Journal of the Korean Society of Radiology (대한영상의학회지)

The Korean Society of Radiology (대한영상의학회)
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Overview of Biliary Atresia

담도폐쇄증의 개요

  • Tae Yeon Jeon (Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • 전태연 (성균관대학교 의과대학 삼성서울병원 영상의학과)
  • Received : 2022.06.08
  • Accepted : 2022.09.10
  • Published : 2022.09.01
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Abstract

Biliary atresia is a progressive, idiopathic, obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction in the neonatal period. It is the most common indication for liver transplantation in children. If untreated, progressive liver cirrhosis leads to death by two years of age. Nowadays, more than 90% of biliary atresia patients survive into adulthood with the development of Kasai portoenterostomy and liver transplantation technology. Early diagnosis is critical since the success rate of the Kasai portoenterostomy decreases with time. This study comprehensively reviews the recent advances in the etiology, classification, prevalence, clinical manifestations, treatment, and prognosis of biliary atresia.

담도폐쇄증은 간외 담도의 진행성, 특발성, 섬유 폐쇄성 염증질환으로 신생아기에 담도 폐쇄가 나타나고 소아 간이식의 가장 흔한 적응증이다. 조기에 치료하지 않으면 간경화가 진행되어 2세 이전에 사망하게 된다. 오늘날 Kasai portoenterostomy와 간이식 수술기법의 발달로 담도폐쇄증 환자의 90% 이상이 성인기까지 생존한다. 따라서 Kasai portoenterostomy의 성공 가능성은 시간이 지날수록 줄어들기 때문에 조기 진단이 매우 중요하다. 이 고찰은 담도폐쇄증의 원인, 분류, 유병률, 임상양상, 치료와 예후를 최신 지견을 중심으로 종합적으로 검토하고자 한다.

