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Biliary atresia

Authors
Jessi Erlichman, MPH
Kathleen M Loomes, MD
Section Editor
Elizabeth B Rand, MD
Deputy Editor
Alison G Hoppin, MD

INTRODUCTION

Biliary atresia (BA) is a progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction exclusively in the neonatal period [1]. Although the overall incidence is low (about one in 10,000 to 20,000 live births [2-5]), BA is the most common cause of neonatal jaundice for which surgery is indicated and the most common indication for liver transplantation in children.

TYPES OF BILIARY ATRESIA

Infants with biliary atresia (BA) can be grouped into three categories:

Biliary atresia without any other anomalies or malformations – This pattern is sometimes referred to as perinatal BA, and occurs in 70 to 85 percent of infants with BA [1,6,7]. Typically, these children are born without jaundice, but within the first two months of life, jaundice develops and stools become progressively acholic.

Biliary atresia in association with laterality malformations – This pattern is also known as Biliary Atresia Splenic Malformation (BASM) or "embryonal" biliary atresia, and occurs 10 to 15 percent of infants with BA [6-9]. The laterality malformations include situs inversus, asplenia or polysplenia, malrotation, interrupted inferior vena cava, and cardiac anomalies. Data suggest that children with BASM have poorer outcomes compared to those with perinatal BA, possibly due to the associated cardiac abnormalities [8-10].

Biliary atresia in association with other congenital malformations – This occurs in the remaining 5 to 10 percent of BA cases; associated congenital malformations include intestinal atresia, imperforate anus, kidney anomalies, and/or heart malformations [7,11,12].

                            

