Pediatric Biliary Atresia Treatment & Management

  • Author: Steven M Schwarz, MD, FAAP, FACN, AGAF; Chief Editor: Carmen Cuffari, MD   more...
 
Updated: Oct 6, 2011
 

Medical Care

  • No primary medical treatment is relevant in the management of extrahepatic biliary atresia. The pediatrician's objective is to confirm the diagnosis.
  • Once biliary atresia is suspected, surgical intervention is the only mechanism available for a definitive diagnosis (intraoperative cholangiogram) and therapy (Kasai portoenterostomy).
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Surgical Care

  • Following a thorough evaluation for causes of neonatal cholestasis, intraoperative cholangiography establishes the diagnosis of extrahepatic biliary atresia.
  • During the operation, the fibrotic biliary tract remnant is identified, and the patency of the biliary system is assessed.
  • In cases in which biliary patency is associated with ductal hypoplasia, further surgical intervention is not indicated, and bile may be collected to evaluate for disorders of bile acid metabolism.
  • In the unusual circumstance of distal patency of the common duct with acceptable proximal luminal caliber, a modified portoenterostomy may be considered in place of the traditional Kasai procedure. However, the clinician must be aware that progression of disease pathophysiology may occur. The author has observed patients undergo modified portoenterostomies (gallbladder Kasai), only to subsequently experience continued inflammation and obliteration of the extrahepatic biliary tree and to ultimately require classic portoenterostomies.
  • In most cases of atresia, dissection into the porta hepatis and creation of a Roux-en-Y anastomosis with a 35-cm to 40-cm retrocolic jejunal segment is the procedure of choice.
  • Studies have shown that extension of the portal dissection beyond the portal vein bifurcation and the umbilical point in the left hilum may improve the likelihood of achieving adequate biliary drainage.
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Consultations

  • The evaluation of neonatal cholestasis may initially be carried out by the primary care provider, depending on the reliability of the laboratory in performing the necessary serum determinations indicated above.
  • Obviously, further nonsurgical testing (eg, hepatobiliary imaging, liver biopsy) and surgical exploration should only be carried out in centers with considerable experience in managing this disorder.
  • The physician must not delay in the diagnosis of extrahepatic biliary atresia. Refer infants for appropriate subspecialty care as soon as a diagnosis of obstructive jaundice is suspected.
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Diet

  • During the evaluation phase of biliary atresia, the infant's diet is typically not changed.
  • Postoperative breastfeeding is encouraged when possible because breast milk contains both lipases and bile salts to aid in lipid hydrolysis and micelle formation. Theoretically, breast milk may also protect against cholangitis, a common complication following portoenterostomy, by suppressing the growth of gram-negative and anaerobic flora. However, no data is available to support this claim.
  • Infants who are fed formula and who achieve adequate bile drainage should not require a special diet. Early in the postoperative course and when the status of biliary continuity may be in question, one of the medium-chain triglyceride-containing formulas (eg, Alimentum, Pregestimil) may enhance lipid digestion.
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Contributor Information and Disclosures
Author

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

Specialty Editor Board

Jorge H Vargas, MD  Professor of Pediatrics and Clinical Professor of Pediatric Gastroenterology, University of California, Los Angeles, David Geffen School of Medicine; Consulting Physician, Department of Pediatrics, University of California at Los Angeles Health System

Jorge H Vargas, MD is a member of the following medical societies: American Liver Foundation, American Society for Gastrointestinal Endoscopy, American Society for Parenteral and Enteral Nutrition, Latin American Society of Pediatric Gastroenterology, Hepatology & Nutrition, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

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.

Stefano Guandalini, MD  Director, Celiac Disease Center, Chief, Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Chicago Medical Center; Professor, Department of Pediatrics, Section of Gastroenterology, Hepatology and Nutrition, University of Chicago Division of the Biological Sciences, The Pritzker School of Medicine

Stefano Guandalini, MD is a member of the following medical societies: American Gastroenterological Association, European Society for Paediatric Gastroenterology, Hepatology & Nutrition, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

David Pallares, MD  Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville School of Medicine

David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology

Disclosure: Nothing to disclose.

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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Biliary atresia.
Bile ductular proliferation in liver biopsy specimen (hematoxylin and eosin stain) from patient with biliary atresia. Also note hepatocellular bile staining as a consequence of cholestasis.
 
 
 
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