Pediatric Budd-Chiari Syndrome Clinical Presentation

  • Author: Cass R Smith, MD; Chief Editor: Carmen Cuffari, MD   more...
 
Updated: Mar 22, 2012
 

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.[4]

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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.

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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
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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, Children's Hospital of Philadelphia; Professor, Department of Pediatrics, Division of Gastroenterology and Nutrition, University of Pennsylvania School of Medicine

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: Abbott, Inc Consulting fee Consulting

Michael C Stephens, MD, FAAP  Assistant Professor of Pediatrics, Section of Pediatric GI and Nutrition, Medical College of Wisconsin; Director of IBD Center, Children's Hospital of Wisconsin

Michael C Stephens, MD, FAAP 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.

Specialty Editor Board

Robert Baldassano, MD  Director, Center for Pediatric Inflammatory Bowel Disease, Children's Hospital of Philadelphia; Professor, Department of Pediatrics, Division of Gastroenterology and Nutrition, University of Pennsylvania School of Medicine

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: Abbott, Inc Consulting fee Consulting

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Steven M Schwarz, MD, FAAP, FACN, AGAF  Professor of Pediatrics, Children's Hospital at Downstate, State University of New York 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: Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor; Johnson & Johnson, 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.

References

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Ultrasound showing hepatic vein thrombus with vessels forming arrow pointing to the thrombus.
Ultrasound showing hepatic vein thrombus.
Calcified thrombus in the inferior vena cava (IVC) of a neonate secondary to an umbilical venous catheter (UVC).
 
 
 
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