What Makes A “Successful” Kasai Portoenterostomy “Unsuccessful”?

Abstract

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.

Response to Letter RE: What Makes A “Successful” Kasai Portoenterostomy “Unsuccessful?”

“…Many thanks for the comments from BB Lal et al on our paper (1,2). You rightly emphasize the role of cholangitis as a key reason for the failure of the “successful” Kasai portoenterostomy (KPE); together with highlighting prolonged intravenous antibiotics as a possible therapeutic option but question our choice of 3 months post-KPE as a point to evaluate prognosis from.…”

Appropriate Treatment of Cholangitis Can Prevent Successful Kasai Portoenterostomy Progressing to Liver Transplantation

“…The recently published article by Matcovici et al (1) highlights the impact of early and recurrent cholangitis for a child to progress to liver transplantation (LT) despite a successful Kasai portoenterostomy (KPE). Ascending cholangitis post‐KPE is indeed the Achilles heel in the management of infants post‐KPE complicated further by varying protocols of prophylactic antibiotics and steroids.…”

“…Ascending cholangitis post-KPE is indeed the Achilles heel in the management of infants post-KPE complicated further by varying protocols of prophylactic antibiotics and steroids. Surprisingly, the authors only utilized the 3 months post-operative variables in logistic regression analysis which is possibly the reason for cholangitis to miss out on being an independent predictor for need of LT in the present study (1). The high incidence of early cholangitis (67% within 3 months; 90% within 1-year post-KPE) coupled with the fact that recurrent cholangitis was the most frequent indication for LT in their cohort, emphasizes the need for timely diagnosis and adequate treatment of cholangitis.…”

“…To the Editor, Many thanks for the comments from BB Lal et al on our paper (1,2). You rightly emphasize the role of cholangitis as a key reason for the failure of the "successful" Kasai portoenterostomy (KPE); together with highlighting prolonged intravenous antibiotics as a possible therapeutic option but question our choice of 3 months post-KPE as a point to evaluate prognosis from.…”