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European Journal of Pediatrics

Published in:

01-04-2010 | Review

Clinical practice:

Management of biliary atresia

Authors: Basem A. Khalil, M. Thamara P. R. Perera, Darius F. Mirza

Published in: European Journal of Pediatrics | Issue 4/2010

Abstract

Biliary atresia is a rare, serious and challenging disease in newborn children. Its aetiology remains unknown. Optimal management at specialist centres with resultant better overall outcomes is achieved through a multidisciplinary team approach. The Kasai portoenterostomy performed early in life remains the only surgical repair procedure. Two thirds of patients will clear their jaundice after a Kasai procedure, but only about one third will retain their livers after the first decade of life. Failure of this procedure leaves liver transplantation as the only chance for survival, and this disease is the commonest indication for liver transplantation in children. With modern medical care and refinements in surgical techniques, survival after either or both of these procedures is about 90%. Early referral to specialist centres and long-term specialist care remains the key to successful treatment of this condition.

Anderson CD, Turmelle YP, Lowell JA et al (2008) SPLIT Research Group: the effect of recipient-specific surgical issues on outcome of liver transplantation in biliary atresia. Am J Transplant 8:1197–1204CrossRefPubMed

Ayuso L, Vila-Carbó JJ, Lluna J et al (2008) Laparoscopic Kasai portoenterostomy: present and future of biliary atresia treatment. Cir Pediatr 21:23–26PubMed

Barshes NR, Lee TC, Balkrishnan R et al (2005) Orthotopic liver transplantation for biliary atresia: the U.S. experience. Liver Transpl 11:1193–1200CrossRefPubMed

Bourdeaux C, Tri TT, Gras J et al (2005) PELD score and post transplant outcome in pediatric liver transplantation: a retrospective study of 100 recipients. Transplantation 79:1273–1276CrossRefPubMed

Burgos L, Hernández F, Barrena S et al (2008) Variant techniques for liver transplantation in pediatric programs. Eur J Pediatr Surg 18:372–374CrossRefPubMed

Caponcelli E, Knisely AS, Davenport M (2008) Cystic biliary atresia: an etiologic and prognostic subgroup. J Pediatr Surg 43:1619–1624CrossRefPubMed

Chardot C, Herrera JM, Debray D et al (1997) Portal vein complications after liver transplantation for biliary atresia. Liver Transpl Surg 3:351–358CrossRefPubMed

Chardot C (2006) Biliary atresia. Orphanet J Rare Dis 1:28CrossRefPubMed

Chiba T, Ohi R, Kamiyama T, Nio M, Ibrahim M (1991) Japanese biliary atresia registry. In: Ohi R (ed) biliary atresia. ICOM Assoc, Tokyo, pp 154–158

10.

Cowles RA, Lobritto SJ, Ventura KA et al (2008) Timing of liver transplantation in biliary atresia-results in 71 children managed by a multidisciplinary team. J Pediatr Surg 43:1605–1609CrossRefPubMed

11.

Cowley AD, Cummins C, Beath SV et al (2007) Paediatric Hepatology Dependency score (PHD score): an audit tool. J Pediatr Gastroenterol Nutr 44:108–115CrossRefPubMed

12.

Davenport M, Ville D, de Goyet J, Stringer MD et al (2004) Seamless management of biliary atresia in England and Wales (1999–2002). Lancet 363:1354–1357CrossRefPubMed

13.

Davenport M, Puricelli V, Farrant P et al (2004) The outcome of the older (> or =100 days) infant with biliary atresia. J Pediatr Surg 39:575–581CrossRefPubMed

14.

Davenport M, Tizzard SA, Underhill J et al (2006) The biliary atresia splenic malformation syndrome: a 28-year single-center retrospective study. J Pediatr 149:393–400CrossRefPubMed

15.

Davenport M, Ure BM, Petersen C et al (2007) Surgery for biliary atresia—is there a European consensus? Eur J Pediatr Surg 17:180–183CrossRefPubMed

16.

Davenport M (2005) Biliary atresia. Semin Pediatr Surg 14:42–48CrossRefPubMed

17.

Davit-Spraul A, Baussan C, Hermeziu B et al (2008) CFC1 gene involvement in biliary atresia with polysplenia syndrome. J Pediatr Gastroenterol Nutr 46:111–112CrossRefPubMed

18.

Hartley JL, Davenport M, Kelly DA (2009) Biliary atresia. Lancet 374:1704–1713CrossRefPubMed

19.