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Literature review current through: Nov 2016. | This topic last updated: Mon Sep 12 00:00:00 GMT+00:00 2016.
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References
Top
  1. Haber BA, Russo P. Biliary atresia. Gastroenterol Clin North Am 2003; 32:891.
  2. Danks DM, Campbell PE, Jack I, et al. Studies of the aetiology of neonatal hepatitis and biliary atresia. Arch Dis Child 1977; 52:360.
  3. Matsui A, Ishikawa T. Identification of infants with biliary atresia in Japan. Lancet 1994; 343:925.
  4. McKiernan PJ, Baker AJ, Kelly DA. The frequency and outcome of biliary atresia in the UK and Ireland. Lancet 2000; 355:25.
  5. Yoon PW, Bresee JS, Olney RS, et al. Epidemiology of biliary atresia: a population-based study. Pediatrics 1997; 99:376.
  6. Shneider BL, Brown MB, Haber B, et al. A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J Pediatr 2006; 148:467.
  7. Schwarz KB, Haber BH, Rosenthal P, et al. Extrahepatic anomalies in infants with biliary atresia: results of a large prospective North American multicenter study. Hepatology 2013; 58:1724.
  8. Davenport M, Savage M, Mowat AP, Howard ER. Biliary atresia splenic malformation syndrome: an etiologic and prognostic subgroup. Surgery 1993; 113:662.
  9. Davenport M, Tizzard SA, Underhill J, et al. The biliary atresia splenic malformation syndrome: a 28-year single-center retrospective study. J Pediatr 2006; 149:393.
  10. Nio M, Wada M, Sasaki H, et al. Long-term outcomes of biliary atresia with splenic malformation. J Pediatr Surg 2015; 50:2124.
  11. De Matos V, Erlichman J, Russo PA, Haber BA. Does "cystic" biliary atresia represent a distinct clinical and etiological subgroup? A series of three cases. Pediatr Dev Pathol 2005; 8:725.
  12. Muise AM, Turner D, Wine E, et al. Biliary atresia with choledochal cyst: implications for classification. Clin Gastroenterol Hepatol 2006; 4:1411.
  13. Caton AR, Druschel CM, McNutt LA. The epidemiology of extrahepatic biliary atresia in New York State, 1983-98. Paediatr Perinat Epidemiol 2004; 18:97.
  14. Bates MD, Bucuvalas JC, Alonso MH, Ryckman FC. Biliary atresia: pathogenesis and treatment. Semin Liver Dis 1998; 18:281.
  15. Hart MH, Kaufman SS, Vanderhoof JA, et al. Neonatal hepatitis and extrahepatic biliary atresia associated with cytomegalovirus infection in twins. Am J Dis Child 1991; 145:302.
  16. Shivakumar P, Campbell KM, Sabla GE, et al. Obstruction of extrahepatic bile ducts by lymphocytes is regulated by IFN-gamma in experimental biliary atresia. J Clin Invest 2004; 114:322.
  17. Tarr PI, Haas JE, Christie DL. Biliary atresia, cytomegalovirus, and age at referral. Pediatrics 1996; 97:828.
  18. Harper P, Plant JW, Unger DB. Congenital biliary atresia and jaundice in lambs and calves. Aust Vet J 1990; 67:18.
  19. Lorent K, Gong W, Koo KA, et al. Identification of a plant isoflavonoid that causes biliary atresia. Sci Transl Med 2015; 7:286ra67.
  20. Davit-Spraul A, Baussan C, Hermeziu B, et al. CFC1 gene involvement in biliary atresia with polysplenia syndrome. J Pediatr Gastroenterol Nutr 2008; 46:111.
  21. Mazziotti MV, Willis LK, Heuckeroth RO, et al. Anomalous development of the hepatobiliary system in the Inv mouse. Hepatology 1999; 30:372.
  22. Tsai EA, Grochowski CM, Falsey AM, et al. Heterozygous deletion of FOXA2 segregates with disease in a family with heterotaxy, panhypopituitarism, and biliary atresia. Hum Mutat 2015; 36:631.
  23. Garcia-Barceló MM, Yeung MY, Miao XP, et al. Genome-wide association study identifies a susceptibility locus for biliary atresia on 10q24.2. Hum Mol Genet 2010; 19:2917.
  24. Leyva-Vega M, Gerfen J, Thiel BD, et al. Genomic alterations in biliary atresia suggest region of potential disease susceptibility in 2q37.3. Am J Med Genet A 2010; 152A:886.
  25. Kohsaka T, Yuan ZR, Guo SX, et al. The significance of human jagged 1 mutations detected in severe cases of extrahepatic biliary atresia. Hepatology 2002; 36:904.
