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

Abstract

Objectives Kasai portoenterostomy(KPE) is the treatment of choice for the fatal devastating infantile type III biliary atresia (BA). The study aimed to analyze short-and long-term outcomes after this procedure and their predictors in a tertiary center. Methods: We retrospectively analyzed 410 infants who underwent KPE for type III BA in the period from February 2000 to December 2019. The overall male/female ratio was 186/224. Results: The early (>6months) complications involved 187(45.6%) of our infants with a higher incidence of early cholangitis that affected 108(26.3%) of them. The jaundice clearance at the 6th post-operative month that reached 138(33.7%) of them had an independent correlation with mild portal tracts ductal and/or ductular proliferation, using postoperative steroids therapy, and absence of early postoperative cholangitis. The early infant mortality that affected 70(17.1%) of our patients was mostly from sepsis. On the other hand, late (<6months) patients complications and mortalities affected 256(62.4%) and 240(58.5%) of patients respectively; moreover, liver failure and sepsis were the most frequent causes of late mortalities in non-transplanted and transplanted cases respectively. Lastly, the long-term (20-year) native liver survival (NLS) that reached 91(22.2%) of patients had an independent correlation with age at operation ≤ 90 days, higher preoperative mean serum alb, portal tract fibrosis grades F0 and F1, absence of intraoperative bleeding, absence of post-operative cholangitis, the occurrence of jaundice clearance at the 6th postoperative month and absence of post-operative portal hypertension (PHN). Conclusions: Sepsis had a direct effect on early and late patient mortalities after Kasai operation for type III BA; moreover, patient age at operation<90 days, higher fibrosis grades, the occurrence of postoperative cholangitis and PHN, and persistence of post-operative jaundice had negative insult on long-term postoperative outcome. So, it is crucial to modulate these factors for a better outcome.

Clinical Characteristics and Outcome of Infants with Biliary Atresia in Bahrain

“…In this study, the majority of BA patients were females (57.7%). Female predominance has also been reported in other studies from the Middle East, 15 - 17 East Asia, 18 , 19 and Western countries. 20 - 22 This female predominance might have a scientific explanation.…”

“…The five-year NLS rate for BA patients referred to our center was 53.8% which matches most of the other cohorts ’ survival rates ranging from 40%–60% [ Table 5 ]. 8 , 15 - 17 , 20 , 30 - 34 Post-transplant survival rate was 76.9% (10/13). Our OLS rate was similar to the cohorts in other Middle Eastern countries but lower in comparison to European or Asian nations.…”

Impact of the Kasai Procedure and the Length of Native Liver Survival Time on Outcomes of Liver Transplantation for Biliary Atresia

“…There are 2 types of patients with BA who eventually receive LT. The patients with BA type Ⅰ require primary LT as the only management choice without prior KP, whereas the patients with BA type Ⅱ have KP first followed by salvage LT. (7,8) There are 2 different views on whether KP affects the clinical outcomes after LT. One view is that KP has no significant effect on the overall patient survival after LT, (9,10) whereas the other view believes that KP has a significant adverse effect on long-term survival (11,12) because of the increased infectious and biliary complications after salvage LT. (3,10) Therefore, the effect of KP on the prognosis of LT in patients with BA need to be further studied, particularly when confounding factors could be controlled effectively besides KP.…”

Neonatal biliary atresia combined with preduodenal portal vein: A case report

“…Theoretically, the application of drugs, such as steroids, after hepatoenteric anastomosis should be very effective in reducing immune-mediated liver damage, improving bile drainage, and reducing the incidence of reflux cholangitis. Since Gad et al [ 20 ] reported that short-term shock therapy with glucocorticoids can increase bile flow, many treatment institutions have adopted short-term shock therapy for 1 to 2 wk after surgery. Dillon et al [ 21 ] proposed that compared with the non-hormonal group, oral high-dose steroids [prednisone 4 ms/(kg·d), initial] in combination with ursodeoxycholic acid and antibiotic treatment at 6-22 wk after surgery can effectively enhance the bile clearance rate of children and improve the survival rate of autologous liver within 5 years.…”