II. Definitions

  1. Budd-Chiari Syndrome (BCS)
    1. Hepatic Venous Outflow Tract Obstruction within IVC or hepatic veins, and due to non-cardiac cause
    2. Primary BCS are conditions affecting the venous lumen (e.g. thrombosis)
    3. Secondary BCS are external conditions that invade or compress venous outflow (hepatic veins, IVC)

III. Epidemiology

  1. Incidence: 0.1 to 10 per Million (rare)
  2. Age: Peaks age 20 to 40 years
  3. Gender Predominance
    1. Non-asian countries: Women (hepatic vein obstruction)
    2. Asian countries: Men (Inferior vena cava or hepatic vein obstruction)

IV. Pathophysiology

  1. Venous congestion occurs when more than one hepatic vein is obstructed
  2. Lymphatics return a part of the excessive fluid
  3. Hepatic sinusoids dilate with congestion
    1. Hepatomegaly
    2. Right upper quadrant pain (liver capsule stretch)
    3. Ascites (leakage of fluid from liver capsule into peritoneum)
  4. Portal venous Hypertension
    1. Centrilobular hepatocyte Hypoxia
  5. Fibrosis and Hepatic failure with longterm obstruction
    1. Caudate liver lobe is most commonly affected by Budd-Chiari (due to shunting to IVC)

V. Causes

  1. Idiopathic in 20% of cases
  2. Hypercoagulable State
    1. Contributes to most of the other Budd-Chiari Syndrome causes
    2. Malignancy
    3. Pregnancy
    4. Oral Contraceptives
    5. Inherited conditions
      1. Factor V (Leiden) mutation
      2. Antiphospholipid Antibody Syndrome
      3. Antithrombin III Deficiency
      4. Protein C Deficiency
      5. Paroxysmal Nocturnal Hemoglobinuria
  3. Myeloproliferative Disorders
    1. Polycythemia Vera
    2. Essential Thrombocythemia
  4. Malignancy (10% of cases)
    1. Mass compresses or invades venous outflow
    2. Hepatocellular Carcinoma
    3. Renal Cell Carcinoma
    4. Adrenal Cancer
    5. Leiomyosarcoma
    6. Right atrial Myxoma
    7. Wilms tumor
  5. Other Compressive Masses
    1. Hepatic Cysts
    2. Abscess
    3. Aortic aneurysm

VI. Findings: Presentations

  1. Acute
    1. Rapid developoment over weeks (too rapidly to have developed collateral venous drainage)
    2. Significant acute hepatic necrosis
    3. Presents with Abdominal Pain, Jaundice, severe Ascites
    4. Hepatic Encephalopathy and fulminant liver failure may occur is some cases
  2. Subacute
    1. Gradual onset over months with more mild symptoms
    2. Collateral venous drainage develops and spares hepatocyte necrosis and minimal Ascites
  3. Chronic
    1. Cirrhosis may be present
    2. Collateral venous drainage is well developed
    3. Progressive Ascites
    4. Jaundice is typically absent
    5. Renal Impairment in 50% of cases

VII. Findings: General

  1. BCS Triad is commonly present (esp. Acute BCS)
    1. Abdominal Pain
    2. Ascites
    3. Hepatomegaly
  2. Other findings
    1. Jaundice
    2. Splenomegaly
    3. Leg Edema
    4. Venous Stasis Ulcers

VIII. Labs

  1. Liver Function Tests
    1. Serum transaminase increased (esp. acute BCS)
    2. Alkaline Phosphatase increased
    3. Serum Direct Bilirubin increased (acute BCS)
    4. Prothrombin (INR) increased (esp. acute and chronic BCS)
  2. Paracentesis
    1. Fluid Protein increased (>2 g) in chronic BCS
    2. White Blood Cell Count low (<500) in chronic BCS
    3. Serum Ascites-albumin gradient low (<1.1 g/dl) in chronic BCS
  3. Liver Biopsy
    1. Centrilobular hepatocyte necrosis
    2. Sinusoidal dilation

IX. Imaging

  1. Right Upper Quadrant Color-Flow Doppler Ultrasound
    1. First-line study in Budd-Chiari Syndrome
    2. High Test Sensitivity and Test Specificity for thrombosis (>85%) within IVC or hepatic veins
    3. Also identifies collateral venous drainage, Ascites and caudate lobe size
  2. Other imaging
    1. Consider CT Abdomen or MRI Abdomen

X. Differential Diagnosis

XI. Management

  1. Consult Hepatology
  2. Anticoagulation
    1. Key management in nearly all patients
    2. Warfarin is most commonly used (after initial low-molecular-weight Heparin)
  3. Relieving obstruction
    1. Thrombolysis with stenting
    2. Transjugular intrahepatic portosystemic shunt (TIPS)
    3. Surgical decompression in refractory acute cases
  4. Liver Transplantation
    1. Indicated in decompensated Cirrhosis
    2. Ten year survival >=70% after transplant

