Remote results of treatment of biliary atresia in children

• Main Authors: A. Yu. Razumovskiy, A. V. Degtyareva, N. V. Kulikova, V. E. Rachkov, S. A. Ratnikov, E. A. Filippova, A. A. Puchkova, M. V. Albegova
• Format: Article
• Language: Russian
• Published: Ltd. “The National Academy of Pediatric Science and Innovation” 2019-03-01
• Series: Rossijskij Vestnik Perinatologii i Pediatrii
• Subjects: children; biliary atresia; laparoscopy; kasai procedure; mini access; laparotomy; jaundice; acholic stool; cholestasis; cholangitis; portal hypertension; survival; catamnesis
• Online Access: https://www.ped-perinatology.ru/jour/article/view/819
• ISSN: 1027-4065; 2500-2228

The objective. To analyze early and long-term results of surgical treatment of biliary atresia in children. 120 patients with biliary atresia underwent Kasai procedure from 2000 to 2018. The follow-up course varied from 6 months to 15 years. The authors assessed the survival rate of children with native liver and the survival rate without indications for liver transplantation in different age periods, as well as the frequency of surgical complications, bacterial cholangitis, portal hypertension, etc.The results. The survival rate of children with native liver within 1 year was 82.7%; within 2 years – 57.72%; 3 years – 49.6%; 5 years – 42.1%; over 10 years – 33.25%. In case of effective surgery the authors noted the restoration of stool color, jaundice relief and gradual normalization of bilirubin level during the 1st year. The activity of gamma-glutamyl transferase and transaminases in most children increased in the early postoperative period and, then, gradually decreased. The most frequent postoperative complications were episodes of cholangitis and portal hypertension. During the first year, episodes of cholangitis were detected in 50 (42.3%) of 119 children, regardless of the effectiveness of the Kasai procedure. 56 children (47.75%) had signs of portal hypertension during the first year of life; its frequency increased to 70% by the age of 5–10 years.Conclusion. In most cases, Kasai procedure prolongs life with the native liver, allowing you to postpone transplantation and it is an effective method of treatment of biliary atresia in children. The most frequent complications are cholangitis and portal hypertension, the early detection and timely correction of them is an integral part of the treatment of these patients.