About this Journal Submit a Manuscript Table of Contents 
ISRN Surgery
Volume 2012 (2012), Article ID 132089, 15 pages
doi:10.5402/2012/132089
Review Article

Biliary Atresia: 50 Years after the First Kasai

Barbara E. Wildhaber

Division of Pediatric Surgery, Department of Pediatrics, University Hospital of Geneva, 1211 Geneva, Switzerland

Received 23 September 2012; Accepted 30 October 2012

Academic Editors: D. W. Blackhurst, J. C. Cendan, D. Laub, and D. E. Ziogas

Copyright © 2012 Barbara E. Wildhaber. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Linked References

  1. M. Davenport, J. A. Bezerra, and R. J. Sokol, “A challenge on the use of the words Embryonic and Perinatal in the context of biliary atresia,” Hepatology, vol. 41, no. 2, pp. 403–405, 2005. View at Publisher · View at Google Scholar · View at Scopus
  2. M. Davenport, S. A. Tizzard, J. Underhill, G. Mieli-Vergani, B. Portmann, and N. Hadžić, “The biliary atresia splenic malformation syndrome: a 28-year single-center retrospective study,” Journal of Pediatrics, vol. 149, no. 3, pp. 393–400, 2006. View at Publisher · View at Google Scholar · View at Scopus
  3. C. Chardot, M. Carton, N. Spire-Bendelac, C. Le Pommelet, J. L. Golmard, and B. Auvert, “Prognosis of biliary atresia in the era of liver transplantation: French National Study from 1986 to 1996,” Hepatology, vol. 30, no. 3, pp. 606–611, 1999. View at Publisher · View at Google Scholar · View at Scopus
  4. C. Chardot, M. Carton, N. Spire-Bendelac, C. L. Pommelet, J. L. Golmard, and B. Auvert, “Epidemiology of biliary atresia in France: a national study 1986–1996,” Journal of Hepatology, vol. 31, no. 6, pp. 1006–1013, 1999. View at Publisher · View at Google Scholar · View at Scopus
  5. N. T. Henriksen, P. A. Drablos, and O. Aagenaes, “Cholestatic jaundice in infancy. The importance of familial and genetic factors in aetiology and prognosis,” Archives of Disease in Childhood, vol. 56, no. 8, pp. 622–627, 1981. View at Scopus
  6. B. Fischler, B. Haglund, and A. Hjern, “A population-based study on the incidence and possible pre- and perinatal etiologic risk factors of biliary atresia,” Journal of Pediatrics, vol. 141, no. 2, pp. 217–222, 2002. View at Publisher · View at Google Scholar · View at Scopus
  7. R. H. J. Houwen, I. I. A. Kerremans, H. A. Van Steensel-Moll, L. K. J. Van Romunde, C. M. A. Bijleveld, and P. Schweizer, “Time-space distribution of extrahepatic biliary atresia in the Netherlands and West Germany,” Zeitschrift für Kinderchirurgie, vol. 43, no. 2, pp. 68–71, 1988. View at Scopus
  8. P. J. McKiernan, A. J. Baker, and D. A. Kelly, “The frequency and outcome of biliary atresia in the UK and Ireland,” Lancet, vol. 355, no. 9197, pp. 25–29, 2000. View at Publisher · View at Google Scholar · View at Scopus
  9. D. M. Danks, P. E. Campbell, and I. Jack, “Studies of the aetiology of neonatal hepatitis and biliary atresia,” Archives of Disease in Childhood, vol. 52, no. 5, pp. 360–367, 1977. View at Scopus
  10. W. K. Shim, M. Kasai, and M. A. Spence, “Racial influence on the incidence of biliary atresia,” Progress in Pediatric Surgery, vol. 6, pp. 53–62, 1974. View at Scopus
  11. M. Nio, R. Ohi, T. Miyano, M. Saeki, K. Shiraki, and K. Tanaka, “Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry,” Journal of Pediatric Surgery, vol. 38, no. 7, pp. 997–1000, 2003. View at Publisher · View at Google Scholar · View at Scopus
  12. S. M. Chen, M. H. Chang, J. C. Du et al., “Screening for biliary atresia by infant stool color card in Taiwan,” Pediatrics, vol. 117, no. 4, pp. 1147–1154, 2006. View at Publisher · View at Google Scholar · View at Scopus
  13. A. D. Strickland and K. Shannon, “Studies in the etiology of extrahepatic biliary atresia: time-space clustering,” Journal of Pediatrics, vol. 100, no. 5, pp. 749–753, 1982. View at Scopus
  14. P. W. Yoon, J. S. Bresee, R. S. Olney, L. M. James, and M. J. Khoury, “Epidemiology of biliary atresia: a population-based study,” Pediatrics, vol. 99, no. 3, pp. 376–382, 1997. View at Publisher · View at Google Scholar · View at Scopus
  15. M. F. Ayas, A. C. Hillemeier, and A. D. Olson, “Lack of evidence for seasonal variation in extrahepatic biliary atresia during infancy,” Journal of Clinical Gastroenterology, vol. 22, no. 4, pp. 292–294, 1996. View at Publisher · View at Google Scholar · View at Scopus
  16. M. Davenport, A. Dhawan, P. Yoon, J. S. Bresee, R. S. Olney, and M. J. Khoury, “Epidemiologic study of infants with biliary atresia,” Pediatrics, vol. 101, no. 4 I, pp. 729–730, 1998. View at Publisher · View at Google Scholar · View at Scopus
  17. R. Hinds, M. Davenport, G. Mieli-Vergani, and N. Hadžić, “Antenatal presentation of biliary atresia,” Journal of Pediatrics, vol. 144, no. 1, pp. 43–46, 2004. View at Publisher · View at Google Scholar · View at Scopus
  18. F. Muller, F. Gauthier, J. Laurent, M. Schmitt, and J. Boue, “Amniotic fluid GGT and congenital extrahepatic biliary damage,” Lancet, vol. 337, no. 8735, pp. 232–233, 1991. View at Scopus
  19. R. Drut, R. M. Drut, M. A. Gómez, E. C. Rúa, and M. M. Lojo, “Presence of human papillomavirus in extrahepatic biliary atresia,” Journal of Pediatric Gastroenterology and Nutrition, vol. 27, no. 5, pp. 530–535, 1998. View at Publisher · View at Google Scholar · View at Scopus
