Pediatric Biliary Atresia Treatment & Management
- Author: Steven M Schwarz, MD, FAAP, FACN, AGAF; Chief Editor: Carmen Cuffari, MD more...
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- 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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- 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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- 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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- 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.
A study examined the medical status of children with biliary atresia (BA) with their native livers after hepatoportoenterostomy (HPE) surgery. The study concluded that over 98% of this North American cohort of subjects with BA living with native livers 5 or more years after HPE have clinical or biochemical evidence of chronic liver disease. Cholangitis and fractures in long-term survivors also underscore the importance of ongoing medical surveillance.
Haber BA, Erlichman J, Loomes KM. Recent advances in biliary atresia: prospects for novel therapies. Expert Opin Investig Drugs. 2008 Dec. 17(12):1911-24. [Medline].
Bassett MD, Murray KF. Biliary atresia: recent progress. J Clin Gastroenterol. 2008 Jul. 42(6):720-9. [Medline].
Mogul D, Zhou M, Intihar P, Schwarz K, Frick K. Cost-Effective Analysis of Screening for Biliary Atresia With The Stool Color Card. J Pediatr Gastroenterol Nutr. 2014 Sep 11. [Medline].
Fischler B, Ehrnst A, Forsgren M, et al. The viral association of neonatal cholestasis in Sweden: a possible link between cytomegalovirus infection and extrahepatic biliary atresia. J Pediatr Gastroenterol Nutr. 1998 Jul. 27(1):57-64. [Medline].
Chang MH, Huang HH, Huang ES, et al. Polymerase chain reaction to detect human cytomegalovirus in livers of infants with neonatal hepatitis. Gastroenterology. 1992 Sep. 103(3):1022-5. [Medline].
Wilson GA, Morrison LA, Fields BN. Association of the reovirus S1 gene with serotype 3-induced biliary atresia in mice. J Virol. 1994 Oct. 68(10):6458-65. [Medline]. [Full Text].
Steele MI, Marshall CM, Lloyd RE, Randolph VE. Reovirus 3 not detected by reverse transcriptase-mediated polymerase chain reaction analysis of preserved tissue from infants with cholestatic liver disease. Hepatology. 1995 Mar. 21(3):697-702. [Medline].
Wang W, Donnelly B, Bondoc A, Mohanty SK, McNeal M, Ward R, et al. The rhesus rotavirus gene encoding VP4 is a major determinant in the pathogenesis of biliary atresia in newborn mice. J Virol. 2011 Jun 22. EPub ahead of print. [Medline]. [Full Text].
Uemura M, Ozawa A, Nagata T, et al. Sox17 haploinsufficiency results in perinatal biliary atresia and hepatitis in C57BL/6 background mice. Development. 2013 Feb. 140(3):639-48. [Medline].
Shneider BL, Magee JC, Karpen SJ, et al. Total Serum Bilirubin within 3 Months of Hepatoportoenterostomy Predicts Short-Term Outcomes in Biliary Atresia. J Pediatr. 2016 Mar. 170:211-217.e2. [Medline].
Zhou L, Shan Q, Tian W, Wang Z, Liang J, Xie X. Ultrasound for the Diagnosis of Biliary Atresia: A Meta-Analysis. AJR Am J Roentgenol. 2016 May. 206 (5):W73-82. [Medline].
Shteyer E, Wengrower D, Benuri-Silbiger I, Gozal D, Wilschanski M, Goldin E. Endoscopic retrograde cholangiopancreatography in neonatal cholestasis. J Pediatr Gastroenterol Nutr. 2012 Aug. 55(2):142-5. [Medline].
Ng VL, Haber BH, Magee JC, Miethke A, Murray KF, Michail S, et al. Medical status of 219 children with biliary atresia surviving long-term with their native livers: results from a North American multicenter consortium. J Pediatr. 2014 Sep. 165 (3):539-546.e2. [Medline].
Willot S, Uhlen S, Michaud L, Briand G, Bonnevalle M, Sfeir R, et al. Effect of ursodeoxycholic acid on liver function in children after successful surgery for biliary atresia. Pediatrics. 2008 Dec. 122(6):e1236-41. [Medline].
Lindor KD, Kowdley KV, Luketic VA, et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology. 2009 Sep. 50(3):808-14. [Medline]. [Full Text].
Bezerra JA, Spino C, Magee JC, Shneider BL, Rosenthal P, Wang KS, et al. Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial. JAMA. 2014 May 7. 311 (17):1750-9. [Medline].
Chen Y, Nah SA, Chiang L, Krishnaswamy G, Low Y. Postoperative steroid therapy for biliary atresia: Systematic review and meta-analysis. J Pediatr Surg. 2015 Jun 5. [Medline].
Superina R, Magee JC, Brandt ML, et al. The Anatomic Pattern of Biliary Atresia Identified at Time of Kasai Hepatoportoenterostomy and Early Postoperative Clearance of Jaundice Are Significant Predictors of Transplant-Free Survival. Ann Surg. 2011 Oct. 254(4):577-585. [Medline].
[Guideline] Murray KF, Carithers RL Jr. AASLD practice guidelines: Evaluation of the patient for liver transplantation. Hepatology. 2005 Jun. 41(6):1407-32. [Medline].