Hyams JS, Glaser JH, Leichtner AM et al (1985) Discordance for biliary atresia in two sets of monozygotic twins. J Pediatr 107:420–422CrossRefPubMed

20.

Jenner RE, Howard ER (1975) Unsaturated monohydroxy bile acids as a cause of idiopathic obstructive cholangiopathy. Lancet 2:1073–1075CrossRefPubMed

21.

Jiang CB, Lee HC, Yeung CY et al (2003) A scoring system to predict the need for liver transplantation for biliary atresia after Kasai portoenterostomy. Eur J Pediatr 162:603–606CrossRefPubMed

22.

Kelly DA, Davenport M (2007) Current management of biliary atresia. Arch Dis Child 92:1132–1135CrossRefPubMed

23.

Kulkarni PB, Beatty E Jr (1977) Cholangiocarcinoma associated with biliary cirrhosis due to congenital biliary atresia. Am J Dis Child 131:442–444PubMed

24.

Kyoden Y, Tamura S, Sugawara Y et al (2008) Outcome of living donor liver transplantation for post-Kasai biliary atresia in adults. Liver Transpl 14:186–192CrossRefPubMed

25.

Lee HJ, Lee SM, Park WH et al (2003) Objective criteria of triangular cord sign in biliary atresia on US scans. Radiology 229:395–400CrossRefPubMed

26.

Lykavieris P, Chardot C, Sokhn M et al (2005) Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver. Hepatology 41:366–371CrossRefPubMed

27.

McKiernan PJ, Baker AJ, Kelly DA (2000) The frequency and outcome of biliary atresia in the UK and Ireland. Lancet 355:25–29CrossRefPubMed

28.

McKiernan PJ, Baker AJ, Lloyd C et al (2009) British paediatric surveillance unit study of biliary atresia: outcome at 13 years. J Pediatr Gastroenterol Nutr 48:78–81CrossRefPubMed

29.

Peeters PM, Sieders E, De Jong KP et al (2001) Comparison of outcome after pediatric liver transplantation for metabolic diseases and biliary atresia. Eur J Pediatr Surg 11:28–35CrossRefPubMed

30.

Serinet MO, Wildhaber BE, Broué P et al (2009) Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening. Pediatrics 123:1280–1286CrossRefPubMed

31.

Sieders E, Peeters PM, TenVergert EM et al (2001) Retransplantation of the liver in children. Transplantation 71:90–95CrossRefPubMed

32.

Stringer MD, Howard ER, Mowat AP (1989) Endoscopic sclerotherapy in the management of esophageal varices in 61 children with biliary atresia. J Pediatr Surg 24:438–442CrossRefPubMed

33.

Takahashi Y, Nishimoto Y, Matsuura T et al (2009) Surgical complications after living donor liver transplantation in patients with biliary atresia: a relatively high incidence of portal vein complications. Pediatr Surg Int 25:745–751CrossRefPubMed

34.

Tan CE, Driver M, Howard ER et al (1994) Extrahepatic biliary atresia: a first-trimester event? Clues from light microscopy and immunohistochemistry. J Pediatr Surg 29:808–814CrossRefPubMed

35.

Tatekawa Y, Asonuma K, Uemoto S et al (2001) Liver transplantation for biliary atresia associated with malignant hepatic tumors. J Pediatr Surg 36:436–439CrossRefPubMed

36.

Utterson EC, Shepherd RW, Sokol RJ et al (2005) Split Research Group: Biliary atresia: clinical profiles, risk factors, and outcomes of 755 patients listed for liver transplantation. J Pediatr 147:180–185CrossRefPubMed

37.

Viner RM, Forton JT, Cole TJ et al (1999) Growth of long-term survivors of liver transplantation. Arch Dis Child 80:235–240CrossRefPubMed

38.

Wong KK, Chung PH, Chan KL et al (2008) Should open Kasai portoenterostomy be performed for biliary atresia in the era of laparoscopy? Pediatr Surg Int 24:931–933CrossRefPubMed

Title: Clinical practice:
Management of biliary atresia
Authors: Basem A. Khalil
M. Thamara P. R. Perera
Darius F. Mirza
Publication date: 01-04-2010
Publisher: Springer-Verlag
Published in: European Journal of Pediatrics / Issue 4/2010
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI: https://doi.org/10.1007/s00431-009-1125-7

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Springer Medicine

Clinical practice:

Management of biliary atresia

Abstract

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Springer Medicine

Abstract

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