Clinical and Histological Factors Predictive of Kasai Procedure Failure

Background: Thirty to 50% of infants with biliary atresia (BA) following a Kasai portoenterostomy (KPE) will undergo liver transplantation before the age of 2. It is currently impossible to predict who will have “early failure” after KPE. We evaluated the predictive nature of clinical and histologic features collected before KPE on jaundice clearance and transplant-free survival.

Methods: A retrospective review of 54 patients who underwent a KPE between 1997 and 2018 was performed. Primary outcomes were 2-year and 5-year transplant-free survival, and jaundice clearance 3 months after KPE. The Chi-square test, Fischer’s exact test, and logistic regression were used. p<0.05 was considered significant. Institutional board review approval was obtained (IRB 2007-12989).

Results: Two and five-year transplant-free survival were 35.1% (19/54) and 24.5% (12/49) respectively. Twenty-eight percent of patients achieved a direct bilirubin (DB) ≤1.0 mg/dL at 3 months while 37.2% achieved a DB ≤2.0 by 3 months post KPE. Median age at biopsy was younger in the 5-year transplant-free survival group (39.0 days [24.5-55.5] vs. 56.0 days [51.0-67.0], p=0.011) in patients transplanted by 5 years. Patients with DB ≤1.0 mg/dL at 3 months after KPE were statistically younger at the time of liver biopsy (DB≤1.0 44.0 days [26.0-56.0] vs. DB>1.0 56.0 days [51.0-69.0], p=0.016). Ductal plate malformation was more frequent in patients who were transplanted at 5 years (transplanted patients 16/17, 94.1%, vs. transplant-free survival patients 1/17, 5.9%, p=0.037). Severe portal fibrosis occurred more frequently in patients transplanted at 2 years (19/23, 82.6%, vs. 4/23, 17.4%, p=0.028) compared to those who were not transplanted, as did those with severe acute cholangitis (6/7, 85.7%, vs. 1/7, 14.3%, p=0.047).

Conclusion: Older age at biopsy, presence of acute cholangitis, portal fibrosis, and ductal plate malformation were associated with lower native liver survival. Further evaluation in a larger study population is needed to validate these results.