  26. Matthews RP, Eauclaire SF, Mugnier M, et al. DNA hypomethylation causes bile duct defects in zebrafish and is a distinguishing feature of infantile biliary atresia. Hepatology 2011; 53:905.
  27. Zahm A, Hand NJ, Horner A, et al. Serum microRNA is a novel biomarker of biliary atresia. Hepatology 2011; 54 (4) Suppl:411A.
  28. Muraji T, Hosaka N, Irie N, et al. Maternal microchimerism in underlying pathogenesis of biliary atresia: quantification and phenotypes of maternal cells in the liver. Pediatrics 2008; 121:517.
  29. Bezerra JA, Tiao G, Ryckman FC, et al. Genetic induction of proinflammatory immunity in children with biliary atresia. Lancet 2002; 360:1653.
  30. Shih HH, Lin TM, Chuang JH, et al. Promoter polymorphism of the CD14 endotoxin receptor gene is associated with biliary atresia and idiopathic neonatal cholestasis. Pediatrics 2005; 116:437.
  31. Franciscovich A, Vaidya D, Doyle J, et al. PoopMD, a Mobile Health Application, Accurately Identifies Infant Acholic Stools. PLoS One 2015; 10:e0132270.
  32. Morinville V, Ahmed N, Ibberson C, et al. Home-Based Screening for Biliary Atresia Using Infant Stool Color Cards in Canada: Quebec Feasibility Study. J Pediatr Gastroenterol Nutr 2016; 62:536.
  33. Gu YH, Yokoyama K, Mizuta K, et al. Stool color card screening for early detection of biliary atresia and long-term native liver survival: a 19-year cohort study in Japan. J Pediatr 2015; 166:897.
  34. Harpavat S, Finegold MJ, Karpen SJ. Patients with biliary atresia have elevated direct/conjugated bilirubin levels shortly after birth. Pediatrics 2011; 128:e1428.
  35. Wang KS, Section on Surgery, Committee on Fetus and Newborn, Childhood Liver Disease Research Network. Newborn Screening for Biliary Atresia. Pediatrics 2015; 136:e1663.
  36. Harpavat S, Garcia-Prats JA, Shneider BL. Newborn Bilirubin Screening for Biliary Atresia. N Engl J Med 2016; 375:605.
  37. Serinet MO, Wildhaber BE, Broué P, et al. Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening. Pediatrics 2009; 123:1280.
  38. Humphrey TM, Stringer MD. Biliary atresia: US diagnosis. Radiology 2007; 244:845.
  39. Lee MS, Kim MJ, Lee MJ, et al. Biliary atresia: color doppler US findings in neonates and infants. Radiology 2009; 252:282.
  40. Takamizawa S, Zaima A, Muraji T, et al. Can biliary atresia be diagnosed by ultrasonography alone? J Pediatr Surg 2007; 42:2093.
  41. Mittal V, Saxena AK, Sodhi KS, et al. Role of abdominal sonography in the preoperative diagnosis of extrahepatic biliary atresia in infants younger than 90 days. AJR Am J Roentgenol 2011; 196:W438.
  42. Zhou L, Shan Q, Tian W, et al. Ultrasound for the Diagnosis of Biliary Atresia: A Meta-Analysis. AJR Am J Roentgenol 2016; 206:W73.
  43. Park WH, Choi SO, Lee HJ, et al. A new diagnostic approach to biliary atresia with emphasis on the ultrasonographic triangular cord sign: comparison of ultrasonography, hepatobiliary scintigraphy, and liver needle biopsy in the evaluation of infantile cholestasis. J Pediatr Surg 1997; 32:1555.
  44. Azar G, Beneck D, Lane B, et al. Atypical morphologic presentation of biliary atresia and value of serial liver biopsies. J Pediatr Gastroenterol Nutr 2002; 34:212.
  45. Moyer V, Freese DK, Whitington PF, et al. Guideline for the evaluation of cholestatic jaundice in infants: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr 2004; 39:115.
  46. Lee SY, Kim GC, Choe BH, et al. Efficacy of US-guided percutaneous cholecystocholangiography for the early exclusion and type determination of biliary atresia. Radiology 2011; 261:916.
  47. Jensen MK, Biank VF, Moe DC, et al. HIDA, percutaneous transhepatic cholecysto-cholangiography and liver biopsy in infants with persistent jaundice: can a combination of PTCC and liver biopsy reduce unnecessary laparotomy? Pediatr Radiol 2012; 42:32.
  48. Davenport M, Ure BM, Petersen C, Kobayashi H. Surgery for biliary atresia--is there a European consensus? Eur J Pediatr Surg 2007; 17:180.
  49. Haber BA, Erlichman J, Thayu M, et al. Successful revision of portoenterostomy in an infant with biliary atresia. J Pediatr Surg 2006; 41:e1.