Keywords

References

  1. Caton AR, Druschel CM, McNutt LA. The epidemiology of extrahepatic biliary atresia in New York State, 1983-98. Paediatr Perinat Epidemiol 2004;18:97-105  https://doi.org/10.1111/j.1365-3016.2003.00536.x
  2. Hopkins PC, Yazigi N, Nylund CM. Incidence of biliary atresia and timing of hepatoportoenterostomy in the United States. J Pediatr 2017;187:253-257  https://doi.org/10.1016/j.jpeds.2017.05.006
  3. Davit-Spraul A, Baussan C, Hermeziu B, Bernard O, Jacquemin E. CFC1 gene involvement in biliary atresia with polysplenia syndrome. J Pediatr Gastroenterol Nutr 2008;46:111-112  https://doi.org/10.1097/01.mpg.0000304465.60788.f4
  4. Hartley JL, Davenport M, Kelly DA. Biliary atresia. Lancet 2009;374:1704-1713  https://doi.org/10.1016/S0140-6736(09)60946-6
  5. Bezerra JA, Wells RG, Mack CL, Karpen SJ, Hoofnagle JH, Doo E, et al. Biliary atresia: clinical and research challenges for the twenty-first century. Hepatology 2018;68:1163-1173  https://doi.org/10.1002/hep.29905
  6. Davenport M. Biliary atresia: from Australia to the zebrafish. J Pediatr Surg 2016;51:200-205  https://doi.org/10.1016/j.jpedsurg.2015.10.058
  7. Napolitano M, Franchi-Abella S, Damasio MB, Augdal TA, Avni FE, Bruno C, et al. Practical approach to imaging diagnosis of biliary atresia, part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound. Pediatr Radiol 2021;51:314-331  https://doi.org/10.1007/s00247-020-04840-9
  8. Schwarz KB, Haber BH, Rosenthal P, Mack CL, Moore J, Bove K, et al. Extrahepatic anomalies in infants with biliary atresia: results of a large prospective North American multicenter study. Hepatology 2013;58:1724-1731  https://doi.org/10.1002/hep.26512
  9. Shneider BL, Brown MB, Haber B, Whitington PF, Schwarz K, Squires R, et al. A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J Pediatr 2006;148:467-474  https://doi.org/10.1016/j.jpeds.2005.12.054
  10. Fouquet V, Alves A, Branchereau S, Grabar S, Debray D, Jacquemin E, et al. Long-term outcome of pediatric liver transplantation for biliary atresia: a 10-year follow-up in a single center. Liver Transpl 2005;11:152-160  https://doi.org/10.1002/lt.20358
  11. Chung PHY, Zheng S, Tam PKH. Biliary atresia: east versus west. Semin Pediatr Surg 2020;29:150950 
  12. Lee KJ, Kim JW, Moon JS, Ko JS. Epidemiology of biliary atresia in Korea. J Korean Med Sci 2017;32:656-660  https://doi.org/10.3346/jkms.2017.32.4.656
  13. Davenport M, Muntean A, Hadzic N. Biliary atresia: clinical phenotypes and aetiological heterogeneity. J Clin Med 2021;10:5675 
  14. Shin HJ, Yoon H, Han SJ, Ihn K, Koh H, Kwon JY, et al. Key imaging features for differentiating cystic biliary atresia from choledochal cyst: prenatal ultrasonography and postnatal ultrasonography and MRI. Ultrasonography 2021;40:301-311  https://doi.org/10.14366/usg.20061
  15. Morel B, Kolanska K, Dhombres F, Jouannic JM, Franchi-Abella S, Ducou Le Pointe H, et al. Prenatal ultrasound diagnosis of cystic biliary atresia. Clin Case Rep 2015;3:1050-1051  https://doi.org/10.1002/ccr3.442
  16. Davenport M. Biliary atresia: clinical aspects. Semin Pediatr Surg 2012;21:175-184  https://doi.org/10.1053/j.sempedsurg.2012.05.010
  17. Masumoto K, Kai H, Oka Y, Otake R, Yoshizato T, Miyamoto S, et al. A case of cystic biliary atresia with an antenatally detected cyst: the possibility of changing from a correctable type with a cystic lesion (I cyst) to an uncorrectable one (IIId). Pediatr Surg Int 2011;27:99-102  https://doi.org/10.1007/s00383-010-2715-1
  18. Suzuki T, Hashimoto T, Hussein MH, Hara F, Hibi M, Kato T. Biliary atresia type I cyst and choledochal cyst [corrected]: can we differentiate or not? J Hepatobiliary Pancreat Sci 2013;20:465-470  https://doi.org/10.1007/s00534-013-0605-3
  19. Fischler B, Ehrnst A, Forsgren M, Orvell C, Nemeth A. The viral association of neonatal cholestasis in Sweden: a possible link between cytomegalovirus infection and extrahepatic biliary atresia. J Pediatr Gastroenterol Nutr 1998;27:57-64  https://doi.org/10.1002/j.1536-4801.1998.tb01101.x
  20. Zani A, Quaglia A, Hadzi'c N, Zuckerman M, Davenport M. Cytomegalovirus-associated biliary atresia: an aetiological and prognostic subgroup. J Pediatr Surg 2015;50:1739-1745  https://doi.org/10.1016/j.jpedsurg.2015.03.001