  20. D. Nadal, W. Wunderli, O. Meurmann, J. Briner, and H. Hirsig, “Isolation of respiratory syncytial virus from liver tissue and extrahepatic biliary atresia material,” Scandinavian Journal of Infectious Diseases, vol. 22, no. 1, pp. 91–93, 1990. View at Scopus
  21. R. Domiati-Saad, D. Brian Dawson, L. R. Margraf, M. J. Finegold, A. G. Weinberg, and B. B. Rogers, “Cytomegalovirus and human herpesvirus 6, but not human papillomavirus, are present in neonatal giant cell and extrahepatic biliary atresia,” Pediatric and Developmental Pathology, vol. 3, no. 4, pp. 367–373, 2000. View at Publisher · View at Google Scholar · View at Scopus
  22. B. Fischler, A. Ehrnst, M. Forsgren, C. Örvell, and A. Nemeth, “The viral association of neonatal cholestasis in Sweden: a possible link between cytomegalovirus infection and extrahepatic biliary atresia,” Journal of Pediatric Gastroenterology and Nutrition, vol. 27, no. 1, pp. 57–64, 1998. View at Publisher · View at Google Scholar · View at Scopus
  23. R. Morecki, J. H. Glaser, and S. Cho, “Biliary atresia and reovirus type 3 infection,” New England Journal of Medicine, vol. 307, no. 8, pp. 481–484, 1982. View at Scopus
  24. M. Riepenhoff-Talty, V. Gouvea, M. J. Evans et al., “Detection of group C rotavirus in infants with extrahepatic biliary atresia,” Journal of Infectious Diseases, vol. 174, no. 1, pp. 8–15, 1996. View at Scopus
  25. L. T. Weaver, R. Nelson, and T. M. Bell, “The association of extrahepatic bile duct atresia and neonatal Epstein-Barr virus infection,” Acta Paediatrica Scandinavica, vol. 73, no. 1, pp. 155–157, 1984. View at Scopus
  26. R. B. Fjær, A. L. Bruu, and S. A. Nordbø, “Extrahepatic bile duct atresia and viral involvement,” Pediatric Transplantation, vol. 9, no. 1, pp. 68–73, 2005. View at Publisher · View at Google Scholar · View at Scopus
  27. B. Fischler, S. Woxenius, A. Nemeth, and N. Papadogiannakis, “Immunoglobulin deposits in liver tissue from infants with biliary atresia and the correlation to cytomegalovirus infection,” Journal of Pediatric Surgery, vol. 40, no. 3, pp. 541–546, 2005. View at Publisher · View at Google Scholar · View at Scopus
  28. R. J. Sokol and C. Mack, “Etiopathogenesis of biliary atresia,” Seminars in Liver Disease, vol. 21, no. 4, pp. 517–524, 2001. View at Publisher · View at Google Scholar · View at Scopus
  29. C. L. Mack, R. M. Tucker, B. R. Lu et al., “Cellular and humoral autoimmunity directed at bile duct epithelia in murine biliary atresia,” Hepatology, vol. 44, no. 5, pp. 1231–1239, 2006. View at Publisher · View at Google Scholar · View at Scopus
  30. S. Rauschenfels, M. Krassmann, A. N. Al-Masri et al., “Incidence of hepatotropic viruses in biliary atresia,” European Journal of Pediatrics, vol. 168, no. 4, pp. 469–476, 2009. View at Publisher · View at Google Scholar · View at Scopus
  31. K. Ando, T. Miyano, T. Fujimoto et al., “Sibling occurrence of biliary atresia and biliary dilatation,” Journal of Pediatric Surgery, vol. 31, no. 9, pp. 1302–1304, 1996. View at Publisher · View at Google Scholar · View at Scopus
  32. C. Danesino, E. Spadoni, and A. Buzzi, “Familial biliary atresia,” American Journal of Medical Genetics, vol. 85, no. 2, p. 195, 1999.
  33. M. L. Cunningham and V. P. Sybert, “Idiopathic extrahepatic biliary atresia: recurrence in sibs in two families,” American Journal of Medical Genetics, vol. 31, no. 2, pp. 421–426, 1988. View at Scopus
  34. J. S. Hyams, J. H. Glaser, A. M. Leichtner, and R. Morecki, “Discordance for biliary atresia in two sets of monozygotic twins,” Journal of Pediatrics, vol. 107, no. 3, pp. 420–422, 1985. View at Scopus
  35. Y. Poovorawan, V. Chongsrisawat, C. Tanunytthawongse, T. Norapaksunthorn, A. Mutirangura, and B. Chandrakamol, “Extrahepatic biliary atresia in twins: zygosity determination by short tandem repeat loci,” Journal of the Medical Association of Thailand, vol. 79, pp. S119–124, 1996. View at Scopus
  36. A. D. Strickland, K. Shannon, and C. D. Coln, “Biliary atresia in two sets of twins,” Journal of Pediatrics, vol. 107, no. 3, pp. 418–420, 1985. View at Scopus
  37. A. Davit-Spraul, C. Baussan, B. Hermeziu, O. Bernard, and E. Jacquemin, “CFC1 gene involvement in biliary atresia with polysplenia syndrome,” Journal of Pediatric Gastroenterology and Nutrition, vol. 46, no. 1, pp. 111–112, 2008. View at Publisher · View at Google Scholar · View at Scopus
  38. C. Coffinier, L. Gresh, L. Fiette et al., “Bile system morphogenesis defects and liver dysfunction upon targeted deletion of HNF1β,” Development, vol. 129, no. 8, pp. 1829–1838, 2002. View at Scopus
  39. R. Redkar, M. Davenport, and E. R. Howard, “Antenatal diagnosis of congenital anomalies of the biliary tract,” Journal of Pediatric Surgery, vol. 33, no. 5, pp. 700–704, 1998. View at Publisher · View at Google Scholar · View at Scopus
  40. K. M. Emerick and P. F. Whitington, “Neonatal liver disease,” Pediatric Annals, vol. 35, no. 4, pp. 280–286, 2006. View at Scopus
  41. O. Bernard, “Plea for early diagnosis of biliary tract atresia. Twelve errors to avoid,” Archives of Pediatrics, vol. 2, no. 10, pp. 937–939, 1995. View at Publisher · View at Google Scholar
  42. G. Mieli-Vergani, B. Portman, E. R. Howard, and A. P. Mowat, “Late referral for biliary atresia—missed opportunities for effective surgery,” Lancet, vol. 1, no. 8635, pp. 421–423, 1989. View at Scopus
  43. K. Oldham, P. Colombani, R. Foglia, et al., Principles and Practice of Pediatric Surgery, Lippincott Williams & Wilkins, 2005.
