men predominantly
    • Pure IVC or combined IVC and hepatic vein blockage more common

Etiology

  • Hypercoagulable disorders (80%):
    • Polycythemia vera and other myeloproliferative disorders
    • Malignancy
    • Paroxysmal nocturnal hemoglobinuria (PNH)
    • Antiphospholipid syndrome
    • Factor V Leiden mutation
    • Antithrombin deficiency
    • Protein C and S deficiency
    • G20210A factor II gene mutation
    • Pregnancy
    • Side effect of medication (e.g., estrogen-containing hormonal contraception)
  • Malignancy (by direct invasion or compression):
    • Hepatocellular carcinoma is the most common.
    • Renal carcinoma
    • Cancer of the adrenal gland
  • Infections or benign liver lesions (e.g., abscess, adenoma)
  • Miscellaneous conditions: 
    • Celiac disease
    • Ulcerative colitis
    • Behcet’s syndrome
    • Sarcoidosis
    • Sjogren’s syndrome
    • Membranous webs (hepatic veins or IVC)
  • Idiopathic

Pathophysiology

  • Occlusion of one hepatic vein usually produces no pathology.
  •  When 2 of 3 hepatic veins are occluded, hepatic venous congestion occurs.
  • Hepatic venous congestion → sinusoidal dilation and postsinusoidal hypertension → increased interstitial fluid → increased liver pressure and congestion:
    • Liver capsule stretches (manifested as pain). 
    • Liver enlargement (hepatomegaly)
    • Filtered fluid exceeds lymphatic drainage capacity, causing fluid extravasation (ascites).
    • Increasing pressure and congestion → reduced blood flow → hepatocellular injury and damage (“nutmeg liver”: mottled appearance of the liver)
    • Persistent hepatic congestion and injury lead to complications:
      • Liver failure
      • Cirrhosis
      • Portal hypertension
      • Hepatocellular carcinoma
Pathophysiology Budd-Chiari syndrome

Budd-Chiari syndrome: Occlusion of hepatic veins causes hepatic venous congestion. This congestion increases the sinusoidal pressure within the liver, leading to reduced hepatic blood flow. This eventually produces hepatocellular injury from hypoxia. Hepatic damage then results in portal hypertension.

Image by Lecturio.

Clinical Presentation

  • Common symptom triad:
    1. Abdominal pain
    2. Hepatomegaly
    3. Ascites
  • May include jaundice, lower extremity edema, encephalopathy, and gastrointestinal bleeding depending on severity
  • Symptoms and abnormal findings vary by onset:
    •  Fulminant liver failure:
      • Least common presentation
      • Hepatic encephalopathy noted within 8 weeks of jaundice onset
      • Elevated transaminases and prothrombin time/international normalized ratio (PT/INR)
      • No venous collaterals
    • Acute Budd-Chiari syndrome:
      • Symptoms and signs unfold in a period of weeks, but without liver failure.
      • Jaundice, abdominal pain, intractable ascites 
      • No venous collaterals
    • Subacute Budd-Chiari syndrome:
      • Most common presentation
      • May be asymptomatic or common symptoms develop gradually over 3 months or more
      • Presence of venous collaterals 
    • Chronic Budd-Chiari syndrome:
      • Common symptoms present when cirrhosis has developed.
      • Stigmata of cirrhosis (spider angiomata, palmar erythema) noted
      • Signs of portal hypertension (esophageal varices, ascites, venous collaterals) also seen
      • Hepatic encephalopathy is uncommon.
Draining ascites cirrhosis

Image showing patient with ascites undergoing paracentesis

Image: “Draining ascites, secondary to hepatic cirrhosis” by John Campbell. License: Public Domain

Diagnosis

  • Doppler ultrasonography (US):  
    • 1st-line diagnostic test
    • Possible findings:
      • Non-visualization of the junction of the major hepatic veins with the IVC 
      • Thick, irregular, or dilated walls of the hepatic veins
      • Webs in the hepatic vein(s)
      • Visualization of thrombus; portal or splenic vein thrombosis can coincide 
    • Abnormal flow patterns in the hepatic vein:
      • Large hepatic vein without flow or with reversed/turbulent flow
      • Large intrahepatic or subcapsular collaterals with continuous flow
  • Computed tomography (CT) scan:
    • To confirm diagnosis and aid in treatment planning
    • Findings:
      • Delayed or absent filling of major hepatic veins
      • Narrowing and/or absence of flow in IVC
      • Patchy appearance of liver
      • Secondary causes such as tumors
  • Magnetic resonance imaging (MRI):
    • Similar findings as CT
    • Can demonstrate hepatic vein abnormalities and intrahepatic collaterals
  • Venography:
    • Gold standard
    • Performed if noninvasive tests are inconclusive
    • Information obtained can direct therapy as it defines which vessels are involved.
    • MRI venography also defines venous anatomy.
  • Liver biopsy:
    • Performed if diagnosis is unclear
    • Findings: centrizonal congestion, necrosis, and hemorrhage
    • Helps identify patients for transjugular intrahepatic portosystemic shunting (TIPS) when significant fibrosis or severe congestion is present
  • Additional tests:
    • Laboratory tests:
      • Acute or fulminant liver failure: ↑ aminotransferases
      • Fulminant liver failure: ↑ PT/INR 
      • Subacute or chronic: may have normal or mild elevation of aminotransferases 
    • Ascites fluid analysis: 
      • Serum-ascites albumin gradient (SAAG) > 1.1
      • WBC < 500/μL
    • As 80% of cases have an underlying condition, further work-up directed toward etiology is recommended.

