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The surgery of biliary atresia
J R Lilly1, F M Karrer, R J Hall
1Department of Surgery, University of Colorado School of Medicine, Denver 80262.
Insights
Biliary atresia surgery in infants achieved sustained bile drainage in 57% of patients, with 28% showing normal liver function long-term. While not a cure, surgical intervention offers improved quality of life for many children with this condition.
Area of Science:
- Pediatric Surgery
- Hepatology
- Gastroenterology
Background:
- Biliary atresia is a severe neonatal liver disease causing bile duct obstruction.
- Early surgical intervention is crucial for managing biliary atresia and improving outcomes.
- Long-term efficacy of surgical procedures for biliary atresia requires ongoing evaluation.
Purpose of the Study:
- To evaluate the long-term outcomes of surgical management for biliary atresia in infants.
- To assess the rates of bile drainage, sustained obstruction relief, and overall survival.
- To analyze postoperative morbidity and the impact of surgical techniques on liver function.
Main Methods:
- Retrospective analysis of 131 infants with biliary atresia operated on between 1973 and 1988.
- Surgical procedures included excision of extrahepatic bile ducts with biliary drainage via gallbladder-common bile duct conduit or Roux-en-Y portoenterostomy.
- Evaluation of immediate postsurgical bile drainage, reoperation success, sustained obstruction relief, and long-term survival and liver function.
Main Results:
- Immediate bile drainage was achieved in 82% of infants, with reoperation restoring flow in 14 of 18.
- Fifty-seven percent of patients experienced sustained relief of biliary obstruction for over a year.
- Twenty-eight percent of survivors exhibited normal to near-normal liver function, with normal growth and development.
Conclusions:
- Surgical management of biliary atresia, including Kasai's operation, can lead to sustained bile drainage and improved long-term liver function in a significant portion of infants.
- While complete cure is rare, surgical intervention offers a chance for normal development and participation in activities for affected children.
- Postoperative morbidity is substantial, highlighting the need for continued advancements in surgical techniques and patient care.
Abstract:
One hundred thirty-one consecutive infants with biliary atresia were operated on during the 15-year period between 1973 and 1988. Six patients did not have biliary reconstruction because of advanced cirrhosis or transplant preference. The other 125 infants had excision of all nonpatent extrahepatic bile ducts; biliary drainage was provided by a gallbladder-common bile duct conduit in 14 patients and by a Roux-en-Y portoenterostomy in 111 infants (including the seven patients with correctable biliary atresia). The bilioenteric conduit was temporarily exteriorized and, for the past 2 years, a conduit intussusception valve was incorporated. Immediate postsurgical bile drainage was achieved in 103 infants (82%). Reoperation during the first 6 postoperative weeks restored bile flow in 14 of 18 infants who had shut down. Seventy-two patients (57%) had sustained (more than 1 year) relief of biliary obstruction. Postoperative morbidity was substantial. The six children not having corrective surgery died within 19 months. Three patients were lost to follow-up. Sixty-eight patients having Kasai's operation died, 55 from complications of liver disease, 1 from a coexisting malformation, and 12 after liver transplantation. Fifty-seven patients are alive, 13 by virtue of liver replacement, 9 with mild-to-moderate hepatic sequelae, and 35 (28%) with normal to near-normal liver function. Although none is considered "cured," the 35 children are anicteric, have normal growth and development, and participate in full school activities (including contact sports). Average follow-up is 85.8 months (range 1 to 15 years).