  21. Xu Y, Yu J, Zhang R, Yin Y, Ye J, Tan L, et al. The perinatal infection of cytomegalovirus is an important etiology for biliary atresia in China. Clin Pediatr (Phila) 2012;51:109-113  https://doi.org/10.1177/0009922811406264
  22. Shen O, Rabinowitz R, Yagel S, Gal M. Absent gallbladder on fetal ultrasound: prenatal findings and postnatal outcome. Ultrasound Obstet Gynecol 2011;37:673-677  https://doi.org/10.1002/uog.8861
  23. Gu YH, Yokoyama K, Mizuta K, Tsuchioka T, Kudo T, Sasaki H, 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;166:897-902.e1  https://doi.org/10.1016/j.jpeds.2014.12.063
  24. Schreiber RA, Masucci L, Kaczorowski J, Collet JP, Lutley P, Espinosa V, et al. Home-based screening for biliary atresia using infant stool colour cards: a large-scale prospective cohort study and cost-effectiveness analysis. J Med Screen 2014;21:126-132  https://doi.org/10.1177/0969141314542115
  25. Lee M, Chen SC, Yang HY, Huang JH, Yeung CY, Lee HC. Infant stool color card screening helps reduce the hospitalization rate and mortality of biliary atresia: a 14-year nationwide cohort study in Taiwan. Medicine (Baltimore) 2016;95:e3166 
  26. Franciscovich A, Vaidya D, Doyle J, Bolinger J, Capdevila M, Rice M, et al. PoopMD, a mobile health application, accurately identifies infant acholic stools. PLoS One 2015;10:e0132270 
  27. Morinville V, Ahmed N, Ibberson C, Kovacs L, Kaczorowski J, Bryan S, et al. Home-based screening for biliary atresia using infant stool color cards in Canada: Quebec feasibility study. J Pediatr Gastroenterol Nutr 2016;62:536-541  https://doi.org/10.1097/MPG.0000000000001042
  28. Fanna M, Masson G, Capito C, Girard M, Guerin F, Hermeziu B, et al. Management of biliary atresia in France 1986 to 2015: long-term results. J Pediatr Gastroenterol Nutr 2019;69:416-424  https://doi.org/10.1097/MPG.0000000000002446
  29. Serinet MO, Wildhaber BE, Broue P, Lachaux A, Sarles J, Jacquemin E, 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;123:1280-1286  https://doi.org/10.1542/peds.2008-1949
  30. Schreiber RA, Barker CC, Roberts EA, Martin SR, Alvarez F, Smith L, et al. Biliary atresia: the Canadian experience. J Pediatr 2007;151:659-665.e1  https://doi.org/10.1016/j.jpeds.2007.05.051
  31. Lien TH, Chang MH, Wu JF, Chen HL, Lee HC, Chen AC, et al. Effects of the infant stool color card screening program on 5-year outcome of biliary atresia in Taiwan. Hepatology 2011;53:202-208  https://doi.org/10.1002/hep.24023
  32. Shneider BL, Magee JC, Karpen SJ, Rand EB, Narkewicz MR, Bass LM, et al. Total serum bilirubin within 3 months of hepatoportoenterostomy predicts short-term outcomes in biliary atresia. J Pediatr 2016;170:211-217  https://doi.org/10.1016/j.jpeds.2015.11.058
  33. Nio M, Ohi R, Miyano T, Saeki M, Shiraki K, Tanaka K; Japanese Biliary Atresia Registry. Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry. J Pediatr Surg 2003;38:997-1000  https://doi.org/10.1016/S0022-3468(03)00178-7
  34. Superina R, Magee JC, Brandt ML, Healey PJ, Tiao G, Ryckman F, 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;254:577-585  https://doi.org/10.1097/SLA.0b013e3182300950
  35. Chardot C, Buet C, Serinet MO, Golmard JL, Lachaux A, Roquelaure B, et al. Improving outcomes of biliary atresia: French national series 1986-2009. J Hepatol 2013;58:1209-1217  https://doi.org/10.1016/j.jhep.2013.01.040
  36. Witt M, van Wessel DBE, de Kleine RHJ, Bruggink JLM, Hulscher JBF, Verkade HJ; NeSBAR (Netherlands Study group on Biliary Atresia Registry). Prognosis of biliary atresia after 2-year survival with native liver: a nationwide cohort analysis. J Pediatr Gastroenterol Nutr 2018;67:689-694  https://doi.org/10.1097/MPG.0000000000002130
  37. Arnon R, Annunziato RA, D'Amelio G, Chu J, Shneider BL. Liver transplantation for biliary atresia: is there a difference in outcome for infants? J Pediatr Gastroenterol Nutr 2016;62:220-225  https://doi.org/10.1097/MPG.0000000000000986
  38. Taylor SA, Venkat V, Arnon R, Gopalareddy VV, Rosenthal P, Erinjeri J, et al. Improved outcomes for liver transplantation in patients with biliary atresia since pediatric end-stage liver disease implementation: analysis of the society of pediatric liver transplantation registry. J Pediatr 2020;219:89-97 https://doi.org/10.1016/j.jpeds.2019.12.023