Complications and Quality of Life in Long-Term Survivors of Biliary Atresia with Their Native Livers

Abstract

Biliary atresia is a rare neonatal disease of unknown etiology that results from a fibroinflammatory obstruction of extrahepatic bile ducts in the first few weeks of life. It is one of the most common causes of neonatal cholestasis, and if untreated, it rapidly proceeds to biliary cirrhosis, which could be fatal within the first few years. About 50 years ago, Japanese surgeons Morio Kasai and Sozo Suzuki introduced a surgical intervention with hepatoportoenterostomy to restore biliary flow to the intestine and prevent further liver damage. 1 Kasai M. Suzuki S. A new operation for non-correctable biliary atresia-hepatic portoenterostomy. Shujutsu. 1959; 13: 733-739 Google Scholar This intervention, which has been subsequently modified, 2 Lilly J.R. Hepatic portocholecystostomy for biliary atresia. J Pediatr Surg. 1979; 14: 301-304 Abstract Full Text PDF PubMed Scopus (33) Google Scholar , 3 Saeki M. Nakano M. Hagane K. Shimizu K. Effectiveness of an intussusceptive antireflux valve to prevent ascending cholangitis after hepatic portojejunostomy in biliary atresia. J Pediatr Surg. 1991; 26: 800-803 Abstract Full Text PDF PubMed Scopus (27) Google Scholar , 4 Ogasawara Y. Yamataka A. Tsukamoto K. Okada Y. Lane G.J. Kobayashi H. et al. The intussusception antireflux valve is ineffective for preventing cholangitis in biliary atresia: a prospective study. J Pediatr Surg. 2003; 38: 1826-1829 Abstract Full Text Full Text PDF PubMed Scopus (40) Google Scholar is still called the “Kasai procedure.” The worldwide dissemination of the Kasai procedure dramatically changed the outcome of biliary atresia. This procedure is not a cure, however, and even with prompt surgical intervention, ongoing injury to the intrahepatic bile ducts and progressive cholangiopathy leads to end-stage cirrhosis and the need for liver transplantation by 2 years of age in approximately 50% of affected children. 5 Shneider B.L. Mazariegos G.V. Biliary atresia: a transplant perspective. Liver Transpl. 2007; 13: 1482-1495 Crossref PubMed Scopus (55) Google Scholar , 6 Nio M. Ohi R. Miyano T. Saeki M. Shiraki K. Tanaka K. et al. 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 Abstract Full Text Full Text PDF PubMed Scopus (267) Google Scholar In contrast, those who can clear jaundice after surgery have been expected to survive long term to adulthood with good health status and quality of life. 7 Davenport M. Biliary atresia: outcome and management. Indian J Pediatr. 2006; 73: 825-828 Crossref PubMed Scopus (23) Google Scholar Recent multicenter studies, however, have found that the majority of those who escaped the need for early transplantation showed some evidence of chronic liver disease. 8 Ng V.L. Haber B.H. Magee J.C. Miethke A. Murray K.F. Michail S. et al. 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: 539-546.e2 Abstract Full Text Full Text PDF PubMed Scopus (52) Google Scholar , 9 Bijl E.J. Bharwani K.D. Houwen R.H. de Man R.A. The long-term outcome of the Kasai operation in patients with biliary atresia: a systematic review. Neth J Med. 2013; 71: 170-173 PubMed Google Scholar