Histopathological findings for prediction of liver cirrhosis and survival in biliary atresia patients after Kasai procedure

Gunadi Gunadi,Dwiki Afandy,Fiko Ryantono,Vincentia Meta Widya Paramita,Naomi Yoshuantari,Leila Rakhma Budiarti,Akhmad Makhmudi,Dian Nirmala Sirait,Hanggoro Tri Rinonce,Aditya Rifqi Fauzi

doi:10.1186/s13000-020-00996-y

Abstract

BackgroundWithout early recognition and Kasai procedure, biliary atresia (BA) results in liver cirrhosis and leads to either transplantation or death at a young age. We aimed to characterize the liver histopathological findings for prediction of cirrhosis and survival in BA patients after Kasai surgery.MethodsWe retrospectively reviewed all histopathological results for BA patients who underwent liver biopsy during Kasai surgery from August 2012 to December 2018 in Dr. Sardjito Hospital, Yogyakarta, Indonesia.ResultsFifty infants with BA were ascertained in our study, of whom 27 were males and 23 were females. The median age of Kasai procedure was 102.5 days (interquartile range (IQR), 75.75–142.25 days). There were 33 (66%) and 17 (34%) BA patients with and without liver cirrhosis, respectively, while the overall survival was 52%. The patients with a severe bile duct proliferation, severe cholestasis, and severe portal inflammation have a higher risk by 27-, 22-, and 19.3-fold, respectively, to develop liver cirrhosis compared with patients with a moderate/mild bile duct proliferation, moderate/mild/without cholestasis, and moderate/mild portal inflammation, respectively (p = 3.6 × 10− 6, 5.6 × 10− 4, and 1.6 × 10− 3, respectively), while the giant cell transformation was not associate with the development of liver cirrhosis (p = 0.77). The bile duct proliferation was strongly correlated with cholestasis and portal inflammation (p = 7.3 × 10− 5 and 2 × 10− 4, respectively), and cholestasis was also significantly correlated with portal inflammation (p = 0.016). Interestingly, the age at Kasai procedure was strongly associated with the development of liver cirrhosis (p = 0.02), but not with the patients’ survival (p = 0.33), while the degree of fibrosis and cholestasis were significantly correlated with the patients’ survival, with HR of 3.9 (95% CI = 1.7–9.0; p = 0.017) and 3.1 (95% CI = 1.4–7.0; p = 0.016), respectively.ConclusionsHistopathological findings of bile duct proliferation, cholestasis, and portal inflammation can predict the liver cirrhosis development in patients with BA. Furthermore, degree of fibrosis and cholestasis affect the patients’ survival following the Kasai operation.

Highlights

Without early recognition and Kasai procedure, biliary atresia (BA) results in liver cirrhosis and leads to either transplantation or death at a young age
The liver biopsies were conducted at the Kasai procedure, with median age of 102.5 days (Table 1)
Bile duct proliferation, cholestasis, and portal inflammation were significantly associated with liver cirrhosis (p = 3.6 × 10− 6, 5.6 × 10− 4, and 1.6 × 10− 3, respectively), whereas giant cell transformation was not (p = 0.77) (Table 2)

Summary

Introduction

Without early recognition and Kasai procedure, biliary atresia (BA) results in liver cirrhosis and leads to either transplantation or death at a young age. We aimed to characterize the liver histopathological findings for prediction of cirrhosis and survival in BA patients after Kasai surgery. The role of histopathological findings on the prognosis of patients with BA following Kasai operation has been reported with conflicting results [6–8]. BA will lead to liver cirrhosis and results in either transplantation or death at a young age if without early diagnosis and Kasai surgery [1, 2]. It is necessary to predict the development of liver cirrhosis and survival in BA patients after Kasai surgery. We aimed to characterize the liver histopathological findings for prediction of cirrhosis and survival in patients with BA following Kasai operation

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