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Age at surgery and native liver survival in biliary atresia: a systematic review and meta-analysis
Eri Hoshino1,2, Yamato Muto3, Kotomi Sakai4
1Comprehensive Unit for Health Economic Evidence Review and Decision Support (CHEERS), Ritsumeikan University, Kyoto, Japan. erihoshino119@gmail.com.
Insights
Early Kasai procedure (KP) before 30 days of life significantly improves native liver survival (NLS) in infants with biliary atresia (BA). Prompt diagnosis and surgical intervention are crucial for better long-term outcomes in BA patients.
Area of Science:
- Pediatric Surgery
- Hepatology
- Neonatal Care
Background:
- Biliary atresia (BA) is a rare, progressive liver disease in infants requiring timely surgical treatment.
- The Kasai procedure (KP) is the primary surgical intervention for BA, but optimal timing remains debated.
- Native liver survival (NLS) is a critical outcome measure for BA patients, influenced by various factors including surgical timing.
Approach:
- A systematic review and meta-analysis was conducted to evaluate the relationship between the age at Kasai procedure (KP) and native liver survival (NLS) in biliary atresia (BA) patients.
- Searched major databases (PubMed, EMBASE, Cochrane, Ichushi Web) for studies published from 1968 to May 2022.
- Included studies examining KP timing at specific age intervals and reporting NLS rates and hazard/risk ratios, assessing study quality with the ROBINS-I tool.
Key Points:
- Meta-analysis of nine studies (1653 initial records) revealed that later KP timing was associated with a faster time to liver transplantation (HR=2.12).
- Patients undergoing KP at ≤30 days had significantly better NLS compared to those undergoing KP at ≥31 days (RR=1.22).
- Sensitivity analysis confirmed improved NLS when comparing KP ≤30 days versus 31-60 days (RR=1.13).
Conclusions:
- Early diagnosis and surgical intervention, ideally before 30 days of life, are crucial for improving native liver survival in infants with biliary atresia.
- Effective newborn screening programs for BA are essential to facilitate prompt diagnosis and timely Kasai procedures.
- The findings underscore the importance of early surgical management for optimizing long-term outcomes in BA patients.
Abstract:
Biliary atresia (BA) is a childhood rare disease of the liver and bile ducts that requires prompt surgical intervention. Age at surgery is an important prognostic factor; however, controversy exists with regard to the benefit of early Kasai procedure (KP). We aimed to conduct a systematic review and meta-analysis to examine the relationship between the age at KP and native liver survival (NLS) of BA patients. We performed the electronic database search using Pubmed, EMBASE, Cochrane, and Ichushi Web and included all relevant studies published from 1968 up to May 3, 2022. Studies that examined the timing of KP at ages 30, 45, 60, 75, 90, 120, and/or 150 days were included. The outcome measures of interest were NLS rates at 5, 10, 15, 20, and 30 years post-KP and the hazard ratio or risk ratio for NLS. The quality assessment was used using the ROBINS-I tool. Among 1653 potentially eligible studies, nine articles met the inclusion criteria for the meta-analysis. Meta-analysis for hazard ratios revealed that there was a significantly faster time to liver transplantation in the group of patients who had KP at later timing as compared with earlier KP (HR = 2.12, 95% CI 1.51-2.97). The risk ratio comparing KP ≤ 30 days and KP ≥ 31 days on native liver survival was 1.22 (95% CI 1.13-1.31). The sensitivity analysis showed that comparing KP ≤ 30 days and KP 31-60 days, the risk ratio was 1.13, 95% CI 1.04-1.22. Conclusion: Our meta-analysis showed the importance of early diagnosis and surgical interventions ideally before 30 days of life in infants with BA on native liver survival on 5, 10, and 20 years. Therefore, effective newborn screening of BA targeting KP ≤ 30 days is needed to ensure prompt diagnosis of affected infants. What is Known: • Age at surgery is an important prognostic factor. What is New: • Our study performed an updated systematic review and meta-analysis to examine the relationship between age at Kasai procedure and native liver survival in patients with BA.
