Pediatric Budd-Chiari Syndrome Workup

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

Laboratory Studies

  • Evaluate patients with Budd-Chiari syndrome (BCS) for underlying predisposing conditions, such as malignancy or hypercoagulable states, and institute appropriate therapy.
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Imaging Studies

  • Budd-Chiari syndrome is usually diagnosed initially using Doppler ultrasonography, which has a sensitivity and specificity of 85% or higher.
  • Detailed imaging studies are required to determine precisely the level and degree of obstruction. CT scanning can rarely provide such detail, unless a mechanical obstruction, such as a locally invading tumor, is suspected.
  • MRI, which has a sensitivity and specificity of 90% or higher, is becoming increasingly useful in providing less-invasive venography, angiography, and cholangiography. MRI may assist in differentiating acute from chronic Budd-Chiari syndrome because it is able to provide a larger image of the vasculature, as well as determine if edema of the parenchyma is present (acute form).
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Procedures

  • Interventional radiology: Catheterization and venography can clearly delineate the nature and severity of the obstruction. Occasionally, therapeutic interventions can be undertaken at the same time, and they can include balloon angioplasty, placement of a stent, localized thrombolysis, or transjugular intrahepatic portacaval shunt (TIPS).[5, 6]
  • Paracentesis: The benefits of therapeutic paracentesis must be carefully weighed against the significant risks that can be associated with this procedure.
  • Percutaneous liver biopsy: Liver biopsy can be of prognostic assistance, particularly if liver transplantation is being considered, to establish the degree of hepatocellular damage and the presence and degree of fibrosis.[7]
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Histologic Findings

  • Histologic findings can range from nearly normal to severe chronic congestion with fibrosis, reversed lobulation, and dilated lymphatic channels.
  • The most severe end of this spectrum can include fulminant hepatic failure with massive centrilobular necrosis.
  • Adult studies have shown that early pathology related to Budd-Chiari syndrome did not have a significant impact on survival.[8]
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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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