Pediatric Budd-Chiari Syndrome Treatment & Management

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

Medical Care

In patients with Budd-Chiari syndrome (BCS), aggressively seek specific therapy aimed at correcting or alleviating the obstruction. Also treat underlying conditions aggressively. Symptomatic treatment for Budd-Chiari syndrome includes diuretics and therapeutic paracentesis, when necessary (can be associated with catastrophic complications, such as bacterial peritonitis).

  • Patients with liver failure and ascites have total body sodium overload, despite typically low serum sodium concentrations. Inducing negative sodium balance can reduce the amount of ascites. Take special care when using diuretics to avoid inducing hepatorenal syndrome or creating electrolyte and fluid disturbances through overly aggressive diuresis. Secondary hyperaldosteronism is a part of this clinical picture, making spironolactone typically the first-line diuretic. Chlorothiazide or furosemide is often added, which can provide synergy and avoid hyperkalemia.
  • The benefits of therapeutic paracentesis must be carefully weighed against the significant risks that can be associated with this procedure.
  • Anticoagulation is frequently used in hypercoagulable states.
  • Systemic thrombolysis can be a high-risk endeavor, and local thrombolysis performed by an interventional radiologist is preferable.
  • Other radiologic interventions available include balloon angioplasty, placement of stents, and transjugular intrahepatic portacaval shunt (TIPS). Adult studies suggest that the use of TIPS is safe and increases survival in patients with progressive liver disease and profound involvement of the hepatic vasculature due to Budd-Chiari syndrome who have failed medical therapy.[9]
  • A stepwise approach of anticoagulation, vascular intervention, TIPS and transplant can increase survival among patients.[10]
Next

Surgical Care

  • Mesocaval shunt
  • Mesoatrial shunt
  • Portacaval shunt
  • Liver transplantation: One study reported that, out of almost 79,000 liver transplantations in the United States from 1987-2006, 510 were for Budd-Chiari syndrome.[11] Graft survival was as high as 85%.
Previous
Next

Consultations

  • Consultants should be selected based on the individual clinical situation.
  • Early involvement of a hepatologist can help to establish the direction of workup and therapy.
  • Consultation with interventional radiologists, hematologists, oncologists, and general surgeons may be required, depending on the situation.
Previous
Next

Diet

  • Sodium restriction can be an important part of maintaining a negative sodium balance.
Previous
Proceed to Medication
 
 
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

  1. Chaudhuri M, Jayaranganath M, Chandra VS. Percutaneous Recanalization of an Occluded Hepatic Vein in a Difficult Subset of Pediatric Budd-Chiari Syndrome. Pediatr Cardiol. Feb 15 2012;[Medline].

  2. Paradis V, Bieche I, Dargere D, et al. Quantitative gene expression in Budd-Chiari syndrome: a molecular approach to the pathogenesis of the disease. Gut. Dec 2005;54(12):1776-81. [Medline].

  3. Rajani R, Melin T, Bjornsson E, et al. Budd-Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival - an 18-year experience. Liver Int. Feb 2009;29(2):253-9. [Medline].

  4. Polson J, Lee WM. AASLD position paper: the management of acute liver failure. Hepatology. May 2005;41(5):1179-97. [Medline]. [Full Text].

  5. Garcia-Pagan JC, Heydtmann M, Raffa S, Plessier A, Murad S, Fabris F. TIPS for Budd-Chiari syndrome: long-term results and prognostics factors in 124 patients. Gastroenterology. Sep 2008;135(3):808-15. [Medline].

  6. Hasija RP, Nagral A, Marar S, Bavdekar AR. Transjugular intrahepatic portosystemic shunt (TIPSS) for Budd Chiari syndrome. Indian Pediatr. Jun 2010;47(6):527-8. [Medline].

  7. Van Brusselen D, Janssen CE, Scott C, Bevers N, Roskams T, Wouters C, et al. Budd-Chiari syndrome as presenting symptom of hepatic sarcoidosis in a child, with recurrence after liver transplantation. Pediatr Transplant. Mar 2012;16(2):E58-62. [Medline].

  8. Tang TJ, Batts KP, de Groen PC. The prognostic value of histology in the assessment of patients with Budd-Chiarisyndrome. J Hepatol. Sep 2001;35(3):338-43. [Medline].

  9. Khuroo MS, Al-Suhabani H, Al-Sebayel M. Budd-Chiari syndrome: long-term effect on outcome with transjugular intrahepaticportosystemic shunt. J Gastroenterol Hepatol. Oct 2005;20(10):1494-502. [Medline].

  10. Valla DC. Primary Budd-Chiari syndrome. J Hepatol. Jan 2009;50(1):195-203. [Medline].

  11. Segev DL, Nguyen GC, Locke JE, et al. Twenty years of liver transplantation for Budd-Chiari syndrome: a national registry analysis. Liver Transpl. Sep 2007;13(9):1285-94. [Medline].

  12. Al-Damegh S. Budd-Chiari syndrome: a short radiological review. J Gastroenterol Hepatol. Nov 1999;14(11):1057-61. [Medline].

  13. Bogin V, Marcos A, Shaw-Stiffel T. Budd-Chiari syndrome: in evolution. Eur J Gastroenterol Hepatol. Jan 2005;17(1):33-5. [Medline].