  44. S. X. Li, Y. Zhang, M. Sun et al., “Ultrasonic diagnosis of biliary atresia: a retrospective analysis of 20 patients,” World Journal of Gastroenterology, vol. 14, no. 22, pp. 3579–3582, 2008. View at Publisher · View at Google Scholar · View at Scopus
  45. J. Takaya, S. Nakano, Y. Imai, Y. Fujii, and K. Kaneko, “Usefulness of magnetic resonance cholangiopancreatography in biliary structures in infants: a four-case report,” European Journal of Pediatrics, vol. 166, no. 3, pp. 211–214, 2007. View at Publisher · View at Google Scholar · View at Scopus
  46. S. J. Han, M. J. Kim, A. Han et al., “Magnetic resonance cholangiography for the diagnosis of biliary atresia,” Journal of Pediatric Surgery, vol. 37, no. 4, pp. 599–604, 2002. View at Publisher · View at Google Scholar · View at Scopus
  47. B. C. Nwomeh, D. A. Caniano, and M. Hogan, “Definitive exclusion of biliary atresia in infants with cholestatic jaundice: the role of percutaneous cholecysto-cholangiography,” Pediatric Surgery International, vol. 23, no. 9, pp. 845–849, 2007. View at Publisher · View at Google Scholar · View at Scopus
  48. T. Okazaki, G. Miyano, A. Yamataka et al., “Diagnostic laparoscopy-assisted cholangiography in infants with prolonged jaundice,” Pediatric Surgery International, vol. 22, no. 2, pp. 140–143, 2006. View at Publisher · View at Google Scholar · View at Scopus
  49. Y. Iinuma, R. Narisawa, M. Iwafuchi et al., “The role of endoscopic retrograde cholangiopancreatography in infants with cholestasis,” Journal of Pediatric Surgery, vol. 35, no. 4, pp. 545–549, 2000. View at Scopus
  50. N. Ohnuma, H. Takahashi, M. Tanabe, H. Yoshida, and J. Iwai, “The role of ERCP in biliary atresia,” Gastrointestinal Endoscopy, vol. 45, no. 5, pp. 365–370, 1997. View at Scopus
  51. E. Shteyer, D. Wengrower, I. Benuri-Silbiger, et al., “Endoscopic retrograde cholangiopancreatography in neonatal cholestasis,” Journal of Pediatric Gastroenterology and Nutrition, vol. 55, no. 2, pp. 142–145, 2012.
  52. K. Ashcraft, Pediatric Surgery, Saunders, 3rd edition, 2000.
  53. V. Moyer, D. K. Freese, P. F. Whitington et al., “Guideline for the evaluation of cholestatic jaundice in infants: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition,” Journal of Pediatric Gastroenterology and Nutrition, vol. 39, no. 2, pp. 115–128, 2004. View at Scopus
  54. O. Bernard, “Early diagnosis of neonatal cholestatic jaundice,” Archives de Pediatrie, vol. 5, no. 9, pp. 1031–1035, 1998. View at Publisher · View at Google Scholar · View at Scopus
  55. P. Lykavieris, C. Chardot, M. Sokhn, F. Gauthier, J. Valayer, and O. Bernard, “Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver,” Hepatology, vol. 41, no. 2, pp. 366–371, 2005. View at Publisher · View at Google Scholar · View at Scopus
  56. A. Carceller, H. Blanchard, F. Alvarez, D. St-Vil, A. L. Bensoussan, and M. Di Lorenzo, “Past and future of biliary atresia,” Journal of Pediatric Surgery, vol. 35, no. 5, pp. 717–720, 2000. View at Scopus
  57. M. Kasai and S. Suzuki, “A new operation for, ‘non-correctable’ biliary atresia: hepatic porto-enterostomy,” Shuiyutsu, vol. 13, pp. 733–739, 1959.
  58. M. Davenport, J. De Ville De Goyet, M. D. Stringer et al., “Seamless management of biliary atresia in England and Wales (1999–2002),” Lancet, vol. 363, no. 9418, pp. 1354–1357, 2004. View at Publisher · View at Google Scholar · View at Scopus
  59. J. W. McClement, E. R. Howard, and A. P. Mowat, “Results of surgical treatment for extrahepatic biliary atresia in United Kingdom 1980–1982. Survey conducted on behalf of the British Paediatric Association Gastroenterology Group and the British Association of Paediatric Surgeons,” British Medical Journal, vol. 290, no. 6465, pp. 345–347, 1985. View at Scopus
  60. F. Gauthier, J. L. Luciani, C. Chardot et al., “Determinants of life span after kasai operation at the era of liver transplantation,” Tohoku Journal of Experimental Medicine, vol. 181, no. 1, pp. 97–107, 1997. View at Scopus
  61. M. Davenport, E. Ong, K. Sharif, et al., “Biliary atresia in England and Wales: results of centralization and new benchmark,” Journal of Pediatric Surgery, vol. 46, no. 9, pp. 1689–1694, 2011.
  62. T. Ito, M. Nagaya, and H. Ando, “Modified hepatic portal enterostomy for biliary atresia,” Zeitschrift fur Kinderchirurgie, vol. 39, no. 4, pp. 242–245, 1984. View at Scopus
  63. A. Toyosaka, E. Okamoto, T. Okasora, K. Nose, Y. Tomimoto, and Y. Seki, “Extensive dissection at the porta hepatis for biliary atresia,” Journal of Pediatric Surgery, vol. 29, no. 7, pp. 896–899, 1994. View at Publisher · View at Google Scholar · View at Scopus
  64. M. Endo, K. Katsumata, and J. Yokoyama, “Extended dissection of the portahepatis and creation of an intussuscepted ileocolic conduit for biliary atresia,” Journal of Pediatric Surgery, vol. 18, no. 6, pp. 784–793, 1983. View at Scopus
  65. H. Nakamura, H. Koga, M. Wada, et al., “Reappraising the portoenterostomy procedure according to sound physiologic/anatomic principles enhances postoperative jaundice clearance in biliary atresia,” Pediatric Surgery International, vol. 28, no. 2, pp. 205–209, 2012.
