Cholangitis Definition and Treatment after Kasai Hepatoportoenterostomy for Biliary Atresia: A Delphi Process and International Expert Panel.

Ana M Calinescu,Omid Madadi-Sanjani,Cara Mack,Riccardo Superina,Deirdre Kelly,Richard A Schreiber,Claus Petersen,Barbara E Wildhaber

doi:10.3390/jcm11030494

Abstract

(1) Background: Acute cholangitis during the first year after Kasai hepatoportoenterostomy (HPE) has a negative impact on patient and native liver survival. There are no consistent guidelines for the definition, treatment, and prophylaxis of cholangitis after HPE. The aim of this study was to develop definition, treatment, and prophylaxis guidelines to allow for expeditious management and for standardization in reporting. (2) Methods: the Delphi method, an extensive literature review, iterative rounds of surveys, and expert panel discussions were used to establish definition, treatment, and prophylaxis guidelines for cholangitis in the first year after HPE. (3) Results: Eight elements (pooled into two groups: clinical and laboratory/imaging) were identified to define cholangitis after HPE. The final proposed definitions for suspected and confirmed cholangitis are a combination of one element, respectively, two elements from each group; furthermore, the finding of a positive blood culture was added to the definition of confirmed cholangitis. The durations for prophylaxis and treatment of suspected and confirmed cholangitis were uniformly agreed upon by the experts. (4) Conclusions: for the first time, an international consensus was found for guidelines for definition, treatment, and prophylaxis for cholangitis during the first year after Kasai HPE. Applicability will need further prospective multicentered studies.

Highlights

Repeated cholangitis episodes are thought to be an important factor contributing to the progression of liver cirrhosis, leading to liver transplantation in biliary atresia patients and to decreased survival rates [3,4,5,6,7]
The clinical definition of cholangitis used in the literature varied largely (Appendix A, Table A1)
This study aimed to find, via the Delphi method and an expert panel, a consensus on the criteria that have diagnostic importance for cholangitis after Kasai HPE, defining suspected and confirmed cholangitis

Summary

Introduction

Acute cholangitis after Kasai hepatoportoenterostomy (HPE) is known to have a negative impact on prognosis; it predicts liver failure [1] and is associated with earlier liver transplantation [2]. Repeated cholangitis episodes are thought to be an important factor contributing to the progression of liver cirrhosis, leading to liver transplantation in biliary atresia patients and to decreased survival rates [3,4,5,6,7]. Reports on the incidence of cholangitis in biliary atresia patients vary between 40%. The Tokyo Guidelines, developed for adult patients, are clearly not applicable to diagnose cholangitis in children during the first year after Kasai HPE [16,17]

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Discussion

Conclusion