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

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.

Keywords: Biliary atresia; Long-term outcome; Short-term outcome; Survival with the native liver.

Fig. 1

a- Preparation for IOC, b- IOC shows non cystic type III BA, c- IOC shows cystic type III BA.

Fig. 2

Non cystic type III BA.

Fig. 3

Atrophic gallbladder.

Fig. 4

Portal plate after dissection and transaction of biliary remnant and before reconstruction.

Fig. 5

a- KPE (Posterior layer), b- KPE (Anterior layer).

Fig. 6

A-KM long-term NLS curve B-KM long-term overall survival curve.

Fig. 7

KM analysis long-term NLS curves: A: Age at operation and long-term NLS (Log rank = 0.000) B- Liver fibrosis and long-term NLS (Log rank = 0.000) C- Jaundice clearance at the 6th post-operative month and long-term NLS (Log rank = 0.000) D- Post-operative cholangitis and long-term NLS (Log rank = 0.000) E− Post-operative PHN and long-term NLS (Log rank = 0.006).