  66. T. Hashimoto, Y. Otobe, Y. Shimizu et al., “A modification of hepatic portoenterostomy (kasai operation) for biliary atresia,” Journal of the American College of Surgeons, vol. 185, no. 6, pp. 548–553, 1997. View at Scopus
  67. H. Kobayashi, A. Yamataka, M. Urao et al., “Innovative modification of the hepatic portoenterostomy. Our experience of treating biliary atresia,” Journal of Pediatric Surgery, vol. 41, no. 5, pp. e19–e22, 2006. View at Publisher · View at Google Scholar · View at Scopus
  68. T. Suzuki, T. Hashimoto, S. Kondo, Y. Sato, and M. H. Hussein, “Evaluating patients' outcome post-Kasai operation: a 19-year experience with modification of the hepatic portoenterostomy and applying a novel steroid therapy regimen,” Pediatric Surgery International, vol. 26, no. 8, pp. 825–830, 2010. View at Publisher · View at Google Scholar · View at Scopus
  69. H. Ando, T. Seo, F. Ito et al., “A new hepatic portoenterostomy with division of the ligamentum venosum for treatment of biliary atresia: a preliminary report,” Journal of Pediatric Surgery, vol. 32, no. 11, pp. 1552–1554, 1997. View at Publisher · View at Google Scholar · View at Scopus
  70. Y. Luo and S. Zheng, “Current concept about postoperative cholangitis in biliary atresia,” World Journal of Pediatrics, vol. 4, no. 1, pp. 14–19, 2008.
  71. K. Lünzmann and P. Schweizer, “The influence of cholangitis on the prognosis of extrahepatic biliary atresia,” European Journal of Pediatric Surgery, vol. 9, no. 1, pp. 19–23, 1999. View at Scopus
  72. N. L. Yanchar, A. M. J. Shapiro, and D. L. Sigalet, “Is early response to portoenterostomy predictive of long-term outcome for patients with biliary atresia?” Journal of Pediatric Surgery, vol. 31, no. 6, pp. 774–778, 1996. View at Publisher · View at Google Scholar · View at Scopus
  73. E. T. Wu, H. L. Chen, Y. H. Ni et al., “Bacterial cholangitis in patients with biliary atresia: impact on short-term outcome,” Pediatric Surgery International, vol. 17, no. 5-6, pp. 390–395, 2001. View at Publisher · View at Google Scholar · View at Scopus
  74. B. E. Wildhaber, A. G. Coran, R. A. Drongowski et al., “The Kasai portoenterostomy for biliary atresia: a review of a 27-year experience with 81 patients,” Journal of Pediatric Surgery, vol. 38, no. 10, pp. 1480–1485, 2003. View at Publisher · View at Google Scholar · View at Scopus
  75. T. Nakajo, K. Hashizume, M. Saeki, and Y. Tsuchida, “Intussusception-type antireflux valve in the Roux-en-Y loop to prevent ascending cholangitis after hepatic portojejunostomy,” Journal of Pediatric Surgery, vol. 25, no. 3, pp. 311–314, 1990. View at Publisher · View at Google Scholar · View at Scopus
  76. B. H. Kaufman, S. R. Luck, and J. G. Raffensperger, “The evolution of a valved hepatoduodenal intestinal conduit,” Journal of Pediatric Surgery, vol. 16, no. 3, pp. 279–283, 1981. View at Scopus
  77. M. Reynolds, S. R. Luck, and J. G. Raffensperger, “The valved conduit prevents ascending cholangitis: a follow-up,” Journal of Pediatric Surgery, vol. 20, no. 6, pp. 696–702, 1985. View at Scopus
  78. M. Saeki, M. Nakano, K. Hagane, and K. Shimizu, “Effectiveness of an intussusceptive antireflux valve to prevent ascending cholangitis after hepatic portojejunostomy in biliary atresia,” Journal of Pediatric Surgery, vol. 26, no. 7, pp. 800–803, 1991. View at Publisher · View at Google Scholar · View at Scopus
  79. Y. Ogasawara, A. Yamataka, K. Tsukamoto et al., “The intussusception antireflux valve is ineffective for preventing cholangitis in biliary atresia: a prospective study,” Journal of Pediatric Surgery, vol. 38, no. 12, pp. 1826–1829, 2003. View at Publisher · View at Google Scholar · View at Scopus
  80. M. Kasai, “Treatment of biliary atresia with special reference to hepatic porto-enterostomy and its modifications,” Progress in Pediatric Surgery, vol. 6, pp. 5–52, 1974. View at Scopus
  81. S. Sawaguchi, T. Nakajo, T. Hori, et al., “Reconstruction of the biliary tract in biliary atresia using jejunal conduit,” Journal of Japan Surgical Society, vol. 69, p. 1317, 1968.
