Acknowledgement
We thank Katie O'Hearn, Msc (Children's Hospital of Eastern Ontario Research Institute) for methodological assistance and Margaret Sampson, MLIS, PhD, AHIP (Children's Hospital of Eastern Ontario) for developing the electronic search strategies.
References
- Balistreri WF, Grand R, Hoofnagle JH, Suchy FJ, Ryckman FC, Perlmutter DH, et al. Biliary atresia: current concepts and research directions. Summary of a symposium. Hepatology 1996;23:1682-92. https://doi.org/10.1002/hep.510230652
- Schreiber RA, Kleinman RE. Biliary atresia. J Pediatr Gastroenterol Nutr 2002;35 Suppl 1:S11-6. https://doi.org/10.1097/00005176-200207001-00005
- Lakshminarayanan B, Davenport M. Biliary atresia: a comprehensive review. J Autoimmun 2016;73:1-9. https://doi.org/10.1016/j.jaut.2016.06.005
- Nomden M, van Wessel DBE, Ioannou S, Verkade HJ, de Kleine RH, Alizadeh BZ, et al. A higher incidence of isolated biliary atresia in rural areas: results from an epidemiological study in the Netherlands. J Pediatr Gastroenterol Nutr 2021;72:202-9. https://doi.org/10.1097/MPG.0000000000002916
- Schreiber RA, Barker CC, Roberts EA, Martin SR, Alvarez F, Smith L, et al. Biliary atresia: the Canadian experience. J Pediatr 2007;151:659-65, 665.e1. https://doi.org/10.1016/j.jpeds.2007.05.051
- Townsend MR, Jaber A, Abi Nader H, Eid SM, Schwarz K. Factors associated with timing and adverse outcomes in patients with biliary atresia undergoing Kasai hepatoportoenterostomy. J Pediatr 2018;199:237-42.e2. https://doi.org/10.1016/j.jpeds.2018.04.001
- Nio M, Sasaki H, Wada M, Kazama T, Nishi K, Tanaka H. Impact of age at Kasai operation on short- and long-term outcomes of type III biliary atresia at a single institution. J Pediatr Surg 2010;45:2361-3. https://doi.org/10.1016/j.jpedsurg.2010.08.032
- Qiao G, Li L, Cheng W, Zhang Z, Ge J, Wang C. Conditional probability of survival in patients with biliary atresia after Kasai portoenterostomy: a Chinese population-based study. J Pediatr Surg 2015;50:1310-5. https://doi.org/10.1016/j.jpedsurg.2015.03.062
- Karrer FM, Price MR, Bensard DD, Sokol RJ, Narkewicz MR, Smith DJ, et al. Long-term results with the Kasai operation for biliary atresia. Arch Surg 1996;131:493-6. https://doi.org/10.1001/archsurg.1996.01430170039006
- Tessier MEM, Harpavat S, Shepherd RW, Hiremath GS, Brandt ML, Fisher A, et al. Beyond the Pediatric end-stage liver disease system: solutions for infants with biliary atresia requiring liver transplant. World J Gastroenterol 2014;20:11062-8. https://doi.org/10.3748/wjg.v20.i32.11062
- Schoen BT, Lee H, Sullivan K, Ricketts RR. The Kasai portoenterostomy: when is it too late? J Pediatr Surg 2001;36:97-9. https://doi.org/10.1053/jpsu.2001.20020
- Capparelli MA, Ayarzabal VH, Halac ET, Questa HA, Minetto MJ, Cervio G, et al. Preoperative risk factors for the early failure of the Kasai portoenterostomy in patients with biliary atresia. Pediatr Surg Int 2021;37:1183-9. https://doi.org/10.1007/s00383-021-04933-y
- El-Araby HA, Saber MA, Radwan NM, Taie DM, Adawy NM, Sira AM. Temporal histopathological changes in biliary atresia: a perspective for rapid fibrosis progression. Ann Hepatol 2021;21:100263.
- He L, Ip DKM, Tam G, Lui VCH, Tam PKH, Chung PHY. Biomarkers for the diagnosis and postKasai portoenterostomy prognosis of biliary atresia: a systematic review and meta-analysis. Sci Rep 2021;11:11692.
- Tang X, Lv Y, Pu L, Ma J, Jin S, Xiang B. Matrix metalloproteinase-7 as a diagnostic marker for biliary atresia: a systematic review and meta-analysis. Indian J Surg 2021. doi: 10.1007/s12262-021-03107-3. [Epub ahead of print].
