Multivariate Analysis of Biliary Flow-Related Factors and Post-Kasai Survival in Biliary Atresia Patients

Abstract

BACKGROUND: Biliary atresia represents the most common surgically treatable cause of cholestasis in newborns. If not corrected, secondary biliary cirrhosis invariably results. OBJECTIVE: To evaluate, through multivariate analysis, the prognostic factors associated with the presence of biliary flow and survival with the native liver following Kasai portoenterostomy. METHODS: The study analyzed data from 117 biliary atresia patients who underwent portoenterostomy and had suitable histological material for evaluation. A logistic regression model was used to assess the presence of biliary flow. Survival was investigated through Kaplan-Meier curves and Cox-adjusted models. RESULTS: One third of patients achieved biliary flow and the median age at surgery was 81 days. Age at surgery, albumin, postoperative complications, biliary atresia structural malformation (BASM), liver architecture, larger duct diameter at porta hepatis, and cirrhosis (Ishak score) were the initial variables for the multivariate analysis. Age at surgery >90 days was the only variable associated with the absence of biliary drainage. Survival analysis revealed that the absence of biliary flow (P<0.0001), age at surgery >90 days (P=0.035), and the presence of BASM (P<0.0001), alone, could predict death or need for liver transplantation. Multivariate analysis demonstrated that the absence of biliary flow (P<0.0001 hazard ratio [HR] 6.25, 95% confidence interval [CI] 3.19-12.22) and the presence of BASM (P=0.014 HR 2.16, 95% CI 1.17-3.99) were associated with lowest survival with the native liver. CONCLUSION: Age at surgery >90 days was associated with absence of biliary flow. The presence of biliary drainage and the absence of structural malformations are cornerstone features for higher survival rates with the native liver.

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.…”

“…All studies consulted secure records and/or liver histology for ascertainment of histopathological parameters and BA, and participants were truly or somewhat representative of the average pediatric patient with BA. Apart from 11 studies that were based on a selected group of users limited to a small sample size, patients were identified via electronic health records or referred to as a consecutive sample later subdivided into ideal vs. non-ideal outcomes [ 28 29 32 35 36 37 39 40 51 54 57 ]. Fourteen studies described pathologists being independently blinded to NLSR status [ 29 31 32 33 34 35 36 39 40 48 49 51 54 57 ].…”

“…Consequently, we extracted the actuarial survival rate at the designated follow-up period (2-, 5-, 10- or 20-years) [ 26 ]. For studies that did not explicitly state NLSR, we extracted data based on how the authors defined “favourable outcome” or “success of operation.” Hence, we extracted the frequency of patients with normal living function without cholangitis or portal hypertension [ 27 ], patients with good quality of life without jaundice relapsing [ 28 ], normal lab parameters and no evidence of medical chronic liver disease [ 29 ], absence of cirrhosis [ 30 31 ] or serum total bilirubin <2 mg/dL at follow-up post-Kasai operation [ 32 33 34 35 36 ].…”

“…For example, 0 -no fibrosis; 1 - mild portal fibrosis with no septa; 2 - porto-septal (rare fibrous septa) and non-bridging fibrosis; 3 - bridging fibrosis with many fibrous septa; and 4 - cirrhosis [ 26 37 ]. Liver fibrosis could be assessed via the Ohkuma’s classification from grades I to IV [ 38 ], Metavir system where F1–F3 implied non-severe fibrosis, and F4 as severe fibrosis (cirrhosis), or the Ishak score where non-severe indicated F1 to F5 while severe was F6 [ 32 ].…”

The usefulness of immunohistochemical staining of bile tracts in biliary atresia

“…Some authors did not find any histological features that predicted successful bile drainage after Kasai portoenterostomy, apart from severity of portal fibrosis. DPM and bile duct injury were associated with shorter survival with the native liver [17,18]. Most cases of BA (80%) are considered perinatal or acquired, while a minority (20%) are congenital and are expected to lead to DPM [6].…”