Features of Nodules in Explants of Children Undergoing Liver Transplantation for Biliary Atresia.

Ana M Calinescu,Barbara E Wildhaber,Anne-Laure Rougemont,Nathalie M Rock,Mehrak Anooshiravani,Valerie A Mclin

doi:10.3390/jcm11061578

Abstract

(1) Background: In patients with biliary atresia (BA) liver nodules can be identified either by pre-transplant imaging or on the explant. This study aimed to (i) analyze the histopathology of liver nodules, and (ii) to correlate histopathology with pretransplant radiological features. (2) Methods: Retrospective analysis of liver nodules in explants of BA patients transplanted in our center (2000–2021). Correlations with pretransplant radiological characteristics, patient age at liver transplantation (LT), time from Kasai hepatoportoenterostomy (KPE) to LT, age at KPE and draining KPE. (3) Results: Of the 63 BA-patients included in the analysis, 27/63 (43%) had nodules on explants. A majority were benign macroregenerative nodules. Premalignant (low-grade and high-grade dysplastic) and malignant (hepatocellular carcinoma) nodules were identified in 6/63 and 2/63 patients, respectively. On pretransplant imaging, only 13/63 (21%) patients had liver nodules, none meeting radiological criteria for malignancy. The occurrence of liver nodules correlated with patient age at LT (p < 0.001), time KPE-LT (p < 0.001) and draining KPE (p = 0.006). (4) Conclusion: In BA patients, pretransplant imaging did not correlate with the presence of liver nodules in explants. Liver nodules were frequent in explanted livers, whereby 25% of explants harboured malignant/pre-malignant nodules, emphasizing the need for careful surveillance in BA children whose clinical course may require LT.

Highlights

Biliary atresia (BA) is the main indication for liver transplantation (LT) in children [1,2].Every BA patient, after Kasai hepatoportoenterostomy (KPE) or in the absence of it, will eventually develop some degree of liver fibrosis or cirrhosis
The following data were collected from the national BA database: demographics, age at KPE, cholangitis episodes fever associated with discolored stool and/or jaundice, or ii) fever associated with inflammatory parameters and/or cholestasis and/or increased transaminases and/or positive blood cultures), draining KPE, pre-LT laboratory values (aspartate aminotransferase (AST), alanine aminotransferase (ALT), γ-glutamyl transferase (GGT), total and conjugated bilirubin, alpha-fetoprotein (AFP)), presence or not of pre-LT
Portal hypertension (defined as both splenomegaly + 2SD and thrombocytopenia or history of a complication of portal hypertension such as varices, ascites, etc.), age at LT, imaging (US performed at 6 weeks, 6 months, 1 year, 2 years pre-LT and/or computed tomography (CT) performed prior to LT, see Section 2.2) and pathology results

Summary

Introduction

Biliary atresia (BA) is the main indication for liver transplantation (LT) in children [1,2]. Every BA patient, after Kasai hepatoportoenterostomy (KPE) or in the absence of it, will eventually develop some degree of liver fibrosis or cirrhosis. As biliary cirrhosis is associated with malignant transformation, children with BA warrant careful monitoring [3]. The usual modalities for the follow up of BA patients are ultrasound (US) and/or computed tomography (CT) and/or magnetic resonance imaging (MRI). If liver nodules identified on imaging exhibit malignant characteristics, biopsy is warranted [4]. The detection of malignant or premalignant nodules is clinically important, as it accelerates the need for LT [5]. It is known that explants of patients undergoing LT for BA can harbor various benign

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Discussion

Conclusion