- Yoon HM, Suh CH, Kim JR, Lee JS, Jung AY, Cho YA. Diagnostic performance of sonographic features in patients with biliary atresia: a systematic review and meta-analysis. J Ultrasound Med 2017;36:2027-38. https://doi.org/10.1002/jum.14234
- Hinojosa-Gonzalez DE, Bueno LC, Roblesgil-Medrano A, Salgado-Garza G, Hurtado-Arellano S, Farias JS, et al. Laparoscopic vs open portoenterostomy in biliary atresia: a systematic review and meta-analysis. Pediatr Surg Int 2021;37:1477-87. https://doi.org/10.1007/s00383-021-04964-5
- Losty P, Guiney E. Biliary atresia--lessons from the Dublin experience. Ir Med J 1992;85:144-7. PUBMED
- Lin JN, Wang KL, Chuang JH. The efficacy of Kasai operation for biliary atresia: a single institutional experience. J Pediatr Surg 1992;27:704-6. https://doi.org/10.1016/S0022-3468(05)80095-8
- Wildhaber BE, Coran AG, Drongowski RA, Hirschl RB, Geiger JD, Lelli JL, et al. The Kasai portoenterostomy for biliary atresia: a review of a 27-year experience with 81 patients. J Pediatr Surg 2003;38:1480-5. https://doi.org/10.1016/S0022-3468(03)00499-8
- Engelskirchen R, Holschneider AM, Gharib M, Vente C. Biliary atresia--a 25-year survey. Eur J Pediatr Surg 1991;1:154-60. https://doi.org/10.1055/s-2008-1042478
- Karrer FM, Lilly JR, Stewart BA, Hall RJ. Biliary atresia registry, 1976 to 1989. J Pediatr Surg 1990;25:1076-80; discussion 1081. https://doi.org/10.1016/0022-3468(90)90222-U
- Higgins JPT, Thomas J, Chandler J, Cumpston M, Li T, Page MJ, et al. Cochrane handbook for systematic reviews of interventions. 2nd ed. Hoboken: Wiley-Blackwell, 2019.
- Page MJ, McKenzie JE, Bossuyt PM, Boutron I, Hoffmann TC, Mulrow CD, et al. The PRISMA 2020 statement: an updated guideline for reporting systematic reviews. Syst Rev 2021;10:89.
- Bramer WM, de Jonge GB, Rethlefsen ML, Mast F, Kleijnen J. A systematic approach to searching: an efficient and complete method to develop literature searches. J Med Libr Assoc 2018;106:531-41.
- Davenport M, Puricelli V, Farrant P, Hadzic N, Mieli-Vergani G, Portmann B, et al. The outcome of the older (> or =100 days) infant with biliary atresia. J Pediatr Surg 2004;39:575-81. https://doi.org/10.1016/j.jpedsurg.2003.12.014
- Okazaki T, Kobayashi H, Yamataka A, Lane GJ, Miyano T. Long-term postsurgical outcome of biliary atresia. J Pediatr Surg 1999;34:312-5. https://doi.org/10.1016/S0022-3468(99)90198-7
- Uchida K, Urata H, Suzuki H, Inoue M, Konishi N, Araki T, et al. Predicting factor of quality of life in long-term jaundice-free survivors after the Kasai operation. J Pediatr Surg 2004;39:1040-4. https://doi.org/10.1016/j.jpedsurg.2004.03.055
- Caruso M, Cuocolo R, Di Dato F, Mollica C, Vallone G, Romeo V, et al. Ultrasound, shear-wave elastography, and magnetic resonance imaging in native liver survivor patients with biliary atresia after Kasai portoenterostomy: correlation with medical outcome after treatment. Acta Radiol 2020;61:1300-8. https://doi.org/10.1177/0284185120902379
- Gunadi, Sirait DN, Budiarti LR, Paramita VMW, Fauzi AR, Ryantono F, et al. Histopathological findings for prediction of liver cirrhosis and survival in biliary atresia patients after Kasai procedure. Diagn Pathol 2020;15:79.
