Biliary Atresia - A 25-Year Survey

Abstract

In the past 25 years, from 1963 to 1988, 90 children from the Department of Pediatric Surgery of the Pediatric Hospital in Cologne, Germany were treated for biliary atresia. Of these, 47 had purely extrahepatic bile duct lesions, 21 had purely intrahepatic bile duct lesions, and 22 had both extrahepatic and intrahepatic bile duct lesions. Forty-five of the children underwent a drainage operation, whereas the remaining 45 children underwent no surgery at all or simply a diagnostic laparotomy. Until 1966 hepato-jejunostomy with implantation of artificial bile ducts was conducted in 12 cases. Later, cholecystoduodenostomy was performed 4 times and hepatoporto-jejunostomy according to Kasai-Kimura 29 times. The latter was performed either without and enterostomy (n = 16) or with an enterostomy in the respective intestinal loop (n = 13). 27 patients survived (30%). If only the children with intrahepatic bile duct hypoplasia are considered, the survival rate was 12 out of 15 patients (80%). Eight children (27.5%) of the 29 with hepatoporto-jejunostomy are still alive today. This survival rate, compared with the survival rate of the total, is comparably large with 27.7%. Current data from the 23 surviving patients was retrospectively gathered in our hospital or was collected from outside the establishment. It was analyzed with regard to prognosis and long-term results. Only in 5 of the 29 cases of children with hepatoporto-jejunostomy could a lasting postoperative biliary flow be achieved. Only one of the children can be classified as completely healthy in regard to his liver.(ABSTRACT TRUNCATED AT 250 WORDS)

Biliary Atresia Revisited

“…Advanced age at time of HPE (more than 2 months of age), white race, cirrhosis at initial biopsy, experience of the surgical center, complete nonpatency of the extrahepatic biliary tree, obliterated bile ducts at the porta hepatis, multicystic dilatation, recurrent cholangitis, portal hypertension, and associated congenital anomalies have been accepted by the majority of the investigators as poor prognostic signs [5, 8, 21, 90, 100 -105]. Recent studies, based on a large series of children with EHBA, have challenged some of these statements [23,87]. Thus, no correlation was found between timing of surgery and outcome, or with size of ducts at the porta hepatis [23].…”

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

“…Despite these SRs, data from published literature originate from single-centers with reduced sample size [ 18 19 ] or based on long-term results from multiple institutions, before pediatric liver transplantation became regularly available [ 20 21 22 ]. Further, no SR to date has examined histopathological parameters, such as degree of liver fibrosis, in relation to NLSR in children who undergo Kasai for BA.…”