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[Prognosis in children with biliary atresia successfully treated with Kasai's operation]
J Murcia1, M López Santamaría, M Gámez
1Departamento de Cirugía, Hospital Infantil La Paz, Madrid.
Insights
The Kasai procedure (portoenterostomy) offers long-term survival for biliary atresia patients. While many maintain liver function, some require transplants, but overall prognosis remains excellent for biliary atresia treatment.
Area of Science:
- Pediatric Surgery
- Hepatology
- Gastroenterology
Context:
- The Kasai procedure (portoenterostomy) is a surgical technique for biliary atresia.
- Long-term outcomes for biliary atresia treated with portoenterostomy are not widely documented.
- Liver transplantation is an available treatment option for biliary atresia.
Purpose:
- To evaluate the long-term evolution of patients with biliary atresia treated with portoenterostomy.
- To assess survival rates and liver function in patients who did not undergo liver transplantation post-portoenterostomy.
Summary:
- This study reviewed 22 patients surviving over 10 years after portoenterostomy for biliary atresia without liver transplant.
- Seven patients eventually required transplantation due to progressive liver damage or hepatopulmonary syndrome.
- Fifteen patients maintained compensated liver function, with five showing no hypersplenism and normal bilirubin levels.
Impact:
- Demonstrates that a subset of biliary atresia patients can achieve excellent long-term outcomes without liver transplantation after portoenterostomy.
- Highlights the importance of continued monitoring for potential complications like liver damage and hepatopulmonary syndrome.
- Provides valuable data on the long-term efficacy and prognosis of the Kasai procedure in managing biliary atresia.
Background:
The Kasai procedure, portoenteroanastomosis (PEA) didn't reach international spreading until the seventy's decade, making difficult to find long-term results from children with ABE successfully treated with this technique. At our institution in the last fifteen years all the therapeutics procedures for these patients can be offered, including the liver transplant.
Aim:
To show the evolution of our patients with ABE treated with the PEA and that survive long-term without being transplanted.
Methods:
The clinical course of 22 patients that survive more than 10 years after the PEA with their own liver is reviewed. The hepatic survival indexes of (success, death or transplant) are beyond the tenth year. The problems raised during the follow-up are analysed.
Results:
From 99 patients with ABE treated primarily in our center, 22 reached the 10 year-old age after the PEA without a liver transplant. In the follow-up, seven if the these finally needed the transplant. Their median age was 12.2 year-old (range: 10.5-13.8) for a progressive hepatocellular damage in 5 cases associated to syndrome hepatopulmonar in two cases. The other fifteen patients have a compensated hepatopathy. Five of them do not have hyperesplenisme and the serum bilirrubine levels are lower than 1.3 mg/dL. The medium age of these patients at the end of the follow-up was 14.8 years.
Conclusions:
In spite of the reestablishment of the biliary flow with the PEA, few are the patients with ABE that preserve their hepatic function lapsed long periods of time. Nevertheless the prognosis of these patients is excellent.