  50. Bondoc AJ, Taylor JA, Alonso MH, et al. The beneficial impact of revision of Kasai portoenterostomy for biliary atresia: an institutional study. Ann Surg 2012; 255:570.
  51. Schreiber RA, Barker CC, Roberts EA, et al. Biliary atresia: the Canadian experience. J Pediatr 2007; 151:659.
  52. HAYS DM, SNYDER WH Jr. LIFE-SPAN IN UNTREATED BILIARY ATRESIA. Surgery 1963; 54:373.
  53. Oh M, Hobeldin M, Chen T, et al. The Kasai procedure in the treatment of biliary atresia. J Pediatr Surg 1995; 30:1077.
  54. Haber BA, Erlichman J, Loomes KM. Recent advances in biliary atresia: prospects for novel therapies. Expert Opin Investig Drugs 2008; 17:1911.
  55. Lindor KD, Kowdley KV, Luketic VA, et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology 2009; 50:808.
  56. Meyers RL, Book LS, O'Gorman MA, et al. High-dose steroids, ursodeoxycholic acid, and chronic intravenous antibiotics improve bile flow after Kasai procedure in infants with biliary atresia. J Pediatr Surg 2003; 38:406.
  57. Stringer MD, Davison SM, Rajwal SR, McClean P. Kasai portoenterostomy: 12-year experience with a novel adjuvant therapy regimen. J Pediatr Surg 2007; 42:1324.
  58. Squires RH, Ng V, Romero R, et al. Evaluation of the pediatric patient for liver transplantation: 2014 practice guideline by the American Association for the Study of Liver Diseases, American Society of Transplantation and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Hepatology 2014; 60:362.
  59. Bezerra JA, Spino C, Magee JC, et al. Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial. JAMA 2014; 311:1750.
  60. Feranchak AP, Sokol R. Medical and nutritional management of cholestasis in infants and children. In: Liver Disease in Children, Suchy FJ, Sokol R, Balistreri W (Eds), Cambridge University Press, New York 2007. p.190.
  61. Novy MA, Schwarz KB. Nutritional considerations and management of the child with liver disease. Nutrition 1997; 13:177.
  62. Pierro A, Koletzko B, Carnielli V, et al. Resting energy expenditure is increased in infants and children with extrahepatic biliary atresia. J Pediatr Surg 1989; 24:534.
  63. Andrews WS, Pau CM, Chase HP, et al. Fat soluble vitamin deficiency in biliary atresia. J Pediatr Surg 1981; 16:284.
  64. Shneider B, Abel R, Raghunathan T. A prospective multi-centered investigation of vitamin supplementation in infants with biliary atresia: Interim analysis from the biliary atresia research consortium (BARC). Hepatology 2009; 50.
  65. Shneider BL, Magee JC, Bezerra JA, et al. Efficacy of fat-soluble vitamin supplementation in infants with biliary atresia. Pediatrics 2012; 130:e607.
  66. Ng J, Paul A, Wright N, et al. Vitamin D Levels in Infants With Biliary Atresia: Pre- and Post-Kasai Portoenterostomy. J Pediatr Gastroenterol Nutr 2016; 62:746.
  67. Luo Y, Zheng S. Current concept about postoperative cholangitis in biliary atresia. World J Pediatr 2008; 4:14.
  68. Wu ET, Chen HL, Ni YH, et al. Bacterial cholangitis in patients with biliary atresia: impact on short-term outcome. Pediatr Surg Int 2001; 17:390.
  69. Bu LN, Chen HL, Chang CJ, et al. Prophylactic oral antibiotics in prevention of recurrent cholangitis after the Kasai portoenterostomy. J Pediatr Surg 2003; 38:590.
  70. Ernest van Heurn LW, Saing H, Tam PK. Cholangitis after hepatic portoenterostomy for biliary atresia: a multivariate analysis of risk factors. J Pediatr 2003; 142:566.
  71. Lien TH, Bu LN, Wu JF, et al. Use of Lactobacillus casei rhamnosus to Prevent Cholangitis in Biliary Atresia After Kasai Operation. J Pediatr Gastroenterol Nutr 2015; 60:654.
  72. Shneider BL, Abel B, Haber B, et al. Portal hypertension in children and young adults with biliary atresia. J Pediatr Gastroenterol Nutr 2012; 55:567.
  73. Lykavieris P, Chardot C, Sokhn M, et al. Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver. Hepatology 2005; 41:366.
  74. Miga D, Sokol RJ, Mackenzie T, et al. Survival after first esophageal variceal hemorrhage in patients with biliary atresia. J Pediatr 2001; 139:291.