  14. Colletti RB, Krawitt E. Ascites. In: Wyllie R, Hyams JS, eds. Pediatric Gastrointestinal Disease: Pathophysiology, Diagnosis, and Management. St. Louis, MO: Mosby Year Book; 1999:104-15.

  15. El-Youssef M, Freese DK. Budd-Chiari Syndrome. In: Walker WA, et al, eds. Pediatric Gastrointestinal Disease: Pathophysiology, Diagnosis, and Management. St. Louis, MO: Mosby Year Book; 2000:1235-6.

  16. Farrell MK, Bucuvalas JC. Systemic disease and the liver. In: Suchy FK, ed. Liver Disease in Children. St. Louis, MO: Mosby; 1994:582-3.

  17. Hirshfeld AB, Getachew A, Sessions J. Drug doses. In: The Harriet Lane Handbook: A Manual for Pediatric House Officers. 15th ed. Philadelphia, PA: WB Saunders; 2000:669, 725, 850-1.

  18. Ilkgul O, Kilic M, Icoz G, et al. Experience with mesocaval shunt with autologous jugular vein interpositionin patients with Budd-Chiari syndrome. Hepatogastroenterology. May-Jun 2005;52(63):662-5. [Medline].

  19. Jackson EK. Diuretics. In: Brunton L, Lazo J, Parker K. Goodman and Gilman's: The Pharmacological Basis of Therapeutics. 1996. 9th edition. New York, NY: McGraw-Hill; 1996:685-713.

  20. Janssen HL, Garcia-Pagan JC, Valla DC. The Budd-Chiari syndrome. N Engl J Med. Apr 29 2004;350(18):1906-8; author reply 1906-8. [Medline].

  21. Mahmoud A, Elias E. New approaches to the Budd-Chiari syndrome. J Gastroenterol Hepatol. Dec 1996;11(12):1121-3. [Medline].

  22. Menon KV, Shah V, Kamath PS. The Budd-Chiari syndrome. N Engl J Med. Feb 5 2004;350(6):578-85. [Medline].

  23. Noone TC, Semelka RC, Siegelman ES. Budd-Chiari syndrome: spectrum of appearances of acute, subacute, and chronicdisease with magnetic resonance imaging. J Magn Reson Imaging. Jan 2000;11(1):44-50. [Medline].

  24. Okuda K, Kage M, Shrestha SM. Proposal of a new nomenclature for Budd-Chiari syndrome: hepatic vein thrombosis versus thrombosis of the inferior vena cava at its hepatic portion. Hepatology. Nov 1998;28(5):1191-8. [Medline].

  25. Olzinski AT, Sanyal AJ. Treating Budd-Chiari Syndrome: making rational choices from a myriad of options. J Clin Gastroenterol. Mar 2000;30(2):155-61. [Medline].

  26. Orloff MJ, Daily PO, Orloff SL. A 27-year experience with surgical treatment of Budd-Chiari syndrome. Ann Surg. Sep 2000;232(3):340-52. [Medline].

  27. Rerksuppaphol S, Hardikar W, Smith AL, et al. Successful stenting for Budd-Chiari syndrome after pediatric liver transplantation: a case series and review of the literature. Pediatr Surg Int. Feb 2004;20(2):87-90. [Medline].

  28. Ruh J, Malago M, Busch Y, et al. Management of Budd-Chiari syndrome. Dig Dis Sci. Mar 2005;50(3):540-6. [Medline].

  29. Rössle M, Olschewski M, Siegerstetter V, Berger E, Kurz K, Grandt D. The Budd-Chiari syndrome: outcome after treatment with the transjugular intrahepatic portosystemic shunt. Surgery. Apr 2004;135(4):394-403. [Medline].

  30. Senzolo M, Cholongitas EC, Patch D. Update on the classification, assessment of prognosis and therapy of Budd-Chiarisyndrome. Nat Clin Pract Gastroenterol Hepatol. Apr 2005;2(4):182-90. [Medline].

  31. Singh V, Sinha SK, Nain CK, et al. Budd-Chiari syndrome: our experience of 71 patients. J Gastroenterol Hepatol. May 2000;15(5):550-4. [Medline].

  32. Slakey DP, Klein AS, Venbrux AC. Budd-Chiari syndrome: current management options. Ann Surg. Apr 2001;233(4):522-7. [Medline].

  33. Wang ZG, Zhang FJ, Yi MQ. Evolution of management for Budd-Chiari syndrome: a team's view from 2564 patients. ANZ J Surg. Jan-Feb 2005;75(1-2):55-63. [Medline].

  34. Xu PQ, Ma XX, Ye XX. Surgical treatment of 1360 cases of Budd-Chiari syndrome: 20-year experience. Hepatobiliary Pancreat Dis Int. Aug 2004;3(3):391-4. [Medline].

  35. Zeitoun G, Escolano S, Hadengue A. Outcome of Budd-Chiari syndrome: a multivariate analysis of factors related to survival including surgical portosystemic shunting. Hepatology. Jul 1999;30(1):84-9. [Medline].

 
Previous
Next
 
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).
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.