Biological and Histological Assessment of the Hepatoportoenterostomy Role in Biliary Atresia as a Stand-Alone Procedure or as a Bridge toward Liver Transplantation

Abstract

Background and objectives: In patients with biliary atresia (BA), hepatoportoenterostomy (HPE) is still a valuable therapeutic tool for prolonged survival or a safer transition to liver transplantation. The main focus today is towards efficient screening programs, a faster diagnostic, and prompt treatment. However, the limited information on BA pathophysiology makes valuable any experience in disease management. This study aimed to analyze the evolution and survival of patients with BA referred for HPE (Kasai operation) in our department. Materials and Methods: A retrospective analysis was performed on fourteen patients with BA, diagnosed in the pediatric department and further referred for HPE in our surgical department between 2010 and 2016. After HPE, the need for transplantation was assessed according to patients cytomegalovirus (CMV) status, and histological and biochemical analysis. Follow-up results at 1–4 years and long term survival were assessed. Results: Mean age at surgery was 70 days. Surgery in patients younger than 60 days was correlated with survival. Jaundice’s clearance rate at three months was 36%. Total and direct bilirubin values had a significant variation between patients with liver transplants and native liver (p = 0.02). CMV was positive in eight patients, half with transplant need and half with native liver survival. Smooth muscle actin (SMA) positivity was proof of advanced fibrosis. The overall survival rate was 79%, with 75% for native liver patients and an 83% survival rate for those with liver transplantation. Transplantation was performed in six patients (43%), with a mean of 10 months between HPE and transplantation. Transplanted patients had better survival. Complications were diagnosed in 63% of patients. The mean follow-up period was six years. Conclusions: HPE, even performed in advanced cirrhosis, allows a significant survival, and ensures an essential time gain for patients requiring liver transplantation. A younger age at surgery is correlated with a better outcome, despite early CMV infection.

Peri-Operative Liver Fibrosis and Native Liver Survival in Pediatric Patients with Biliary Atresia: A Systematic Review and Meta-Analysis

“…Characteristics of the 28 studies with 1,171 pediatric patients who had BA and underwent Kasai operation are located in Table 1 . Briefly, there were 25 cohort [ 26 27 28 29 30 31 32 33 34 36 37 39 40 41 45 46 47 48 49 50 51 52 53 54 55 ] and 3 case control studies [ 35 56 57 ]. Six hundred and thirty-one patients survived with their native liver, while 573 required Ltx and 99 reportedly died after Kasai during follow-up.…”

“…Fourteen studies described pathologists being independently blinded to NLSR status [ 29 31 32 33 34 35 36 39 40 48 49 51 54 57 ]. Length of follow-up was 12 months for capturing survival outcomes in 17 studies [ 26 27 28 29 31 34 35 36 37 46 47 48 50 51 54 56 57 ]. The studies were mainly scored as ‘poor’ because they did not describe adjusting for confounders in a regression model, such as age at Kasai operation, anatomical pattern, polysplenia syndrome, level of centre experience, sex, albumin, total or direct bilirubin, alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase, gamma-glutamyl transferase, and/or degree of liver failure.…”