eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology

Budd-Chiari Syndrome

Author: Cass R Smith, MD, Pediatric Gastroenterologist, Idaho Pediatric Gastroenterology, PA, and St Luke's Boise Regional Medical Center
Coauthor(s): Robert Baldassano, MD, Director, Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology and Nutrition, Associate Professor, Department of Pediatrics, The Children's Hospital of Philadelphia, University of Pennsylvania; Michael Stephens, MD, Assistant Professor, Department of Pediatrics, Section of Gastroenterology and Nutrition, Children's Hospital of Wisconsin
Contributor Information and Disclosures

Updated: Apr 15, 2009

Introduction

Background

Budd-Chiari syndrome (BCS) refers to the noncardiogenic obstruction of hepatic venous flow at any level above the venule. Obstruction can result from various conditions, particularly prothrombotic states. Budd-Chiari syndrome should be considered separate from veno-occlusive disease (VOD), also known as sinusoidal obstruction syndrome, which is characterized by toxin-induced nonthrombotic obstruction of prehepatic veins.

Ultrasound showing hepatic vein thrombus with ves...

Ultrasound showing hepatic vein thrombus with vessels forming arrow pointing to the thrombus.

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Ultrasound showing hepatic vein thrombus with ves...

Ultrasound showing hepatic vein thrombus with vessels forming arrow pointing to the thrombus.


Ultrasound showing hepatic vein thrombus.

Ultrasound showing hepatic vein thrombus.

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Ultrasound showing hepatic vein thrombus.

Ultrasound showing hepatic vein thrombus.


Pathophysiology

Occlusion of a single hepatic vein is usually silent. Overt Budd-Chiari syndrome generally requires the occlusion of at least 2 hepatic veins. Venous congestion of the liver causes hepatomegaly, which can stretch the liver capsule and be very painful. Enlargement of the caudate lobe is common because blood is shunted through it directly into the inferior vena cava (IVC). Hepatic function can be affected to a degree, depending on the amount of stasis and resultant hypoxia. Increased sinusoidal pressure can itself cause hepatocellular necrosis. The literature also suggests that upregulation of specific genes in chronic Budd-Chiari syndrome contributes to liver destruction through the stimulation of extracellular matrix proliferation, which contributes to liver fibrosis. The most prominent genes involved include matrix metalloproteinase 7 and superior cervical ganglion 10 (SCG10), which are increased in expression, and thrombospondin-1, which is decreased.1

Frequency

International

Budd-Chiari syndrome is extremely rare, and the incidence is not well reported in the literature; however, membranous (or congenital) forms of Budd-Chiari syndrome are the most common cause of Budd-Chiari syndrome worldwide, particularly in Asia. One study in Sweden reports an incidence of about 1 case per million population per year.2

Mortality/Morbidity

The mortality rate can be high in patients who develop fulminant hepatic failure. Morbidity and mortality are generally related to complications of liver failure and ascites. The type of concomitant underlying disease, if any, can also impact morbidity and mortality. Long-term observation in adults has demonstrated 10-year survival rates as high as 55%.

Sex

No data suggest that sex affects predisposition. However, in the United States, Budd-Chiari syndrome is predominantly seen in women and is associated with hematologic disorders.

Age

Budd-Chiari syndrome is rare in the general population and even more so in children. Peak incidence seems to be in persons aged 40-50 years.

Clinical

History

Patients with acute onset of obstruction typically present with acute right upper quadrant pain. Abdominal distention can also be a significant symptom because ascites develop. Jaundice is rarely observed. Various symptoms that are potentially related to underlying and predisposing conditions can accompany the onset of Budd-Chiari syndrome (BCS). If the liver has had time to develop collaterals and decompress, patients can be asymptomatic or present with few symptoms. Progression of Budd-Chiari syndrome can lead to liver failure and portal hypertension with corresponding symptoms (eg, encephalopathy, hematemesis). The American Association for the Study of Liver Diseases has released guidelines for the management of acute liver failure.3

Physical

Tender hepatomegaly with ascites and splenomegaly are common findings. Engorgement of the vessels of the chest and abdominal wall can also be observed. Bilirubin and transaminases are often mildly elevated. Prolongation of the prothrombin time (PT) is common and can be confusing in the setting of a hypercoagulable state.