  82. D. U. Tagge, E. P. Tagge, R. A. Drongowski, K. T. Oldham, and A. G. Coran, “A long-term experience with biliary atresia: reassessment of prognostic factors,” Annals of Surgery, vol. 214, no. 5, pp. 590–598, 1991. View at Scopus
  83. T. G. Canty, T. W. Self, D. L. Collins, and L. Bonaldi, “Recent experience with a modified Sawaguchi procedure for biliary atresia,” Journal of Pediatric Surgery, vol. 20, no. 3, pp. 211–216, 1985. View at Scopus
  84. K. Suruga, T. Miyano, and T. Kitahara, “Treatment of biliary atresia: a study of our operative results,” Journal of Pediatric Surgery, vol. 16, no. 4, pp. 621–626, 1981. View at Scopus
  85. M. Endo, K. Watanabe, T. Hirabayashi, H. Ikawa, J. Yokoyama, and M. Kitajima, “Outcomes of ileocolic conduit for biliary drainage in infants with biliary atresia; Comparison with Roux-en-Y type reconstruction,” Journal of Pediatric Surgery, vol. 30, no. 5, pp. 700–704, 1995. View at Scopus
  86. J. Liu and G. Li, “A comparison of spur valve and percutaneous enterostomy in Roux-Y portoenterostomy for biliary atresia,” Chinese Medical Journal, vol. 114, no. 9, pp. 986–987, 2001. View at Scopus
  87. L. Ayuso, J. J. Vila-Carbó, J. Lluna, E. Hernández, and A. Marco, “Laparoscopic Kasai portoenterostom: present and future of biliary atresia treatment,” Cirugía Pediátrica, vol. 21, no. 1, pp. 23–26, 2008. View at Scopus
  88. E. Esteves, E. C. Neto, M. O. Neto, J. Devanir, and R. E. Pereira, “Laparoscopic Kasai portoenterostomy for biliary atresia,” Pediatric Surgery International, vol. 18, no. 8, pp. 737–740, 2002. View at Scopus
  89. S. Dutta, R. Woo, and C. T. Albanese, “Minimal access portoenterostomy: advantages and disadvantages of standard laparoscopic and robotic techniques,” Journal of Laparoendoscopic and Advanced Surgical Techniques, vol. 17, no. 2, pp. 258–264, 2007. View at Publisher · View at Google Scholar · View at Scopus
  90. B. M. Ure, J. F. Kuebler, N. Schukfeh, C. Engelmann, J. Dingemann, and C. Petersen, “Survival with the native liver after laparoscopic versus conventional kasai portoenterostomy in infants with biliary atresia: a prospective trial,” Annals of Surgery, vol. 253, no. 4, pp. 826–830, 2011. View at Publisher · View at Google Scholar · View at Scopus
  91. C. O. von Sochaczewski, C. Petersen, B. M. Ure, et al., “Laparoscopic versus conventional Kasai portoenterostomy does not facilitate subsequent liver transplantation in infants with biliary atresia,” Journal of Laparoendoscopic & Advanced Surgical Techniques A, vol. 22, no. 4, pp. 408–411, 2012.
  92. F. M. Karrer, J. R. Lilly, B. A. Stewart, and R. J. Hall, “Biliary atresia registry, 1976 to 1989,” Journal of Pediatric Surgery, vol. 25, no. 10, pp. 1076–1081, 1990. View at Publisher · View at Google Scholar · View at Scopus
  93. S. Matsuo, S. Suita, M. Kubota, K. Shono, T. Kamimura, and Y. Kinugasa, “Hazards of hepatic portocholecystostomy in biliary atresia,” European Journal of Pediatric Surgery, vol. 11, no. 1, pp. 19–23, 2001. View at Publisher · View at Google Scholar · View at Scopus
  94. F. Gauthier, F. Brunelle, and O. Bernard, “Choleperitonitis following hepatoportocholecystostomy for biliary atresia,” Chirurgie Pediatrique, vol. 25, no. 2, pp. 90–94, 1984. View at Scopus
  95. L. N. Bu, H. L. Chen, C. J. Chang et al., “Prophylactic oral antibiotics in prevention of recurrent cholangitis after the Kasai portoenterostomy,” Journal of Pediatric Surgery, vol. 38, no. 4, pp. 590–593, 2003. View at Publisher · View at Google Scholar · View at Scopus
  96. D. A. Kelly and M. Davenport, “Current management of biliary atresia,” Archives of Disease in Childhood, vol. 92, no. 12, pp. 1132–1135, 2007. View at Publisher · View at Google Scholar · View at Scopus
  97. P. Vajro, M. Couturier, F. Lemonnier, and M. Odievre, “Effects of postoperative cholestyramine and phenobarbital administration on bile flow restoration in infants with extrahepatic biliary atresia,” Journal of Pediatric Surgery, vol. 21, no. 4, pp. 362–365, 1986. View at Scopus
  98. I. J. Elenkov, “Glucocorticoids and the Th1/Th2 balance,” Annals of the New York Academy of Sciences, vol. 1024, pp. 138–146, 2004. View at Publisher · View at Google Scholar · View at Scopus
  99. P. B. Miner Jr. and J. M. Gaito, “Bile flow in response to pharmacologic agents. Hepatic DNA as a reference standard,” Biochemical Pharmacology, vol. 28, no. 7, pp. 1063–1066, 1979. View at Publisher · View at Google Scholar · View at Scopus
  100. Y. Tatekawa, T. Muraji, and C. Tsugawa, “Glucocorticoid receptor α expression in the intrahepatic biliary epithelium and adjuvant steroid therapy in infants with biliary atresia,” Journal of Pediatric Surgery, vol. 40, no. 10, pp. 1574–1580, 2005. View at Publisher · View at Google Scholar · View at Scopus
  101. P. W. Dillon, E. Owings, R. Cilley, D. Field, A. Curnow, and K. Georgeson, “Immunosuppression as adjuvant therapy for biliary atresia,” Journal of Pediatric Surgery, vol. 36, no. 1, pp. 80–85, 2001. View at Publisher · View at Google Scholar · View at Scopus
  102. R. L. Meyers, L. S. Book, M. A. O'Gorman et al., “High-dose steroids, ursodeoxycholic acid, and chronic intravenous antibiotics improve bile flow after Kasai procedure in infants with biliary atresia,” Journal of Pediatric Surgery, vol. 38, no. 3, pp. 406–411, 2003. View at Publisher · View at Google Scholar · View at Scopus
  103. H. Kobayashi, A. Yamataka, H. Koga et al., “Optimum prednisolone usage in patients with biliary atresia postportoenterostomy,” Journal of Pediatric Surgery, vol. 40, no. 2, pp. 327–330, 2005. View at Publisher · View at Google Scholar · View at Scopus
