Past and future of biliary atresia
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Published on: August 23, 2022
A Carceller1, H Blanchard, F Alvarez
1Division of Pediatrics, General Surgery, and Gastroenterology, Ste-Justine Hospital, Montreal, Quebec, Canada.
Insights
Kasai hepatic portoenterostomy (KHPE) remains a valuable treatment for biliary atresia (BA) in infants. Successful KHPE improves long-term survival by enabling transplantation at an optimal age, reducing mortality and morbidity.
Area of Science:
- Pediatric surgery
- Hepatology
- Gastroenterology
Background:
- Liver transplantation has improved outcomes for children with biliary atresia (BA).
- The continued value of Kasai hepatic portoenterostomy (KHPE) in BA treatment is questioned.
- Assessing the long-term efficacy of KHPE in pediatric BA management is crucial.
Purpose of the Study:
- To evaluate the long-term outcomes of Kasai hepatic portoenterostomy (KHPE) in infants with biliary atresia (BA).
- To determine if KHPE remains a viable option in the era of liver transplantation for BA.
- To analyze the impact of successful KHPE on subsequent liver transplantation and overall survival.
Main Methods:
- Retrospective analysis of 77 pediatric patients with biliary atresia treated between 1974 and 1998.
- Detailed review of surgical procedures, including Kasai hepatic portoenterostomy (KHPE) and its variations.
- Assessment of patient outcomes, including need for orthotopic liver transplantation (OLT), survival rates, and age at transplantation or failure.
Main Results:
- 74 patients underwent KHPE, with 11 considered failures. 17 patients survived long-term post-KHPE.
- 33 patients (43%) ultimately required orthotopic liver transplantation (OLT).
- Overall survival for KHPE plus OLT was 55%. Successful KHPE correlated with improved survival (P < .001) and allowed transplantation at a mean age of 9 years, versus 11 months for failed KHPE.
Conclusions:
- Kasai hepatic portoenterostomy (KHPE) remains a valuable initial surgical procedure for infants with biliary atresia (BA).
- Successful KHPE facilitates liver transplantation at an older age, which is associated with decreased mortality and morbidity.
- KHPE can significantly improve long-term outcomes for children with BA, even with the availability of liver transplantation.
Background:
With the advent of liver transplantation the outcome of children with biliary atresia (BA) has improved. Is Kasai hepatic portoenterostomy (KHPE) still a valuable option for the treatment of these patients?
Methods:
From 1974 to 1998, 77 patients with biliary atresia have been treated at our institution: 50 girls and 27 boys.
Results:
Seventy-four patients had a KHPE, and 3 patients had no KHPE because of delay in diagnosis. A total of 65 of 74 patients (88%) had undergone KHPE type I, 4 patients (5.4%) KHPE type II, 3 patients (4%) had a Suruga modification, and 2 patients (2.6%) had a portocholecystostomy. Among the 74 patients, 11 were lost to follow-up and their cases were considered failures. Seventeen of our patients are alive at long-term follow-up after KHPE. Among the 77 patients, 33 (43%) had an orthotopic liver transplantation (OLT). Successful KHPE patients underwent transplant at a mean age of 9 years, and KHPE failed at a mean age of 11 months. A total of 25 of 77 (32%) of patients are alive thanks to OLT. In the cohort, the overall survival rate for the KHPE plus OLT is 42 of 77 (55%). Mortality and morbidity rates were more frequent among the younger patients who had early OLT after KHPE failure. In our series, overall survival rate was improved when the patient had a successful KHPE (P < .001).
Conclusions:
Kasai hepatic portoenterostomy (KHPE) continues to be a valuable procedure in the treatment of infants with biliary atresia (BA). Successful KHPE permits transplantation at an age at which mortality and morbidity are decreased leading to a better outcome.
