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Kasai Procedure in Patients Older Than 90 Days: Worth a Cut
Marie Uecker1, Joachim F Kuebler1, Nagoud Schukfeh1
1Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany.
Insights
Kasai portoenterostomy (KPE) in infants older than 90 days with biliary atresia (BA) can extend native liver survival and serves as a bridge to liver transplantation (LT). This approach remains a viable option for late-presenting BA patients.
Area of Science:
- Pediatric Surgery
- Hepatology
- Gastroenterology
Background:
- Biliary atresia (BA) management often involves Kasai portoenterostomy (KPE), with age at surgery being crucial for native-liver survival.
- Improved pediatric liver transplantation (LT) outcomes raise questions about primary LT for late-presenting BA infants versus KPE.
- This study evaluates KPE outcomes in BA infants older than 90 days.
Purpose of the Study:
- To assess the efficacy of KPE in infants with biliary atresia diagnosed later than 90 days of age.
- To determine the impact of delayed KPE on native liver survival and the need for subsequent liver transplantation.
- To evaluate KPE as a primary treatment option for late-presenting BA.
Main Methods:
- Retrospective chart review of BA patients undergoing KPE between January 2010 and December 2020.
- Inclusion criteria: patients aged 90 days or older at the time of KPE.
- Data collected included patient characteristics, perioperative details, and follow-up outcomes.
Main Results:
- Eleven patients met the criteria, with a mean age of 108 days at KPE.
- Two-year jaundice clearance was achieved in 27% of patients.
- 73% of patients required liver transplantation, with a median of 626 days post-KPE; 36% transplanted within 1 year.
- Two deaths occurred due to disease-related complications; one patient maintained native liver function at 10 years old.
Conclusions:
- Kasai portoenterostomy (KPE) in older infants (≥90 days) with biliary atresia (BA) can prolong native liver survival.
- KPE serves as an effective bridge to liver transplantation (LT) for these patients.
- Delayed KPE remains a viable primary treatment option for late-presenting BA infants.
Introduction:
Age at Kasai portoenterostomy (KPE) has been identified as a predictive factor for native-liver survival in patients with biliary atresia (BA). Outcomes of pediatric liver transplantation (LT) have improved over recent years. It has been proposed to consider primary LT as a treatment option for late-presenting BA infants instead of attempting KPE. We present our experience with patients older than 90 days undergoing KPE.
Materials And Methods:
A retrospective chart review of patients with BA undergoing KPE at our institution between January 2010 and December 2020 was performed. Patients 90 days and older at the time of surgery were included. Patients' characteristics, perioperative data, and follow-up results were collected. Eleven patients matched the inclusion criteria. Mean age at KPE was 108 days (range: 90-133 days).
Results:
Postoperative jaundice clearance (bilirubin < 2 mg/dL) at 2-year follow-up was achieved in three patients (27%). Eight patients (73%) received a liver transplant at a mean of 626 days (range: 57-2,109 days) after KPE. Four patients (36%) were transplanted within 12 months post-KPE. Two patients died 237 and 139 days after KPE due to disease-related complications. One patient is still alive with his native liver, currently 10 years old.
Conclusion:
Even when performed at an advanced age, KPE can help prolong native-liver survival in BA patients and offers an important bridge to transplant. In our opinion, it continues to represent a viable primary treatment option for late-presenting infants with BA.
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