title: The anatomic pattern of biliary atresia identified at time of Kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival.
abstract: OBJECTIVE
  The goals of this study were to describe the clinical and anatomic features of infants undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these parameters and outcomes.
  
  
  METHODS
  Infants enrolled in the prospective Childhood Liver Disease Research and Education Network, who underwent KPE were studied. Patients enrolled in a blinded, interventional trial were excluded from survival analysis. Primary endpoints were successful surgical drainage (total bilirubin less than 2 mg/dL within the first 3 months), transplant-free survival (Kaplan-Meier), and time to transplant/death (Cox regression).
  
  
  RESULTS
  KPE was performed in 244 infants (54% female; mean age: 65 ± 29 days). Transplant-free survival was 53.7% and 46.7% at 1 and 2 years post-KPE. The risk of transplant/death was significantly lower in the 45.6% of patients who achieved successful bile drainage within 3 months post-KPE (HR: 0.08, P < 0.001). The risk of transplant/death was increased in patients with porta hepatis atresia (Ohi type II and III vs type I; HR: 2.03, P = 0.030), nonpatent common bile duct (Ohi subtype: b, c, and d vs a; HR: 4.31, P = 0.022), BA splenic malformation syndrome (HR: 1.92, P = 0.025), ascites > 20 mL (HR: = 1.90, P = 0.0230), nodular liver appearance compared to firm (HR: = 1.61, P = 0.008), and age at KPE ≥ 75 days (HR: 1.73, P < 0.002). Outcome was not associated with gestational age, gender, race, ethnicity, or extent of porta hepatis dissection.
  
  
  CONCLUSIONS
  Anatomic pattern of BA, BASM, presence of ascites and nodular liver appearance at KPE, and early postoperative jaundice clearance are significant predictors of transplant-free survival.
authors.affiliation: Children's Memorial Hospital, Chicago, IL, USA.
authors.forename: Riccardo
authors.initials: R
authors.lastname: Superina
authors.forename: John C
authors.initials: JC
authors.lastname: Magee
authors.forename: Mary L
authors.initials: ML
authors.lastname: Brandt
authors.forename: Patrick J
authors.initials: PJ
authors.lastname: Healey
authors.forename: Greg
authors.initials: G
authors.lastname: Tiao
authors.forename: Fred
authors.initials: F
authors.lastname: Ryckman
authors.forename: Frederick M
authors.initials: FM
authors.lastname: Karrer
authors.forename: Kishore
authors.initials: K
authors.lastname: Iyer
authors.forename: Annie
authors.initials: A
authors.lastname: Fecteau
authors.forename: Karen
authors.initials: K
authors.lastname: West
authors.forename: R Cartland
authors.initials: RC
authors.lastname: Burns
authors.forename: Alan
authors.initials: A
authors.lastname: Flake
authors.forename: Hanmin
authors.initials: H
authors.lastname: Lee
authors.forename: Jeff A
authors.initials: JA
authors.lastname: Lowell
authors.forename: Pat
authors.initials: P
authors.lastname: Dillon
authors.forename: Paul
authors.initials: P
authors.lastname: Colombani
authors.forename: Richard
authors.initials: R
authors.lastname: Ricketts
authors.forename: Yun
authors.initials: Y
authors.lastname: Li
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Moore
authors.forename: Kasper S
authors.initials: KS
authors.lastname: Wang
mesh_terms: D001656:Biliary Atresia / Q000150:complications / Q000473:pathology* / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D007565:Jaundice / Q000209:etiology; D008297:Male; D011171:Portoenterostomy, Hepatic; D011446:Prospective Studies