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Published on: March 12, 2019
What Makes A "Successful" Kasai Portoenterostomy "Unsuccessful"?
Melania Matcovici1, Ionica Stoica, Kristy Smith
1From the Department of Paediatric Surgery, Kings College Hospital, London, United Kingdom.
Insights
Clearance of jaundice after Kasai portoenterostomy for biliary atresia is crucial for prognosis. Failure leading to liver transplant is often caused by recurrent cholangitis and portal hypertension, not initial surgical outcomes.
Area of Science:
- Pediatric Surgery
- Hepatology
- Gastroenterology
Background:
- Clearance of jaundice (CoJ) following Kasai portoenterostomy (KPE) is the primary goal for biliary atresia (BA).
- Achieving CoJ is the strongest predictor of long-term prognosis in BA patients.
- Identifying factors leading to subsequent liver transplant (LT) is essential for improving patient outcomes.
Purpose of the Study:
- To investigate the reasons for failure (requiring LT) in infants who underwent KPE for BA.
- To identify predictive factors for LT at 3 months post-KPE.
Main Methods:
- A prospective database of BA patients undergoing KPE was reviewed.
- Successful KPE was defined as postoperative bilirubin ≤20 µmol/L.
- Multivariate logistic regression identified predictors of LT by 2 years post-KPE.
Main Results:
- Of 135 infants, 90 (67%) achieved CoJ; 20 (22%) of these later required LT.
- Recurrent cholangitis and portal hypertension (gastrointestinal bleeding) were significantly more frequent in the LT group.
- High APRi, bilirubin, INR, and ascites at 3 months post-KPE predicted LT with high accuracy (AUROC = 0.98).
Conclusions:
- Failure of KPE leading to LT is not determined at the time of surgery.
- Recurrent cholangitis and symptomatic portal hypertension are the main drivers of LT in BA patients post-KPE.
Objectives:
Clearance of jaundice (CoJ) is the first key objective of Kasai portoenterostomy (KPE) for biliary atresia (BA) and its achievement is by far the best index of long-term prognosis. We sought to identify the reasons for failure [subsequent liver transplant (LT)] in this cohort.
Methods:
Review of single-center prospective BA database. Successful KPE was defined by achieving a postoperative bilirubin of ≤20 µmol/L. Pre-KPE and post-KPE variables were identified together with a multivariate logistic regression model to identify those observable at 3 months post-KPE. Data are quoted as median (range). A P value of ≤0.05 was significant.
Results:
One hundred thirty-five infants underwent KPE between January 2012 and December 2018, of which 90 (67%) achieved CoJ. From these 20 (22%) (Cohort A) underwent LT with the remainder continuing with native liver (Cohort B) (median follow-up of 4.15 years). There was no difference in age at KPE ( P = 0.41), APRi (aspartate aminotransferase-to-platelet ratio) ( P = 0.07), associated anomalies ( P = 0.7), and cytomegalovirus status ( P = 0.7) between the 2 groups. Postoperatively, both cholangitis [any episode, 18/20 (90%) vs 15/70 (21%); P < 0.0001] and portal hypertension (PHT) [gastrointestinal (GI) bleed, 10/20 (50%) vs 2/70 (2.8%); P < 0.0001] were significantly more common in cohort A. Univariate analysis showed that the most significant predictive values at 3 months for LT by 2 years were high APRi, bilirubin, international normalized ratio, and ultrasound (US)-detected ascites with multivariate logistic modeling confirming these variables with predictive values of r2 = 0.79, AUROC = 0.98.
Conclusions:
Failure is not preordained at KPE but due to recurrent cholangitis and/or symptoms of PHT.