  104. M. A. Escobar, C. L. Jay, R. M. Brooks et al., “Effect of corticosteroid therapy on outcomes in biliary atresia after Kasai portoenterostomy,” Journal of Pediatric Surgery, vol. 41, no. 1, pp. 99–103, 2006. View at Publisher · View at Google Scholar · View at Scopus
  105. M. Davenport, M. D. Stringer, S. A. Tizzard, P. McClean, G. Mieli-Vergani, and N. Hadzic, “Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia,” Hepatology, vol. 46, no. 6, pp. 1821–1827, 2007. View at Publisher · View at Google Scholar · View at Scopus
  106. M. Saeki, M. Nakano, and K. Hagane, “Early complications after hepatic porto-jejunostomy in biliary atresia,” Nippon Geka Gakkai zasshi, vol. 90, no. 9, pp. 1353–1356, 1989. View at Scopus
  107. C. S. Eberhardt, L. Merlini, V. A. Mclin, and B. E. Wildhaber, “Cholestasis as the leading sign of a transmesenteric hernia in a split-liver transplanted child—a case report and review of literature,” Pediatric Transplantation, vol. 16, no. 5, pp. 172–176, 2012. View at Publisher · View at Google Scholar · View at Scopus
  108. S. B. Moon, K. W. Park, H. J. Jae, and S. E. Jung, “Caudate arterial branch bleeding: a rare complication after a Kasai portoenterostomy,” Journal of Pediatric Surgery, vol. 44, no. 7, pp. e27–e30, 2009. View at Publisher · View at Google Scholar · View at Scopus
  109. M. Duché, M. Fabre, B. Kretzschmar, M. O. Serinet, F. Gauthier, and C. Chardot, “Prognostic value of portal pressure at the time of Kasai operation in patients with biliary atresia,” Journal of Pediatric Gastroenterology and Nutrition, vol. 43, no. 5, pp. 640–645, 2006. View at Scopus
  110. M. Duché, D. Habès, P. Roulleau, V. Haas, E. Jacquemin, and O. Bernard, “Prophylactic endoscopic sclerotherapy of large esophagogastric varices in infants with biliary atresia,” Gastrointestinal Endoscopy, vol. 67, no. 4, pp. 732–737, 2008. View at Publisher · View at Google Scholar · View at Scopus
  111. P. J. McKiernan, “Treatment of variceal bleeding,” Gastrointestinal Endoscopy Clinics of North America, vol. 11, no. 4, pp. 789–812, 2001. View at Scopus
  112. J. F. Botha, B. D. Campos, W. J. Grant et al., “Portosystemic shunts in children: a 15-year experience,” Journal of the American College of Surgeons, vol. 199, no. 2, pp. 179–185, 2004. View at Publisher · View at Google Scholar · View at Scopus
  113. P. E. Huppert, P. Goffette, W. Astfalk et al., “Transjugular intrahepatic portosystemic shunts in children with biliary atresia,” Cardiovascular and Interventional Radiology, vol. 25, no. 6, pp. 484–493, 2002. View at Publisher · View at Google Scholar · View at Scopus
  114. P. A. Lange and J. K. Stoller, “The hepatopulmonary syndrome,” Annals of Internal Medicine, vol. 122, no. 7, pp. 521–529, 1995. View at Scopus
  115. M. Castro and M. J. Krowka, “Hepatopulmonary syndrome: a pulmonary vascular complication of liver disease,” Clinics in Chest Medicine, vol. 17, no. 1, pp. 35–48, 1996. View at Publisher · View at Google Scholar · View at Scopus
  116. S. Ikeda, Y. Sera, S. Uchino et al., “Resolution of cirrhosis-related pulmonary shunting in two children with a transplanted liver,” Transplant International, vol. 9, no. 6, pp. 596–599, 1996. View at Publisher · View at Google Scholar · View at Scopus
  117. A. A. Condino, D. D. Ivy, J. A. O'Connor et al., “Portopulmonary hypertension in pediatric patients,” Journal of Pediatrics, vol. 147, no. 1, pp. 20–26, 2005. View at Publisher · View at Google Scholar · View at Scopus
  118. E. Y. Cheng and H. J. Woehlck, “Pulmonary artery hypertension complicating anesthesia for liver transplantation,” Anesthesiology, vol. 77, no. 2, pp. 389–392, 1992. View at Scopus
  119. H. Egawa, M. Kasahara, Y. Inomata et al., “Long-term outcome of living related liver transplantation for patients with intrapulmonary shunting and strategy for complications,” Transplantation, vol. 67, no. 5, pp. 712–717, 1999. View at Scopus
  120. S. Uemoto, Y. Inomata, H. Egawa et al., “Effects of hypoxemia on early postoperative course of liver transplantation in pediatric patients with intrapulmonary shunting,” Transplantation, vol. 63, no. 3, pp. 407–414, 1997. View at Publisher · View at Google Scholar · View at Scopus
  121. M. Watanabe, T. Hori, M. Kaneko et al., “Intrahepatic biliary cysts in children with biliary atresia who have had a Kasai operation,” Journal of Pediatric Surgery, vol. 42, no. 7, pp. 1185–1189, 2007. View at Publisher · View at Google Scholar · View at Scopus
  122. T. Nakama, T. Kitamura, A. Matsui, S. I. Makino, O. F. Senyuz, and K. Kanazawa, “Ultrasonographic findings and management of intrahepatic biliary tract abnormalities after portoenterostomy,” Journal of Pediatric Surgery, vol. 26, no. 1, pp. 32–36, 1991. View at Publisher · View at Google Scholar · View at Scopus
  123. Y. Tsuchida, T. Honna, and H. Kawarasaki, “Cystic dilatation of the intrahepatic biliary system in biliary atresia after hepatic portoenterostomy,” Journal of Pediatric Surgery, vol. 29, no. 5, pp. 630–634, 1994. View at Scopus
  124. A. Brunati, Z. Feruzi, E. Sokal et al., “Early occurrence of hepatocellular carcinoma in biliary atresia treated by liver transplantation,” Pediatric Transplantation, vol. 11, no. 1, pp. 117–119, 2007. View at Publisher · View at Google Scholar · View at Scopus
  125. J. M. Kim, S. K. Lee, C. H. Kwon, et al., “Hepatocellular carcinoma in an infant with biliary atresia younger than 1 year,” Journal of Pediatric Surgery, vol. 47, no. 4, pp. 819–821, 2012.
