Current management of biliary atresia based on 35 years of experience at a single center

Abstract

OBJECTIVE:The prognosis of patients with biliary atresia undergoing Kasai portoenterostomy is related to the timing of the diagnosis and the indication for the procedure. The purpose of the present study is to present a practical flowchart based on 257 children who underwent Kasai portoenterostomy.METHODS:We conducted a retrospective cohort study of patients who underwent Kasai portoenterostomy between 1981 and 2016.RESULTS:During the first period (1981 to 2009), 230 infants were treated, and the median age at the time of surgery was 84 days; jaundice was resolved in 77 patients (33.5%). During the second period, from 2010 to 2016, a new diagnostic approach was adopted to shorten the wait time for portoenterostomy; an ultrasonography examination suggestive of the disease was followed by primary surgical exploration of the biliary tract without complementary examination or liver biopsy. Once the diagnosis of biliary atresia was confirmed, a portoenterostomy was performed during the same surgery. During this period, 27 infants underwent operations; the median age at the time of surgery was 66 days (p<0.001), and jaundice was resolved in 15 patients (55.6% - p=0.021), with a survival rate of the native liver of 66.7%.CONCLUSION:Primary surgical exploration of the biliary tract without previous biopsy was effective at improving the prognostic indicators of patients with biliary atresia undergoing Kasai portoenterostomy.

Short- and long-term outcomes after Kasai operation for type III biliary atresia: Twenty years of experience in a single tertiary Egyptian center-A retrospective cohort study

“…Biliary atresia (BA) is a neonatal progressive destructive fibro-obliterative cholangiopathy of extra- and intra-hepatic biliary system with fatal outcome in the 1st 2 years of life if left untreated due to the resulting liver cirrhosis, portal hypertension (PHN), end-stage liver disease (ESLD) and liver failure [ [1] , [2] , [3] , [4] , [5] ]. Despite being a disease of unknown origin; viral infection, ductal plate malformations, genetic predisposition, and abnormal bile acid metabolism are possible causes [ 6 ]; while, immunologic, inflammatory, infectious, and obstructive pathways are possible theories of pathogenesis [ 7 ].…”

“…In brief; a small incision in the right upper abdomen was done, then after its entry; the abdomen was grossly inspected to identify any associated anomalies. In many cases, the diagnosis of BA was confirmed by visual inspection of the liver that appeared cholestatic or fibrotic with absent, fibrotic, or atrophic gallbladder; figure [ 3 ]. However, if a normal gallbladder or hilar cyst appeared, cholangiography was performed through them to confirm the diagnosis; figure [ 1 ].…”