title: Variability of diagnostic approach, surgical technique, and medical management for children with biliary atresia in Canada - Is it time for standardization?
abstract: BACKGROUND
  The Canadian 4-year native liver survival rate for biliary atresia (BA) after Kasai Portoenterostomy (KP) is 39%. The Canadian Biliary Atresia Registry (CBAR) was used to examine variability of surgical and medical management of BA.
  
  
  METHODS
  Gastroenterologists and surgeons in all 14 Canadian pediatric tertiary centers were invited to complete an online survey of their BA management practices.
  
  
  RESULTS
  Of gastroenterologists, diagnostic procedures included liver biopsy (92%), HIDA scan (58%), and percutaneous cholangiogram (46%). Surgeons reported Roux-en-Y lengths of 20-50cm with 78% avoiding diathermy at the portal plate; 16% performed laparoscopic exploration, but none laparoscopic KP. Postoperative corticosteroids and antibiotics were used by 24% and 85% of gastroenterologists, respectively, with similar rates for surgeons. At discharge, gastroenterologists prescribed oral antibiotics (80%), and ursodeoxycholic acid (95%), while surgeons reported lower rates (62% and 55%). Considerable variation existed in follow-up monitoring. No center had a standard protocol for evaluating suspected cholangitis. There was a lack of consensus for defining failed KP and referral criteria for transplant evaluation.
  
  
  CONCLUSIONS
  In Canada, treatment of BA is not centralized, and there is variability in diagnostic approaches and management. Collaboration through CBAR will allow for implementation and evaluation of standardized surgical and medical management with a goal to improve outcomes.
  
  
  METHODS
  Survey study. Level IV evidence.
authors.affiliation: Department of Pediatric Surgery, Montreal Children's Hospital, Montreal, QC, Canada H4A 3J1.
authors.forename: Hayden
authors.initials: H
authors.lastname: Stagg
authors.affiliation: Division of Pediatric Surgery, McMaster Children's Hospital, Hamilton, ON, Canada L8N3Z5.
authors.forename: Brian H
authors.initials: BH
authors.lastname: Cameron
authors.affiliation: Division of Pediatric Gastroenterology, Montreal Children's Hospital, Montreal, QC, Canada H4A 3J1.
authors.forename: Najma
authors.initials: N
authors.lastname: Ahmed
authors.affiliation: Department of Pediatrics, University of British Columbia, BC Children's Hospital, Vancouver, BC, Canada V6H 3V4.
authors.forename: Alison
authors.initials: A
authors.lastname: Butler
authors.affiliation: Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada K1H 8L1.
authors.forename: Carolina
authors.initials: C
authors.lastname: Jimenez-Rivera
authors.affiliation: Division of Pediatric Surgery, IWK Health Centre, Halifax, Nova Scotia, Canada B3K 6R8.
authors.forename: Natalie L
authors.initials: NL
authors.lastname: Yanchar
authors.affiliation: Division of Pediatric Gastroenterology, Alberta Children's Hospital, Calgary, AB, Canada T3B 6A8.
authors.forename: Steve R
authors.initials: SR
authors.lastname: Martin
authors.affiliation: Department of Pediatric Surgery, Montreal Children's Hospital, Montreal, QC, Canada H4A 3J1.
authors.forename: Sherif
authors.initials: S
authors.lastname: Emil
authors.affiliation: Canadian Biliary Atresia Registry Patient/Parent Representative.
authors.forename: George
authors.initials: G
authors.lastname: Anthopoulos
authors.affiliation: Division of Pediatric Gastroenterology, Department of Pediatrics, University of British Columbia, BC Children's Hospital, Vancouver, BC, Canada V6H 3V4.
authors.forename: Richard A
authors.initials: RA
authors.lastname: Schreiber
authors.affiliation: Department of Pediatric Surgery, Montreal Children's Hospital, Montreal, QC, Canada H4A 3J1. Electronic address: jean-martin.laberge@muhc.mcgill.ca.
authors.forename: Jean-Martin
authors.initials: JM
authors.lastname: Laberge
mesh_terms: D000359:Aftercare / Q000379:methods / Q000706:statistics & numerical data; D001656:Biliary Atresia / Q000175:diagnosis / Q000601:surgery; D002170:Canada; D002648:Child; D002675:Child, Preschool; D002758:Cholangiography / Q000706:statistics & numerical data; D003131:Combined Modality Therapy / Q000706:statistics & numerical data; D019538:Health Care Surveys; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D010535:Laparoscopy / Q000706:statistics & numerical data; D016031:Liver Transplantation / Q000706:statistics & numerical data; D011171:Portoenterostomy, Hepatic / Q000379:methods / Q000706:statistics & numerical data; D010818:Practice Patterns, Physicians' / Q000592:standards / Q000706:statistics & numerical data*; D016896:Treatment Outcome
keywords: Biliary atresia; Kasai portoenterostomy; Outcomes research; Registry; Variability