  126. F. Taat, D. K. Bosman, and D. C. Aronson, “Hepatoblastoma in a girl with biliary atresia: coincidence or co-incidence,” Pediatric Blood and Cancer, vol. 43, no. 5, pp. 603–605, 2004. View at Publisher · View at Google Scholar · View at Scopus
  127. P. B. Kulkarni and E. C. Beatty, “Cholangiocarcinoma associated with biliary cirrhosis due to congenital biliary atresia,” American Journal of Diseases of Children, vol. 131, no. 4, pp. 442–444, 1977. View at Scopus
  128. A. Vera, D. Villaveces, and R. Lopez, “Orthotopic liver transplantation for biliary atresia complicated by incidental cholangiocarcinoma,” Journal of Pediatric Gastroenterology and Nutrition, vol. 55, no. 3, pp. 336–337, 2012.
  129. K. L. Cox, W. E. Berquist, and R. O. Castillo, “Paediatric liver transplantation: indications, timing and medical complications,” Journal of Gastroenterology and Hepatology, vol. 14, pp. S61–S66, 1999. View at Scopus
  130. K. I. Wayman, K. L. Cox, and C. O. Esquivel, “Neurodevelopmental outcome of young children with extrahepatic biliary atresia 1 year after liver transplantation,” Journal of Pediatrics, vol. 131, no. 6, pp. 894–898, 1997. View at Publisher · View at Google Scholar · View at Scopus
  131. S. Fujita, I. D. Kim, K. Uryuhara et al., “Hepatic grafts from live donors: donor morbidity for 470 cases of live donation,” Transplant International, vol. 13, no. 5, pp. 333–339, 2000. View at Scopus
  132. K. Umeshita, K. Fujiwara, K. Kiyosawa et al., “Operative morbidity of living liver donors in Japan,” Lancet, vol. 362, no. 9385, pp. 687–690, 2003. View at Publisher · View at Google Scholar · View at Scopus
  133. J. C. Bucuvalas, K. M. Campbell, C. R. Cole, and S. L. Guthery, “Outcomes after liver transplantation: keep the end in mind,” Journal of Pediatric Gastroenterology and Nutrition, vol. 43, no. 1, pp. S41–S48, 2006. View at Publisher · View at Google Scholar · View at Scopus
  134. C. Chardot and M. O. Serinet, “Prognosis of biliary atresia: what can be further improved?” Journal of Pediatrics, vol. 148, no. 4, pp. 432–435, 2006. View at Publisher · View at Google Scholar · View at Scopus
  135. M. O. Serinet, P. Broué, E. Jacquemin et al., “Management of patients with biliary atresia in France: results of a decentralized policy 1986–2002,” Hepatology, vol. 44, no. 1, pp. 75–84, 2006. View at Publisher · View at Google Scholar · View at Scopus
  136. M. L. Santamaria, M. Gamez, J. Murcia, et al., “Kasai operation in the age of liver transplantation. Healing or merely palliative technique?” Cirugía Pediátrica, vol. 13, no. 3, pp. 102–105, 2000 (Spanish).
  137. B. E. Wildhaber, P. Majno, J. Mayr et al., “Biliary atresia: Swiss national study, 1994–2004,” Journal of Pediatric Gastroenterology and Nutrition, vol. 46, no. 3, pp. 299–307, 2008. View at Scopus
  138. E. R. Howard and M. Davenport, “The treatment of biliary atresia in Europe 1969–1995,” Tohoku Journal of Experimental Medicine, vol. 181, no. 1, pp. 75–83, 1997. View at Scopus
  139. F. M. Karrer, M. R. Price, D. D. Bensard et al., “Long-term results with the Kasai operation for biliary atresia,” Archives of Surgery, vol. 131, no. 5, pp. 493–496, 1996. View at Scopus
  140. E. R. Howard, G. MacLean, M. Nio, N. Donaldson, J. Singer, and R. Ohi, “Survival patterns in biliary atresia and comparison of quality of life of long-term survivors in Japan and England,” Journal of Pediatric Surgery, vol. 36, no. 6, pp. 892–897, 2001. View at Publisher · View at Google Scholar · View at Scopus
  141. T. Kumagi, J. P. Drenth, O. Guttman, et al., “Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review,” Liver International, vol. 32, no. 3, pp. 510–518, 2012.
  142. T. E. Starzl, L. J. Koep, and G. P. J. Schroter, “Liver replacement for pediatric patients,” Pediatrics, vol. 63, no. 6, pp. 825–829, 1979. View at Scopus
  143. B. L. Shneider, M. B. Brown, B. Haber et al., “A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000,” Journal of Pediatrics, vol. 148, no. 4, pp. 467–467, 2006. View at Publisher · View at Google Scholar · View at Scopus
  144. H. Kobayashi and M. D. Stringer, “Biliary atresia,” Seminars in Neonatology, vol. 8, no. 5, pp. 383–391, 2003. View at Publisher · View at Google Scholar · View at Scopus
  145. E. Shteyer, G. A. Ramm, C. Xu, F. V. White, and R. W. Shepherd, “Outcome after portoenterostomy in biliary atresia: pivotal role of degree of liver fibrosis and intensity of stellate cell activation,” Journal of Pediatric Gastroenterology and Nutrition, vol. 42, no. 1, pp. 93–99, 2006. View at Publisher · View at Google Scholar · View at Scopus
  146. P. Schweizer and K. Lünzmann, “Extrahepatic bile duct atresia: how efficient is the hepatoporto- enterostomy?” European Journal of Pediatric Surgery, vol. 8, no. 3, pp. 150–154, 1998. View at Scopus
  147. P. Y. Hung, C. C. Chen, W. J. Chen et al., “Long-term prognosis of patients with biliary atresia: a 25 year summary,” Journal of Pediatric Gastroenterology and Nutrition, vol. 42, no. 2, pp. 190–195, 2006. View at Publisher · View at Google Scholar · View at Scopus
  148. Y. Ohhama, M. Shinkai, S. Fujita, T. Nishi, and H. Yamamoto, “Early prediction of long-term survival and the timing of liver transplantation after the Kasai operation,” Journal of Pediatric Surgery, vol. 35, no. 7, pp. 1031–1034, 2000. View at Publisher · View at Google Scholar · View at Scopus
  149. R. Subramaniam, C. M. Doig, J. Bowen, and J. Bruce, “Initial response to portoenterostomy determines long-term outcome in patients with biliary atresia,” Journal of Pediatric Surgery, vol. 35, no. 4, pp. 593–597, 2000. View at Scopus
  150. S. Chittmittrapap, B. Chandrakamol, Y. Poovorawan, and P. Suwangool, “Factors influencing outcome after hepatic portoenterostomy for biliary atresia: a logistic regression analysis,” Journal of the Medical Association of Thailand, vol. 88, no. 8, pp. 1077–1082, 2005. View at Scopus
  151. H. Lampela, A. Ritvanen, S. Kosola, et al., “National centralization of biliary atresia care to an assigned multidisciplinary team provides high-quality outcomes,” Scandinavian Journal of Gastroenterology, vol. 47, no. 1, pp. 99–107, 2012.