Tessier ME, Harpavat S, Shepherd RW, Hiremath GS, Brandt ML, Fisher A, et al. Beyond the Pediatric end-stage liver disease system: solutions for infants with biliary atresia requiring liver transplant. World J Gastroenterol. 2014 Aug 28. 20(32):11062-8. [Medline]. [Full Text].
Balistreri WF, Grand R, Hoofnagle JH, et al. Biliary atresia: current concepts and research directions. Summary of a symposium. Hepatology. 1996 Jun. 23(6):1682-92. [Medline].
Barshes NR, Lee TC, Balkrishnan R, et al. Orthotopic liver transplantation for biliary atresia: the U.S. experience. Liver Transpl. 2005 Oct. 11(10):1193-200. [Medline].
[Guideline] Bates MD, Bucuvalas JC, Alonso MH, Ryckman FC. Biliary atresia: pathogenesis and treatment. Semin Liver Dis. 1998. 18(3):281-93. [Medline].
Bittmann S. Surgical experience in children with biliary atresia treated with portoenterostomy. Curr Surg. 2005 Jul-Aug. 62(4):439-43. [Medline].
Chin LT, D'Alessandro AM, Knechtle SJ, et al. Liver transplantation for biliary atresia: 19-year, single-center experience. Exp Clin Transplant. 2004 Jun. 2(1):178-82. [Medline].
el-Youssef M, Whitington PF. Diagnostic approach to the child with hepatobiliary disease. Semin Liver Dis. 1998. 18(3):195-202. [Medline].
Karrer FM, Price MR, Bensard DD, et al. Long-term results with the Kasai operation for biliary atresia. Arch Surg. 1996 May. 131(5):493-6. [Medline].
Kasai M. Treatment of biliary atresia with special reference to hepatic porto- enterostomy and its modifications. Prog Pediatr Surg. 1974. 6:5-52. [Medline].
Lai MW, Chang MH, Hsu SC, et al. Differential diagnosis of extrahepatic biliary atresia from neonatal hepatitis: a prospective study. J Pediatr Gastroenterol Nutr. 1994 Feb. 18(2):121-7. [Medline].
Mack CL, Sokol RJ. Unraveling the pathogenesis and etiology of biliary atresia. Pediatr Res. 2005 May. 57(5 Pt 2):87R-94R. [Medline]. [Full Text].
Matsuo S, Suita S, Kubota M, Shono K. Long-term results and clinical problems after portoenterostomy in patients with biliary atresia. Eur J Pediatr Surg. 1998 Jun. 8(3):142-5. [Medline].
Mowat AP. Biliary atresia into the 21st century: a historical perspective. Hepatology. 1996 Jun. 23(6):1693-5. [Medline].
Muraji T, Higashimoto Y. The improved outlook for biliary atresia with corticosteroid therapy. J Pediatr Surg. 1997 Jul. 32(7):1103-6; discussion 1106-7. [Medline].
Nio M, Ohi R, Shimaoka S, et al. The outcome of surgery for biliary atresia and the current status of long-term survivors. Tohoku J Exp Med. 1997 Jan. 181(1):235-44. [Medline].
Okazaki T, Kobayashi H, Yamataka A, et al. Long-term postsurgical outcome of biliary atresia. J Pediatr Surg. 1999 Feb. 34(2):312-5. [Medline].
Otte JB, de Ville de Goyet J, Reding R, et al. Sequential treatment of biliary atresia with Kasai portoenterostomy and liver transplantation: a review. Hepatology. 1994 Jul. 20(1 Pt 2):41S-48S. [Medline].
[Guideline] Ryckman FC, Alonso MH, Bucuvalas JC, Balistreri WF. Biliary atresia--surgical management and treatment options as they relate to outcome. Liver Transpl Surg. 1998 Sep. 4(5 Suppl 1):S24-33. [Medline].
Tan CE, Davenport M, Driver M, Howard ER. Does the morphology of the extrahepatic biliary remnants in biliary atresia influence survival? A review of 205 cases. J Pediatr Surg. 1994 Nov. 29(11):1459-64. [Medline].
Tanaka H, Kita Y, Kawarasaki H, et al. Beneficial effect of ursodeoxycholic acid on serum gamma-GTP in patients with biliary atresia following living related liver transplantation. Transplant Proc. 1998 Nov. 30(7):3326-7. [Medline].
[Guideline] Utterson EC, Shepherd RW, Sokol RJ, et al. Biliary atresia: clinical profiles, risk factors, and outcomes of 755 patients listed for liver transplantation. J Pediatr. 2005 Aug. 147(2):180-5. [Medline].
Valayer J. Conventional treatment of biliary atresia: long-term results. J Pediatr Surg. 1996 Nov. 31(11):1546-51. [Medline].
Visser BC, Suh I, Hirose S, et al. The influence of portoenterostomy on transplantation for biliary atresia. Liver Transpl. 2004 Oct. 10(10):1279-86. [Medline].
Yoon PW, Bresee JS, Olney RS, et al. Epidemiology of biliary atresia: a population-based study. Pediatrics. 1997 Mar. 99(3):376-82. [Medline]. [Full Text].