- Hukkinen M, Kerola A, Lohi J, Jahnukainen T, Heikkila P, Pakarinen MP. Very low bilirubin after portoenterostomy improves survival of the native liver in patients with biliary atresia by deferring liver fibrogenesis. Surgery 2019;165:843-50. https://doi.org/10.1016/j.surg.2018.10.032
- Ferreira AR, Queiroz TCN, Vidigal PVT, Ferreira RDP, Wanderley DC, Fagundes EDT. Multivariate analysis of biliary flow-related factors and post-Kasai survival in biliary atresia patients. Arq Gastroenterol 2019;56:71-8. https://doi.org/10.1590/s0004-2803.201900000-18
- Nguyen AHP, Pham YHT, Vu GH, Nguyen MH, Hoang TN, Holterman A. Biliary atresia liver histopathological determinants of early post-Kasai outcome. J Pediatr Surg 2021;56:1169-73. https://doi.org/10.1016/j.jpedsurg.2021.03.039
- Ramachandran P, Unny AK, Vij M, Safwan M, Balaji MS, Rela M. α-Smooth muscle actin expression predicts the outcome of Kasai portoenterostomy in biliary atresia. Saudi J Gastroenterol 2019;25:101-5. https://doi.org/10.4103/sjg.SJG_242_18
- Udomsinprasert W, Angkathunyakul N, Klaikeaw N, Vejchapipat P, Poovorawan Y, Honsawek S. Hepatic glypican-3 and alpha-smooth muscle actin overexpressions reflect severity of liver fibrosis and predict outcome after successful portoenterostomy in biliary atresia. Surgery 2020;167:560-8. https://doi.org/10.1016/j.surg.2019.10.013
- Wu JF, Lee CS, Lin WH, Jeng YM, Chen HL, Ni YH, et al. Transient elastography is useful in diagnosing biliary atresia and predicting prognosis after hepatoportoenterostomy. Hepatology 2018;68:616-24.
- Zhou W, Li X, Zhang N, Liao B, Xie X, Zhang X, et al. The combination of conventional ultrasound and shear-wave elastography in evaluating the segmental heterogeneity of liver fibrosis in biliary atresia patients after Kasai portoenterostomy. Pediatr Surg Int 2021;37:1099-108. https://doi.org/10.1007/s00383-021-04920-3
- Shimadera S, Iwai N, Deguchi E, Kimura O, Ono S, Furukawa T, et al. Predicting factors on the occurrence of cystic dilatation of intrahepatic biliary system in biliary atresia. Pediatr Surg Int 2010;26:611-4. https://doi.org/10.1007/s00383-010-2601-x
- Azarow KS, Phillips MJ, Sandler AD, Hagerstrand I, Superina RA. Biliary atresia: should all patients undergo a portoenterostomy? J Pediatr Surg 1997;32:168-72; discussion 172-4. https://doi.org/10.1016/S0022-3468(97)90173-1
- Santo K, Nakano N, Kasahara M, Sakamoto S, Fukuda A, Kanamori Y, et al. Segmental atrophy of explanted livers in biliary atresia: pathological data from 63 cases of failed portoenterostomy. J Pediatr Gastroenterol Nutr 2021;72:88-94. https://doi.org/10.1097/MPG.0000000000002929
- Lang T, Kappler M, Dietz H, Harms HK, Bertele-Harms R. Biliary atresia: which factors predict the success of a Kasai operation? An analysis of 36 patients. Eur J Med Res 2000;5:110-4.
- Wells G, Shea B, O'Connell D, Peterson J, Welch V, Losos M, et al. The Newcastle-Ottawa Scale (NOS) for assessing the quality of nonrandomised studies in meta-analyses. Ottawa: Ottawa Hospital Research Institute, 2000.
- Stang A. Critical evaluation of the Newcastle-Ottawa scale for the assessment of the quality of nonrandomized studies in meta-analyses. Eur J Epidemiol 2010;25:603-5. https://doi.org/10.1007/s10654-010-9491-z
- Wang J, Leeflang M. Recommended software/packages for meta-analysis of diagnostic accuracy. J Lab Precis Med 2019;4:22.
- Meyers RL, Book LS, O'Gorman MA, Jackson WD, Black RE, Johnson DG, 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;38:406-11. https://doi.org/10.1053/jpsu.2003.50069
- Oh M, Hobeldin M, Chen T, Thomas DW, Atkinson JB. The Kasai procedure in the treatment of biliary atresia. J Pediatr Surg 1995;30:1077-80; discussion 1080-1. https://doi.org/10.1016/0022-3468(95)90345-3
- Serinet MO, Broue P, Jacquemin E, Lachaux A, Sarles J, Gottrand F, et al. Management of patients with biliary atresia in France: results of a decentralized policy 1986-2002. Hepatology 2006;44:75-84. https://doi.org/10.1002/hep.21219
- Shteyer E, Ramm GA, Xu C, White FV, Shepherd RW. Outcome after portoenterostomy in biliary atresia: pivotal role of degree of liver fibrosis and intensity of stellate cell activation. J Pediatr Gastroenterol Nutr 2006;42:93-9. https://doi.org/10.1097/01.mpg.0000189324.80323.a6
- Volpert D, White F, Finegold MJ, Molleston J, DeBaun M, Perlmutter DH. Outcome of early hepatic portoenterostomy for biliary atresia. J Pediatr Gastroenterol Nutr 2001;32:265-9. https://doi.org/10.1097/00005176-200103000-00006
- Apostu RC, Fagarasan V, Ciuce CC, Drasovean R, Gheban D, Scurtu RR, et al. Biological and histological assessment of the hepatoportoenterostomy role in biliary atresia as a stand-alone procedure or as a bridge toward liver transplantation. Medicina (Kaunas) 2020;57:16.
