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Biliary Atresia (BA) occurs in about 1:15.000 live birth. The diagnosis is confirmed by Cholangiography. Kasai Portoenterostomy (KPE) is one of the management and age at surgery is the important factors for the success. This case report described the clinical presentation and management of BA. Three cases of BA were reported during 2016. They were two cases of two months old and four months old. All came with persisted jaundice skin > 14 days of life. The laboratory showed elevated direct bilirubin serum. Three of them underwent cholangiography and KPE. After surgery, one suffered from repeated Cholangitis, one repeated abdominal leakage, and other without complication. All of this three cases were survived until this case report was made. The diagnosis of BA should be considered for any infant who came with jaundice skin which persist after 14 days of life. Age at KPE is an important determinant of jaundice clearance.




  1. Moreira RK, Cabral R, Cowles RA, Lobritto SJ. Biliry Atresia: A Multidisciplinary Approach to Diagnosis and Management. Arch Pathol Lab Med.2012;136:746-758
  2. Sinha CK, Davenport M. Biliary Atresia. J Indian Assoc Pediatr Surg.2008;13(2):49-54
  3. Purnomo B, Hegar B. Biliary Atresia in Infants with Cholestasis. The Indonesia Journal of Gastroenterology, Hepatology, and Digestive Endoscopy.2011;12(3):160-163
  4. Wang KS. Newborn Screening for Biliary Atresia. Pediatrics.2015;136:1663-1669
  5. Carvalho E, Ivantes CAP, Bezerra JA. Extrahepatic biliary atresia: current concepts and future directions. J Pediatr (Rio J).2007;83(2):105-120
  6. Urganci N, Cetinkaya F, Kalyoncu D, Cakir EP, Yilmaz B. Infants with cholestasis : Diagnosis, Management and Outcome. Marmara Medical Journal.2012;25:83-86
  7. Voeks JH, Howard VJ. Newborn Bilirubin Screening for Biliary Atresia. N Eng J Med.2016;375:605-606
  8. Hopkins P, Yazigi N, Nylund C. Incidence of Biliary Atresia and Timing of Hepatoportoenterostomy in the United States. J Pediatr.2017;05:1-5
  9. Shanmugam NP, Narasimhan G, Rajindratjith S. Living with biliary atresia: from Kasai Portoenterostomy to liver transplantation. Sri Lanka Journal of Child Health.2016;45(2):116-122
  10. Agin M, Tumgor G, Alkan M, Ozden O, Satar M, Tuncer R. Clues to the diagnosis of biliary atresia in neonatal cholestasis. Turk J Gastroenterol.2016;27:37-41
  11. Chen S, Chang M, Du J, Lin C, Chen A, Lee H, Lau B, Yang Y, Wu T, Chu C, Lai M, Chen H. Screening for Biliary Atresia by Infant Stool Color Card in Taiwan. Pediatrics.2006;117:1147-1154
  12. Zhou L, Shan Q, Tian W, Wang Z, Liang J, Xie X. Ultrasound for the Diagnosis of Biliary Atresia: A Meta Analysis. AJR.2016;206:W1-W10
  13. Willot S, Uhlen S, Michaud L, Briand G, Bonnevalle M, Sfeir R, Gottrand F. Effect of Ursodeoxycholic Acid on Liver Function in Children after successful surgery for Biliary Atresia. Pediatrics.2008;122(6):1236-1241
  14. Moghtaderi M, Gorji M, Farahmand F, Fallahi G, Ashjai B. Long-Term Outcome of Children with Biliary Atresia after Kasei Surgery in Iranian Infants. Pediatr Ther.2016;6(1):1-4
  15. Superina R, Magee JC, Brandt ML, Healey PJ, Tiao G, Ryckman F, Karrer FM, Lyer K, Fecteau A, West K, Burns RC, Flake A, Lee H, Lowell JA, Dillon P, Colombani P, Ricketts R, Li Y, Moore J, Wang KS. 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(4):577-585
  16. Bhatia V, Bavdekar A, Matthai J, Waikar Y, Sibal A. Management of Neonatal Cholestasis : Consensus Statement of Pediatric Gastroenterology Chapter in Indian Academy of Pediatrics. Indian Pediatrics.2014;51:203-208
  17. Shneider BL. Screening for Biliary Atresia: A Ray of Hope. Hepatology.2008;47(4):1105-1107
