The extended Kasai portoenterostomy for biliary atresia: A preliminary report

Abstract

Aims:In 1955, Professor Morio Kasai first performed a hepatic portoenterostomy. Since then, the procedure has changed the lives of children with biliary atresia (BA). We report our initial experience in performing “extended” Kasai portoenterostomy (KPE), a modification of the original procedure.Materials and Methods:Since 2013, we have used the technique of “extended KPE” and prospectively recorded data on all children undergoing this operation. Data on demographics, clinical features, liver function tests, and perioperative cholangiogram findings were collected. Outcome of KPE was measured by Jaundice Disappearance Rate (JDR) and Native Liver Survival Rate (NLSR). We present our preliminary results from a 30-month period (February 2013 to May 2015).Results:Thirty-one children underwent KPE during this period (19 males) and only 1 child had biliary atresia splenic malformation (BASM). The mean age at KPE was 73 ± 24 days. Five (16.1%) children were more than 90 days old at the time of KPE. Fourteen children cleared jaundice (JDR 45.2%). Eleven (35.5%) children developed episodes of cholangitis, of whom 8 had early cholangitis (within 3 months of the operation). The proportion of children who survived with their own liver 6 months after KPE (NLSR) was 84.2%. Of those children older than 90 days, 2 cleared jaundice and have survived with their native livers for more than 16 months.Conclusion:In our preliminary report of 31 children, we conclude that the extended KPE leads to increased jaundice clearance and improved NLSR in children with BA.

Biliary Atresia: pathology, Etiology and Pathogenesis

“…In a Japanese study, the rate of early jaundice clearance was 57% and the 10-year survival rate with their native liver was 54% [8]. Various studies from India have reported 36.2-47.2% of early jaundice clearance [9][10][11]. Our group reported successful KPE in a 29.4% of children over 90 days of age and concluded that in the absence of synthetic liver failure, age should not be a disqualification for performing KPE [12].…”

Increasing use of intraoperative cholangiogram in Australia: is it evidence‐based?

“…Analysis of IOC services data in the context of cholecystectomy assumes IOC is only performed during cholecystectomy surgery. However, hepatoportoenterostomy in children would require an IOC but no cholecystectomy, 16 meaning that inclusion of services for children would artificially inflate IOC rates. Accordingly, service claim data for children aged 0–4 and 5–14 years were excluded.…”

Biliary atresia, changing trends in management: Outlook of a pediatric liver transplant surgeon

“…[ 6 7 ] Conversely, few infants presenting after 90 days of age can also benefit from KPE. [ 4 8 ] Hence, grade of “liver fibrosis” is the guiding principle for KPE rather than “age.”[ 4 ] Hence, its “age” versus “liver fibrosis” at presentation and the latter appears to be the critical prognostic indicator. However, in most centers across the world, majority of BA cases are subjected to KPE without the evaluation for grade of 'liver fibrosis'.…”

“…KPE is a palliative surgery; several modifications have improved “Jaundice Disappearance Rate” (JDR) and prolonged NLS;[ 8 ] however, both depend on the grade and “rate” of progressive liver fibrosis, pre- and post-KPE, respectively. The progressive liver damage can continue despite the “so-called” successful KPE, slowly in cases with adequate bile flow and rapidly in cases with no bile flow, leading to decompensated cirrhosis requiring early sequential LT.[ 1 ]…”