- Jaramillo C, Guthery SL, Lowichik A, Stoddard G, Kim T, Li Y, et al. Quantitative liver fibrosis using collagen hybridizing peptide to predict native liver survival in biliary atresia: a pilot study. J Pediatr Gastroenterol Nutr 2021;70:87-92.
- Lemoine C, Melin-Aldana H, Brandt K, Mohammad S, Superina R. The evolution of early liver biopsy findings in babies with jaundice may delay the diagnosis and treatment of biliary atresia. J Pediatr Surg 2020;55:866-72. https://doi.org/10.1016/j.jpedsurg.2020.01.027
- Patel KR, Harpavat S, Khan Z, Dhingra S, Quintanilla N, Firan M, et al. Biliary atresia patients with successful Kasai portoenterostomy can present with features of obliterative portal venopathy. J Pediatr Gastroenterol Nutr 2020;71:91-8. https://doi.org/10.1097/MPG.0000000000002701
- Suda K, Muraji T, Ohtani H, Aiyoshi T, Sasaki T, Toma M, et al. Histological significance of hepatitis-like findings in biliary atresia: an analysis of 34 Japanese cases. Pediatr Int 2019;61:364-8. https://doi.org/10.1111/ped.13816
- Ueno T, Toyama C, Yoneyama T, Deguchi K, Nomura M, Saka R, et al. Impact of serum autotaxin level correlating with histological findings in biliary atresia. J Pediatr Surg 2021;56:1174-8.
- Kobayashi H, Hayashi N, Hayashi K, Yamataka A, Lane GJ, Miyano T. Connective tissue growth factor and progressive fibrosis in biliary atresia. Pediatr Surg Int 2005;21:12-6. https://doi.org/10.1007/s00383-004-1254-z
- Kerola A, Lohi J, Heikkila P, Mutanen A, Jalanko H, Pakarinen MP. Divergent expression of liver transforming growth factor superfamily cytokines after successful portoenterostomy in biliary atresia. Surgery 2019;165:905-11. https://doi.org/10.1016/j.surg.2018.12.003
- Lee JYJ, Sullivan K, El Demellawy D, Nasr A. The value of preoperative liver biopsy in the diagnosis of extrahepatic biliary atresia: a systematic review and meta-analysis. J Pediatr Surg 2016;51:753-61. https://doi.org/10.1016/j.jpedsurg.2016.02.016
- Webb NL, Jiwane A, Ooi CY, Nightinghale S, Adams SE, Krishnan U. Clinical significance of liver histology on outcomes in biliary atresia. J Paediatr Child Health 2017;53:252-6. https://doi.org/10.1111/jpc.13371
- Pape L, Olsson K, Petersen C, von Wasilewski R, Melter M. Prognostic value of computerized quantification of liver fibrosis in children with biliary atresia. Liver Transpl 2009;15:876-82. https://doi.org/10.1002/lt.21711
- Russo P, Magee JC, Anders RA, Bove KE, Chung C, Cummings OW, et al. Key histopathologic features of liver biopsies that distinguish biliary atresia from other causes of infantile cholestasis and their correlation with outcome: a multicenter study. Am J Surg Pathol 2016;40:1601-15. https://doi.org/10.1097/PAS.0000000000000755
- Hukkinen M, Kerola A, Lohi J, Heikkila P, Merras-Salmio L, Jahnukainen T, et al. Treatment policy and liver histopathology predict biliary atresia outcomes: results after national centralization and protocol biopsies. J Am Coll Surg 2018;226:46-57.e1. https://doi.org/10.1016/j.jamcollsurg.2017.09.009
- Kerola A, Lampela H, Lohi J, Heikkila P, Mutanen A, Jalanko H, et al. Molecular signature of active fibrogenesis prevails in biliary atresia after successful portoenterostomy. Surgery 2017;162:548-56. https://doi.org/10.1016/j.surg.2017.04.013
- Jeon TY, Yoo SY, Kim JH, Eo H, Lee SK. Serial ultrasound findings associated with early liver transplantation after Kasai portoenterostomy in biliary atresia. Clin Radiol 2013;68:588-94. https://doi.org/10.1016/j.crad.2012.12.004
- Hukkinen M, Pihlajoki M, Pakarinen MP. Predicting native liver injury and survival in biliary atresia. Semin Pediatr Surg 2020;29:150943.
