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Biliary atresia remains one of the most challenging conditions in pediatric surgery. The incidence ranges from 1 in 5,000 in Asia to about 1 in 16,000 live births in Europe with a slight female preponderance. The cause is still unknown with a number of possible causes giving rise to a range of different presentations. It is usually an isolated condition but can be associated with other anomalies.
Currently the management is almost entirely surgical with an initial attempt to restore bile flow and preserve the native liver using a Kasai portoenterostomy. Liver transplantation is an option if this fails or for those infants who present late with obvious end-stage cirrhosis. Beyond effective surgical technique, the role of adjuvant medical therapy is unclear and evidence of benefit is lacking. Despite this the use of postoperative steroids, prophylactic antibiotics, and choleretic agents such as ursodeoxycholic acid is common.
Experience from high-volume centers suggests clearance of jaundice can be achieved in 50–60% of infants with 10-year native liver and real survival rates of 40% and 90%, respectively.
KeywordsBiliary atresia Kasai portoenterostomy Surgical jaundice Cirrhosis BASM Cystic biliary atresia
- Davenport M, Savage M, Mowat AP, Howard ER. The biliary atresia splenic malformation syndrome. Surg. 1993;113:662–8.Google Scholar
- Kasai M, Suzuki S. A new operation for “non-correctable” biliary atresia – portoenterostomy. Shijitsu. 1959;13:733–9.Google Scholar
- Ng VL, Haber BH, Magee JC, et al. Childhood Liver Disease Research and Education Network (CHiLDREN). Medical status of 219 children with biliary atresia surviving long-term with their native livers: results from a North American multicenter consortium. J Pediatr. 2014;165:539–46.Google Scholar
- 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;32:1555–9.CrossRefPubMedGoogle Scholar
- Russo P, Magee JC, Anders RA, et al. Childhood Liver Disease Research Network (ChiLDReN). 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(12):1601–15.Google Scholar
- Shneider BL, Abel B, Haber B, et al. Childhood Liver Disease Research and Education Network. Cross-sectional multi-center analysis of portal hypertension in children and young adults with biliary atresia. J Pediatr Gastroenterol Nutr. 2012b;55: 567–73.Google Scholar
- Stagg H, Cameron BH, Ahmed N, et al. Canadian Biliary Atresia Registry. Variability of diagnostic approach, surgical technique, and medical management for children with biliary atresia in Canada – is it time for standardization? J Pediatr Surg. 2017;pii: S0022-3468(17)30074–X.Google Scholar
- 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;254:577–85.CrossRefPubMedPubMedCentralGoogle Scholar
- Thomson J. On congenital obliteration of the bile ducts. Edinburgh Med J. 1891;37:523–31.Google Scholar