  75. Lampela H, Kosola S, Koivusalo A, et al. Endoscopic surveillance and primary prophylaxis sclerotherapy of esophageal varices in biliary atresia. J Pediatr Gastroenterol Nutr 2012; 55:574.
  76. Shneider BL, Bosch J, de Franchis R, et al. Portal hypertension in children: expert pediatric opinion on the report of the Baveno v Consensus Workshop on Methodology of Diagnosis and Therapy in Portal Hypertension. Pediatr Transplant 2012; 16:426.
  77. Jiang CB, Lee HC, Yeung CY, et al. A scoring system to predict the need for liver transplantation for biliary atresia after Kasai portoenterostomy. Eur J Pediatr 2003; 162:603.
  78. Barshes NR, Lee TC, Balkrishnan R, et al. Orthotopic liver transplantation for biliary atresia: the U.S. experience. Liver Transpl 2005; 11:1193.
  79. Okamoto T, Yokoi A, Okamoto S, et al. Pretransplant risk factors and optimal timing for living-related liver transplantation in biliary atresia: experience of one Japanese children's hospital and transplantation center. J Pediatr Surg 2008; 43:489.
  80. Chardot C, Carton M, Spire-Bendelac N, et al. Is the Kasai operation still indicated in children older than 3 months diagnosed with biliary atresia? J Pediatr 2001; 138:224.
  81. Nio M, Ohi R, Miyano T, et al. Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry. J Pediatr Surg 2003; 38:997.
  82. Fouquet V, Alves A, Branchereau S, et al. Long-term outcome of pediatric liver transplantation for biliary atresia: a 10-year follow-up in a single center. Liver Transpl 2005; 11:152.
  83. Wildhaber BE, Coran AG, Drongowski RA, et al. The Kasai portoenterostomy for biliary atresia: A review of a 27-year experience with 81 patients. J Pediatr Surg 2003; 38:1480.
  84. 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; 147:180.
  85. Wildhaber BE, Majno P, Mayr J, et al. Biliary atresia: Swiss national study, 1994-2004. J Pediatr Gastroenterol Nutr 2008; 46:299.
  86. Chardot C, Buet C, Serinet MO, et al. Improving outcomes of biliary atresia: French national series 1986-2009. J Hepatol 2013; 58:1209.
  87. Arnon R, Annunziato RA, D'Amelio G, et al. Liver Transplantation for Biliary Atresia: Is There a Difference in Outcome for Infants? J Pediatr Gastroenterol Nutr 2016; 62:220.
  88. Chardot C, Carton M, Spire-Bendelac N, et al. Prognosis of biliary atresia in the era of liver transplantation: French national study from 1986 to 1996. Hepatology 1999; 30:606.
  89. Laurent J, Gauthier F, Bernard O, et al. Long-term outcome after surgery for biliary atresia. Study of 40 patients surviving for more than 10 years. Gastroenterology 1990; 99:1793.
  90. Davenport M, Puricelli V, Farrant P, et al. The outcome of the older (> or =100 days) infant with biliary atresia. J Pediatr Surg 2004; 39:575.
  91. Karrer FM, Price MR, Bensard DD, et al. Long-term results with the Kasai operation for biliary atresia. Arch Surg 1996; 131:493.
  92. Tagge DU, Tagge EP, Drongowski RA, et al. A long-term experience with biliary atresia. Reassessment of prognostic factors. Ann Surg 1991; 214:590.
  93. Shinkai M, Ohhama Y, Take H, et al. Long-term outcome of children with biliary atresia who were not transplanted after the Kasai operation: >20-year experience at a children's hospital. J Pediatr Gastroenterol Nutr 2009; 48:443.
  94. de Vries W, Homan-Van der Veen J, Hulscher JB, et al. Twenty-year transplant-free survival rate among patients with biliary atresia. Clin Gastroenterol Hepatol 2011; 9:1086.
  95. Lien TH, Chang MH, Wu JF, et al. Effects of the infant stool color card screening program on 5-year outcome of biliary atresia in Taiwan. Hepatology 2011; 53:202.
  96. Wong KK, Chung PH, Chan IH, et al. Performing Kasai portoenterostomy beyond 60 days of life is not necessarily associated with a worse outcome. J Pediatr Gastroenterol Nutr 2010; 51:631.
  97. Davenport M, De Ville de Goyet J, Stringer MD, et al. Seamless management of biliary atresia in England and Wales (1999-2002). Lancet 2004; 363:1354.
  98. 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; 254:577.
  99. 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; 170:211.