- Luo Z, Shivakumar P, Mourya R, Gutta S, Bezerra JA. Gene expression signatures associated with survival times of pediatric patients with biliary atresia identify potential therapeutic agents. Gastroenterology 2019;157:1138-52.e14. https://doi.org/10.1053/j.gastro.2019.06.017
- Duche M, Fabre M, Kretzschmar B, Serinet MO, Gauthier F, Chardot C. Prognostic value of portal pressure at the time of Kasai operation in patients with biliary atresia. J Pediatr Gastroenterol Nutr 2006;43:640-5. https://doi.org/10.1097/01.mpg.0000235754.14488.f9
- Vazquez-Estevez J, Stewart B, Shikes RH, Hall RJ, Lilly JR. Biliary atresia: early determination of prognosis. J Pediatr Surg 1989;24:48-50; discussion 50-1. https://doi.org/10.1016/S0022-3468(89)80300-8
- Mukhopadhyay SG, Roy P, Chatterjee U, Datta C, Banerjee M, Banerjee S, et al. A histopathological study of liver and biliary remnants in the long-term survivors (>10 years) of cases of biliary atresia. Indian J Pathol Microbiol 2014;57:380-5. https://doi.org/10.4103/0377-4929.138722
- Santos JL, Kieling CO, Meurer L, Vieira S, Ferreira CT, Lorentz A, et al. The extent of biliary proliferation in liver biopsies from patients with biliary atresia at portoenterostomy is associated with the postoperative prognosis. J Pediatr Surg 2009;44:695-701. https://doi.org/10.1016/j.jpedsurg.2008.09.013
- Kerola A, Lampela H, Lohi J, Heikkila P, Mutanen A, Hagstrom J, et al. Increased MMP-7 expression in biliary epithelium and serum underpins native liver fibrosis after successful portoenterostomy in biliary atresia. J Pathol Clin Res 2016;2:187-98. https://doi.org/10.1002/cjp2.50
- Wu JF, Jeng YM, Chen HL, Ni YH, Hsu HY, Chang MH. Quantification of serum matrix metallopeptide 7 Levels may assist in the diagnosis and predict the outcome for patients with biliary atresia. J Pediatr 2019;208:30-7.e1. https://doi.org/10.1016/j.jpeds.2018.12.006
- Bessho K, Bezerra JA. Biliary atresia: will blocking inflammation tame the disease? Annu Rev Med 2011;62:171-85. https://doi.org/10.1146/annurev-med-042909-093734
- Wu JF, Kao PC, Chen HL, Lai HS, Hsu HY, Chang MH, et al. A high serum interleukin-12p40 level prior to Kasai surgery predict a favourable outcome in children with biliary atresia. Liver Int 2012;32:1557-63. https://doi.org/10.1111/liv.12001
- Goda SS, Khedr MA, Elshenawy SZ, Ibrahim TM, El-Araby HA, Sira MM. Preoperative serum IL-12p40 Is a potential predictor of Kasai portoenterostomy outcome in infants with biliary atresia. Gastroenterol Res Pract 2017;2017:9089068.
- Deeks JJ, Higgins JPT, Altman DG. Chapter 10: analysing data and undertaking meta-analyses. In: Higgins JPT, Thomas J, Chandler J, Cumpston M, Li T, Page MJ, et al., eds. Cochrane handbook for systematic reviews of interventions version 6.2. London: Cochrane, 2021.
- Sarkhy A, Schreiber RA, Milner RA, Barker CC. Does adjuvant steroid therapy post-Kasai portoenterostomy improve outcome of biliary atresia? Systematic review and meta-analysis. Can J Gastroenterol 2011;25:440-4. https://doi.org/10.1155/2011/125610
- Zhang D, Yang HY, Jia J, Zhao G, Yue M, Wang JX. Postoperative steroids after Kasai portoenterostomy for biliary atresia: a meta-analysis. Int J Surg 2014;12:1203-9. https://doi.org/10.1016/j.ijsu.2014.08.407
- Chen Y, Nah SA, Chiang L, Krishnaswamy G, Low Y. Postoperative steroid therapy for biliary atresia: systematic review and meta-analysis. J Pediatr Surg 2015;50:1590-4. https://doi.org/10.1016/j.jpedsurg.2015.05.016
- Lopez RN, Ooi CY, Krishnan U. Early and peri-operative prognostic indicators in infants undergoing hepatic portoenterostomy for biliary atresia: a review. Curr Gastroenterol Rep 2017;19:16.