  18. Lee WS, Chai PF. Clinical Features Differentiating Biliary Atresia from other causes of Neonatal Cholestasis. Ann Acad Med Singapore.2010;39:648-654
  19. Rouzrokh M, Sobhiyeh MR, Heibatollahi M. The sensitivity, specificity, positive and negative predictive values of stool color test, triangular cord sign and hepatobiliary scintigraphy in diagnosis of Infantile Biliary Atresia. IRCMJ.2009;11(4):425-430
  20. Kelly DA, Davenport M. Current Management of Biliary Atresia. Arch Dis Child 2007;92:1132-1135
  21. Ramachandran P, Safwan M, Reddy MS, Rela M. Recent Trends in the Diagnosis and Management of Biliary Atresia in Developing Countries. Indian Pediatrics.2015;52:871-877
  22. Bezerra JA. Novel approaches to the treatment of Biliary Atresia. Clinical Liver Disease.2016;8(6):145-148
  23. D’Agata ID, Balistreri WF. Evaluation of Liver Disease in the Pediatric Patient. Pediatrics in Review.1999;20(11):376-388
  24. Lien T, Bu L, Wu J, Chen H, Chen A, Lai M, Shih H, Lee I, Hsu H, Ni Y, and Chang M. Use of Lactobacillus casei rhamnosus to prevent Cholangitis in Billiary Atresia after Kasai operation. JPGN.2015;60:654-658,
  25. Song Z, Dong R, Shen Z, Chen G, Yang Y, Zheng S. Surgical outcome and etiologic heterogeneity of infants with biliary atresia who received Kasai operation less than 60 days after birth. A Retrospective study. Medicine.2017:96(26);1-5.
  26. Sira MM, Sira AM, Elhenawy IA, Khalil FO. Prevalence of Serological Markers of TORCH Infections in Biliary Atresia and Other Neonatal Cholestatic Disorders. Peertechz J Pediatr Ther;2(1):013-017
  27. Wong K, Chung P, Chan I, Lan L, and Tam P. Performing Kasai Portoenterostomy beyond 60 days of life is not necessarily associated with a worse outcome. JPGN.2010.Vol 51(5);631-634
  28. Lee S, Park H, Moon S, Jung S, Kim J, Kwon C, Kim S, Joh J, Seo J, Lee S. Long-term results of biliary atresia in the era of liver transplantation. Pediatr Surg Int. 2013:29;1297-1301
  29. Kim S, Kim M, Lee M, Yoon C, Han SJ, Koh H. Ultrasonograpic findings of typoe IIIa biliary atresia. Ultrasonography.2014;33(4):267-273
  30. Shneider BL, Magee JC, Bezerra JA, Haber B, Karpen SJ, Raghunathan T, Rosenthal P, Schwarz K, Suchy FJ, Kerkar N, Turmelle Y, Whitington PF, Robuck PR, Sokol RJ. Efficacy of Fat-Soluble Vitamin Supplementation in Infants with Biliary Atresia. PEDIATRICS.2012;130(3):e607-e614
  31. Decharun K, Leys CM, West KW, Finnell ME. Prophylactic Antibiotics for Prevention of Cholangitis in Patients with Biliary Atresia status Post-Kasai Portoenterostomy: A Systematic Review. Clinical Pediatrics.2015;1-6
  32. Khorasani EN, Mansouri F. Application of Ursodeoxycholic Acid in Hepatobiliary Scintigraphy for Neonatal Hyperbilirubinemia: Comparison with Phenobarbital. Iran J Nucl Med.2009;17(2):20-25
  33. Buck ML. Use of Ursodiol in Infants and Children. Pediatric Pharmacotherapy;15(2)
  34. Luo Y, Zheng S. Current concept about postoperative cholangitis in biliary atresia. World J Pediatr.2008;4(1):14-18
  35. Zhang S, Wu Y, Liu Z, Tao Q, Huang J, Yang W. Hepatic Pathology of biliary atresia: A new comprehensive evaluation method using liver biopsy. Turk J Gastroenterol.2016;27:257-263
  36. Bijl EJ, Bharwani KD, Houwen RHJ, de Man RA. The Long-term outcome of the Kasai operation in patients with biliary atresia: a systematic review. The Journal of Medicine.2013;71(4):170-173
  37. Sokol RJ, Shepherd RW, Superina R, Bezerra JA, Robuck P, Hoofnagle JH. Screening and outcomes in Biliary Atresia: Summary of A. National Institutes of Health Workshop. Hepatology;46(2):566-578

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