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The Assessment and Management of Biliary Atresia in Hawai'i, 2009-2023
Sneha A Singh1, Kalpana K Balaraman2, Madeline I Johnson2
1General Surgery, Tripler Army Medical Center, Honolulu, HI (SAS).
Insights
Biliary atresia (BA) diagnosis and Kasai procedure in Hawaii occur significantly later than recommended for optimal outcomes. Implementing a statewide screening program for BA in Hawaii is crucial for early intervention and improved pediatric liver health.
Area of Science:
- Pediatric Hepatology
- Neonatal Surgery
- Public Health
Background:
- Biliary atresia (BA) is a rare neonatal liver disorder, a leading cause of pediatric end-stage liver disease.
- Early diagnosis and the Kasai procedure can decrease the need for liver transplants in children.
- Optimal outcomes are associated with the Kasai procedure performed between 30-45 days of life.
Purpose of the Study:
- To analyze the age of BA diagnosis and Kasai procedure in Hawaii.
- To compare current Hawaii practices with established optimal timing for BA treatment.
- To assess the need for a statewide BA screening program in Hawaii.
Main Methods:
- Retrospective chart review of BA patients at Hawaii's primary children's hospital (2009-2023).
- Identification of patients who underwent the Kasai procedure.
- Analysis of mean age at diagnosis and mean age at Kasai procedure.
Main Results:
- The mean age of BA diagnosis in Hawaii was 71.4 days (n=19).
- The mean age for the Kasai procedure in Hawaii was 72.0 days (n=19).
- These ages are significantly later than the recommended 30-45 days for best outcomes.
Conclusions:
- Current BA diagnosis and treatment ages in Hawaii fall short of optimal benchmarks.
- The incidence of BA in Hawaii is notably higher than in the continental US.
- A statewide screening program for BA in Hawaii is strongly recommended to improve patient outcomes.
Abstract:
Although biliary atresia (BA) is a rare neonatal disorder, it remains the leading cause of pediatric end-stage liver disease. Early diagnosis of BA and treatment with the Kasai procedure can significantly reduce the need for pediatric liver transplant. Current data suggests that performing the Kasai procedure at 30-45 days of life is associated with longer native liver survival rates and reduction of the need for liver transplant. The incidence rate of BA in the state of Hawai'i is nearly double the incidence rate in the continental US. International studies have demonstrated that screening programs for BA reduce the age at diagnosis and treatment. However, there has been no statewide analysis on the ages at diagnosis or at Kasai, nor does a statewide screening program for BA exist. The purpose of this study is to review the age of diagnosis and treatment of BA to determine if the current practice in Hawai'i is in line with the published data. A retrospective chart review of all patients diagnosed with BA at the state's primary children's hospital was performed (2009-2023) and 19 patients who underwent the Kasai procedure were identified. The mean age at diagnosis is 71.4 days (n=19) and the mean age at Kasai procedure is 72.0 days (n=19). Both the average age at diagnosis and treatment for BA in Hawai'i is significantly higher than published data suggesting best outcomes at 30-45 days of life. This review suggests that the implementation of a statewide screening program for BA in Hawai'i is warranted.