Causes

Budd-Chiari syndrome can frequently be idiopathic; however, several main causes of this disorder are noted.

  • Mechanical causes
    • Congenital membranous obstruction
      • Type I: Thin membrane is present at the vena cava or atrium.
      • Type II: A segment of the vena cava is absent.
      • Type III: The inferior vena cava (IVC) cannot be filled, and collaterals have developed.
    • Hepatic venous stenosis
    • Hypoplasia of the suprahepatic veins
    • Postsurgical obstruction
    • Posttraumatic obstruction
    • Tumor invasion
    • Total parenteral nutrition (TPN): BCS has been reported as a complication of TPN via an IVC catheter in a neonate.
  • Causes related to hypercoagulable states
    • Hematologic disorders
      • Polycythemia vera
      • Essential thrombocytosis
      • Paroxysmal nocturnal hemoglobinuria
      • Myeloproliferative disorders, which may account for almost half of cases
    • Coagulopathies
      • Factor V Leiden mutation
      • Protein C deficiency
      • Protein S deficiency
      • Antithrombin II deficiency
    • Antiphospholipid antibody syndrome
    • Other causes of hypercoagulability
      • Collagen vascular diseases
      • Sickle cell disease
      • Inflammatory bowel disease
      • Oral contraceptives
      • Postpartum malignancy
  • Causes related to infection

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
Multimedia: Budd-Chiari Syndrome
References
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References

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Further Reading

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Keywords

Budd-Chiari syndrome, BCS, membranous Budd-Chiari syndrome, membranous BCS, hepatic vein thrombosis, congenital Budd-Chiari syndrome, congenital BCS, veno-occlusive disease, sinusoidal obstruction syndrome, hepatomegaly, liver failure, ascites, jaundice, encephalopathy, hematemesis, congenital membranous obstruction, hepatic venous stenosis, tumor invasion, treatment, diagnosis, polycythemia vera, thrombocytosis, paroxysmal nocturnal hemoglobinuria, myeloproliferative disorder, factor V Leiden mutation, protein C deficiency, protein S deficiency, antithrombin II deficiency, antiphospholipid antibody syndrome, sickle cell disease, inflammatory bowel disease, tuberculosis, aspergillosis, filariasis, echinococcus

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Contributor Information and Disclosures

Author

Cass R Smith, MD, Pediatric Gastroenterologist, Idaho Pediatric Gastroenterology, PA, and St Luke's Boise Regional Medical Center
Cass R Smith, MD is a member of the following medical societies: North American Society for Pediatric Gastroenterology, Hepatology and Nutrition
Disclosure: Nothing to disclose.

Coauthor(s)

Robert Baldassano, MD, Director, Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology and Nutrition, Associate Professor, Department of Pediatrics, The Children's Hospital of Philadelphia, University of Pennsylvania
Robert Baldassano, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

Michael Stephens, MD, Assistant Professor, Department of Pediatrics, Section of Gastroenterology and Nutrition, Children's Hospital of Wisconsin
Michael Stephens, MD is a member of the following medical societies: American Academy of Pediatrics, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

Medical Editor

Robert Baldassano, MD, Director, Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology and Nutrition, Associate Professor, Department of Pediatrics, The Children's Hospital of Philadelphia, University of Pennsylvania
Robert Baldassano, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

CME Editor

Steven M Schwarz, MD, FAAP, FACN, AGAF, Professor of Pediatrics, Children's Hospital at Downstate, SUNY-Downstate Medical Center
Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research
Disclosure: TAP Pharmaceuticals Honoraria Speaking and teaching; Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor

Chief Editor

Carmen Cuffari, MD, Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine
Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

 
 
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