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Published on: February 10, 2023
Predicting Cirrhosis and Poor Outcomes of Bile Drainage Surgery for Biliary Atresia: A Multicentric Observational
Hirofumi Tomita1, Naoki Shimojima1, Hideyuki Sasaki2
1Department of Surgery, Tokyo Metropolitan Children's Medical Center, Fuchu, Tokyo, Japan.
Insights
The infant BA liver fibrosis (iBALF) score can identify biliary atresia patients with poor bile drainage surgery outcomes. A score above 5.27 indicates a need for potential primary liver transplantation.
Area of Science:
- Pediatric Hepatology
- Surgical Outcomes Research
- Biomarker Development
Background:
- Biliary atresia (BA) management remains challenging, with ongoing debate regarding primary liver transplantation versus bile drainage surgery.
- Identifying patients unlikely to benefit from bile drainage surgery is crucial for optimizing treatment strategies.
Purpose of the Study:
- To evaluate the infant BA liver fibrosis (iBALF) score's utility in predicting outcomes of bile drainage surgery in BA patients.
- To identify BA patients with extremely poor prognoses who may be better suited for primary liver transplantation.
Main Methods:
- Retrospective analysis of 380 BA patients who underwent bile drainage surgery (2015-2019) across 60 hospitals.
- Utilized receiver operating characteristic curve analysis to determine the iBALF score's cutoff for predicting 1-year native liver survival.
- Established a cutoff value of 5.27 for the iBALF score, prioritizing >99% sensitivity.
Main Results:
- Native liver survival at 1 year was achieved in 67.9% of patients.
- An iBALF score >5.27 identified a subgroup of 4.7% of patients with significantly poorer outcomes (1.4% native liver survival).
- Patients with iBALF score >5.27 exhibited higher mortality and required earlier salvage liver transplantation.
Conclusions:
- A preoperative iBALF score >5.27 strongly predicts extremely poor outcomes following bile drainage surgery in biliary atresia.
- These patients represent a high-risk group who should be considered for primary liver transplantation.
Objective:
To identify patients with biliary atresia (BA) with extremely poor outcomes of bile drainage surgery using the infant BA liver fibrosis (iBALF) score, a liver fibrosis marker based on standard blood analysis.
Background:
Although primary liver transplantation is beginning to be considered as an alternative to bile drainage surgery in patients with BA, those most likely to benefit from this procedure have not yet been identified.
Methods:
The medical records of 380 patients with BA with bile drainage surgery between 2015 and 2019 were collected for retrospective analysis from 60 participating hospitals. To predict native liver survival at age 1 year, a receiver operating characteristic curve was drawn for the iBALF score. The cutoff value was determined as the point indicating >99% sensitivity.
Results:
The median age at surgery was 56 days (range: 4-183 days), and native liver survival at age 1 year was achieved in 258 (67.9%) patients. An iBALF score of 5.27 was chosen as the cutoff, and 18 patients (4.7%) were found to have an iBALF score >5.27; of these, only 2 (95% CI: 1.4%-34.7%) had native liver survival at age 1 year, indicating a significantly poorer outcome than in the other patients (95% CI: 65.7%-75.4%). Moreover, patients with an iBALF score >5.27 had significantly higher mortality and younger age at salvage liver transplantation.
Conclusions:
Patients with BA having a preoperative iBALF score >5.27 had extremely poor outcomes of bile drainage surgery and may be considered candidates for primary LTx.