Management and Prognosis

Management

  • Stabilize and treat the underlying etiology.
  • Prevent propagation of the clot: anticoagulants (heparin or warfarin) 
  • Relieve obstruction and restore blood flow:
    • Localized thrombolysis if with acute Budd-Chiari syndrome
    • Balloon angioplasty +/- stenting for acute or subacute disease
  • Decompress the congested liver by TIPS or surgical therapy:
    • Focus of treatment in chronic Budd-Chiari syndrome
    • An option for acute or subacute types if initial management is unsuccessful
    • TIPS placement creates an alternative outflow tract.
    • Surgical shunt:
      • A less common procedure
      • Drains the portal venous system into the IVC or another systemic vein
  • Prevent further liver deterioration and complication:
    • Dietary sodium restriction, diuretics, paracentesis to control portal hypertension complications
    • Liver transplantation: if initial management/liver decompression fails or if with decompensated cirrhosis

Prognosis

  • Prognosis worsens with the following factors:
    • Older age
    • Chronic disease at the time of diagnosis
    • Severe liver failure
    • Refractory ascites
    • Presence of portal vein thrombosis
  • 3-year survival of 10% without treatment
  • 5-year survival of up to 75% with treatment
  • Causes of death: intractable ascites, gastrointestinal bleeding, and liver failure

Differential Diagnosis

  • Congestive heart failure (CHF): inability of the heart to supply the body with normal cardiac output to meet metabolic needs. Right-sided heart failure causes congestive hepatopathy. Can present with nutmeg liver and signs of portal hypertension such as ascites, tender hepatomegaly, and lower extremity edema. Presence of elevated jugular venous pressure on exam is noted in CHF. Further differentiation of Budd-Chiari syndrome from CHF can be established using Doppler US.
  • Hepatic sinusoidal obstruction syndrome (veno-occlusive disease): a condition that can develop in adults who undergo hematopoietic cell transplantation. The pathophysiology is injury to the sinusoidal endothelial cells leading to liver necrosis. Presents as edema, painful hepatomegaly, ascites, and jaundice as in Budd-Chiari syndrome. History with laboratory and imaging tests leads to the diagnosis.
  • Other causes of acute or chronic liver disease (viral hepatitis, alcoholic liver disease, drug-induced hepatitis): Different types of liver disease also present with a similar presentation (jaundice, ascites, hepatomegaly). These conditions can have acute and chronic onset. Progression of liver injury, regardless of cause, can lead to cirrhosis and portal hypertension. History (medication, alcohol, exposure), laboratory tests, and imaging studies aid in determining the etiology of liver disease.

References

  1. Hitawala, A., Gupta, V. (2020). Budd Chiari Syndrome. https://www.ncbi.nlm.nih.gov/books/NBK558941/
  2. Lai, M. (2019). Budd-Chiari syndrome: Epidemiology, clinical manifestations, and diagnosis. UpToDate. Retrieved 5 Nov 2020 from: https://www.uptodate.com/contents/budd-chiari-syndrome-epidemiology-clinical-manifestations-and-diagnosis?search=budd%20chiari&source=search_result&selectedTitle=1~129&usage_type=default&display_rank=1#H79561969
  3. Lai, M. (2020) Etiology of the Budd-Chiari syndrome. UpToDate. Retrieved 16 Nov 2020 from https://www.uptodate.com/contents/etiology-of-the-budd-chiari-syndrome
  4. Lai, M. (2020) Budd-Chiari syndrome: Management. UpToDate. Retrieved 16 Nov 2020, from https://www.uptodate.com/contents/budd-chiari-syndrome-management
  5. Moore, C., Van Thiel, D. (2013) Cirrhotic ascites review: Pathophysiology, Diagnosis and Management. World J Hepatol. 5(5): 251–263.

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