  152. R. P. Altman, J. R. Lilly, J. Greenfeld, A. Weinberg, K. Van Leeuwen, and L. Flanigan, “A multivariable risk factor analysis of the portoenterostomy (Kasai) procedure for biliary atresia: twenty-five years of experience from two centers,” Annals of Surgery, vol. 226, no. 3, pp. 348–355, 1997. View at Publisher · View at Google Scholar · View at Scopus
  153. J. L. dos Santos, C. T. Cerski, V. D. da Silva, et al., “Factors related to the post-portoenterostomy prognosis of biliary atresia,” Jornal de Pediatria, vol. 78, no. 4, pp. 341–346, 2002.
  154. S. Sangkhathat, K. Tadtayathikom, S. Patrapinyokul, and S. Osatakul, “Peri-operative factors predicting the outcome of hepatic porto-enterostomy in infants with biliary atresia,” Journal of the Medical Association of Thailand, vol. 86, no. 3, pp. 224–231, 2003. View at Scopus
  155. M. Davenport, E. Caponcelli, E. Livesey, N. Hadzic, and E. Howard, “Surgical outcome in biliary atresia: etiology affects the influence of age at surgery,” Annals of Surgery, vol. 247, no. 4, pp. 694–698, 2008. View at Publisher · View at Google Scholar · View at Scopus
  156. B. T. Schoen, H. Lee, K. Sullivan, and R. R. Ricketts, “The Kasai portoenterostomy: when is it too late?” Journal of Pediatric Surgery, vol. 36, no. 1, pp. 97–99, 2001. View at Publisher · View at Google Scholar · View at Scopus
  157. C. Chardot, M. Carton, N. Spire-Bendelac et al., “Is the Kasai operation still indicated in children older than 3 months diagnosed with biliary atresia?” Journal of Pediatrics, vol. 138, no. 2, pp. 224–228, 2001. View at Publisher · View at Google Scholar · View at Scopus
  158. R. A. Schreiber, C. C. Barker, E. A. Roberts, et al., “Biliary atresia: the Canadian experience,” Journal of Pediatrics, vol. 151, no. 6, pp. 659–665, 2007.
  159. M. O. Serinet, B. E. Wildhaber, P. Broué et al., “Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening,” Pediatrics, vol. 123, no. 5, pp. 1280–1286, 2009. View at Publisher · View at Google Scholar · View at Scopus
  160. J. S. Heo, J. W. Park, K. W. Lee et al., “Posttransplantation lymphoproliferative disorder in pediatric liver transplantation,” Transplantation Proceedings, vol. 36, no. 8, pp. 2307–2308, 2004. View at Publisher · View at Google Scholar · View at Scopus
  161. I. Mushtaq, S. Logan, M. Morris et al., “Screening of newborn infants for cholestatic hepatobiliary disease with tandem mass spectrometry,” British Medical Journal, vol. 319, no. 7208, pp. 471–477, 1999. View at Scopus
  162. A. P. Mowat, L. L. Davidson, and M. C. Dick, “Earlier identification of biliary atresia and hepatobiliary disease: selective screening in the third week of life,” Archives of Disease in Childhood, vol. 72, no. 1, pp. 90–92, 1995. View at Scopus
  163. Z. Song, R. Dong, Y. Fan, et al., “Identification of serum protein biomarkers in biliary atresia by mass spectrometry and ELISA,” Journal of Pediatric Gastroenterology and Nutrition, vol. 55, no. 4, pp. 370–375, 2012.
  164. A. Matsui, Y. Kasano, Y. Yamauchi et al., “Direct enzymatic assay of urinary sulfated bile acids to replace serum bilirubin testing for selective screening of neonatal cholestasis,” Journal of Pediatrics, vol. 129, no. 2, pp. 306–308, 1996. View at Publisher · View at Google Scholar · View at Scopus
  165. T. Akiyama and Y. Yamauchi, “Use of near infrared reflectance spectroscopy in the screening for biliary atresia,” Journal of Pediatric Surgery, vol. 29, no. 5, pp. 645–647, 1994. View at Scopus
  166. M. W. Lai, M. H. Chang, S. C. Hsu et al., “Differential diagnosis of extrahepatic biliary atresia from neonatal hepatitis: a prospective study,” Journal of Pediatric Gastroenterology and Nutrition, vol. 18, no. 2, pp. 121–127, 1994. View at Scopus
  167. A. Matsui and M. Dodoriki, “Screening for biliary atresia,” Lancet, vol. 345, no. 8958, p. 1181, 1995. View at Scopus
  168. A. Matsui and T. Ishikawa, “Identification of infants with biliary atresia in Japan,” Lancet, vol. 343, no. 8902, p. 925, 1994. View at Scopus
  169. C. H. Hsiao, M. H. Chang, H. L. Chen et al., “Universal screening for biliary atresia using an infant stool color card in Taiwan,” Hepatology, vol. 47, no. 4, pp. 1233–1240, 2008. View at Publisher · View at Google Scholar · View at Scopus
  170. J. J. Tseng, M. S. Lai, M. C. Lin, et al., “Stool color card screening for biliary atresia,” Pediatrics, vol. 128, no. 5, pp. 1209–1215, 2011.
  171. B. E. Wildhaber, “Screening for biliary atresia: Swiss stool color card,” Hepatology, vol. 54, no. 1, pp. 368–368, 2011. View at Publisher · View at Google Scholar · View at Scopus
  172. T. H. Lien, M. H. Chang, J. F. Wu et al., “Effects of the infant stool color card screening program on 5-year outcome of biliary atresia in Taiwan,” Hepatology, vol. 53, no. 1, pp. 202–208, 2011. View at Publisher · View at Google Scholar · View at Scopus