XII. Prognosis: Better Prognostic Factors

  1. Younger age
  2. Low Child-Pugh Score
  3. No Ascites
  4. Normal Serum Creatinine

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Related Studies

Ontology: Budd-Chiari Syndrome (C0856761)

Definition (MSHCZE) Onemocnění charakterizované obliterací jaterních žil (zejm. v důsledku jejich trombózy), které vedou krev z jater do dolní duté žíly. Jedna z příčin posthepatální (portální) hypertenze. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (MSH) A condition in which the hepatic venous outflow is obstructed anywhere from the small HEPATIC VEINS to the junction of the INFERIOR VENA CAVA and the RIGHT ATRIUM. Usually the blockage is extrahepatic and caused by blood clots (THROMBUS) or fibrous webs. Parenchymal FIBROSIS is uncommon.
Concepts Disease or Syndrome (T047)
MSH D006502
ICD9 453.0
ICD10 I82.0
SnomedCT 82385007, 195436007
English Chiari Syndrome, Chiari's Syndrome, Chiaris Syndrome, BUDD CHIARI SYNDROME, Budd Chiari Syndrome, HEPATIC VEIN OBSTRUCTION, BUDD-CHIARI SYNDROME, Hepatic Venous Outflow Obstruction, Budd-Chiari syndrome (diagnosis), Syndrome Budd-Chiari, Hepatic venous outflow obstruction, Budd-Chiari Syndrome [Disease/Finding], Budd-Chiari Syndrome, Hepatic vein obstruction, BDCHS, Budd-chiari syndrome, Budd Chiari syndrome, Budd - Chiari syndrome (hepatic vein thrombosis), Budd-Chiari syndrome, Obstruction of hepatic veins, Budd-Chiari syndrome (disorder), block; hepatic vein, Budd-Chiari, Chiari; disease or syndrome, syndrome; Chiari, v.hepatica; block
Portuguese SINDROME DE BUDD-CHIARI, Obstrução do escoamento venoso hepático, Obstrução da veia hepática, Obstrução do Fluxo Venoso Hepático, Síndrome de Budd-Chiari, Obstrução Hepática do Fluxo Venoso, Síndrome de Chiari
Spanish SINDROME DE BUDD-CHIARI, Síndrome de Budd Chiari, Síndrome de Bud-Chiari, Obstrucción venosa hepática, Obstrucción de vena hepática, Obstrucción del Flujo Venoso Hepático, Síndrome de Budd-Chiari, Obstrucción Hepática del Flujo Venoso, obstrucción de las venas hepáticas, obstrucción de las venas suprahepáticas, síndrome de Budd - Chiari (trastorno), síndrome de Budd - Chiari, Síndrome de Chiari
Dutch trombose van vena hepatica, veneuze afvloedbelemmering lever, syndroom van Budd-Chiari, vena hepatica obstructie, Chiari; ziekte of syndroom, block; vena hepatica, syndroom; Chiari, v.hepatica; block, Syndroom van Budd-Chiari, Budd-Chiari-syndroom
French Occlusion de la veine hépatique, Obstruction du débit veineux hépatique, Bloc supra-hépatique, Obstruction des veines sus-hépatiques, Syndrome de Budd-Chiari, SYNDROME DE BUDD-CHIARI, Maladie de Budd-Chiari
German Lebervenenobstruktion, Lebererkrankung mit Venenokklusion, Budd-Chiari-Syndrom, Lebervenenverschluss, BUDD-CHIARI SYNDROM, Budd-Chiari Syndrom, Chiari-Syndrom
Italian Ostruzione all'efflusso venoso del fegato, Ostruzione della vena epatica, Sindrome di Budd Chiari, Sindrome di Chiari, Sindrome di Budd-Chiari, Ostruzione dell'efflusso venoso epatico
Japanese 肝静脈流出路閉塞, 肝静脈閉塞, バッドキアリ症候群, バッドキアリショウコウグン, カンジョウミャクヘイソク, カンジョウミャクリュウシュツロヘイソク, バッド・キアリ症候群, 肝静脈血栓症, Budd-Chiari症候群, Chiari症候群, キアリ病, キアリ症候群, バッド-キアリ症候群, 血栓症-肝静脈
Finnish Budd-Chiarin oireyhtymä
Russian БАДДА-КИАРИ СИНДРОМ, ПЕЧЕНОЧНОЙ ВЕНЫ ТРОМБОЗ, PECHENOCHNOI VENY TROMBOZ, KIARI SINDROM, BADDA-KIARI SINDROM, КИАРИ СИНДРОМ
Swedish Budd-Chiaris syndrom
Czech Budd-Chiariho syndrom, Obstrukce jaterní žíly, Obstrukce odvodných jaterních žil, Buddův-Chiariho syndrom
Korean 버드-키아리 증후군
Polish Zespół Budda-Chiariego, Zakrzep żył wątrobowych
Hungarian Budd-Chiari-syndroma, Budd-Chiari syndroma, Hepatikus vena obstructio, Venás kiáramlási obstructio a májban, Budd Chiari syndroma
Norwegian Budd-Chiari-syndrom