title: Perioperative management and outcomes of esophageal atresia and tracheoesophageal fistula.
abstract: OBJECTIVE
  Esophageal atresia/tracheoesophageal fistula (EA/TEF) is a rare congenital anomaly lacking contemporary data detailing patient demographics, medical/surgical management and outcomes. Substantial variation in the care of infants with EA/TEF may affect both short- and long-term outcomes. The purpose of this study was to characterize the demographics, management strategies and outcomes in a contemporary multi-institutional cohort of infants diagnosed with EA/TEF to identify potential areas for standardization of care.
  
  
  METHODS
  A multi-institutional retrospective cohort study of infants with EA/TEF treated at 11 children's hospitals between 2009 and 2014 was performed. Over the 5year period, 396 cases were identified in the 11 centers (7±5 per center per year). All infants with a diagnosis of EA/TEF made within 30days of life who had surgical repair of their defect defined as esophageal reconstruction with or without ligation of TEF within the first six months of life were included. Demographic, operative, and outcome data were collected and analyzed to detect associations between variables.
  
  
  RESULTS
  Prenatal suspicion or diagnosis of EA/TEF was present in 53 (13%). The most common anatomy was proximal EA with distal TEF (n=335; 85%) followed by pure EA (n=27; 7%). Clinically significant congenital heart disease (CHD) was present in 137 (35%). Mortality was 7.5% and significantly associated with CHD (p<0.0001). Postoperative morbidity occurred in 62% of the population, including 165 (42%) cases with anastomotic stricture requiring intervention, anastomotic leak in 89 (23%), vocal cord paresis/paralysis in 26 (7%), recurrent fistula in 19 (5%), and anastomotic dehiscence in 9 (2%). Substantial variation in practice across our institutions existed: bronchoscopy prior to repair was performed in 64% of cases (range: 0%-100%); proximal pouch contrast study in 21% (0%-69%); use of interposing material between the esophageal and tracheal suture lines in 38% (0%-69%); perioperative antibiotics ≥24h in 69% (36%-97%); and transanastomotic tubes in 73% (21%-100%).
  
  
  CONCLUSIONS
  Contemporary treatment of EA/TEF is characterized by substantial variation in perioperative management and considerable postoperative morbidity and mortality. Future studies are planned to establish best practices and clinical care guidelines for infants with EA/TEF.
  
  
  METHODS
  Type of study: Treatment study. Level IV.
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI, United States. Electronic address: dlal@chw.org.
authors.forename: Dave R
authors.initials: DR
authors.lastname: Lal
authors.affiliation: Section of Pediatric Surgery, Department of Surgery, University of Michigan, Ann Arbor, MI, United States.
authors.forename: Samir K
authors.initials: SK
authors.lastname: Gadepalli
authors.affiliation: Division of Pediatric Surgery, Hiram C. Polk, Jr., M.D. Department of Surgery, University of Louisville, Louisville, KY, United States.
authors.forename: Cynthia D
authors.initials: CD
authors.lastname: Downard
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, University of Wisconsin, Madison, WI, United States.
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Ostlie
authors.affiliation: Center for Surgical Outcomes Research, The Research Institute at Nationwide Children's Hospital, Department of Surgery, University of Ohio, Columbus, OH, United States.
authors.forename: Peter C
authors.initials: PC
authors.lastname: Minneci
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI, United States.
authors.forename: Ruth M
authors.initials: RM
authors.lastname: Swedler
authors.affiliation: Division of Epidemiology, Institute for Health and Society, Medical College of Wisconsin, Milwaukee, WI, United States.
authors.forename: Thomas
authors.initials: T
authors.lastname: Chelius
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI, United States; Division of Epidemiology, Institute for Health and Society, Medical College of Wisconsin, Milwaukee, WI, United States.
authors.forename: Laura
authors.initials: L
authors.lastname: Cassidy
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI, United States.
authors.forename: Cooper T
authors.initials: CT
authors.lastname: Rapp
authors.affiliation: Center for Surgical Outcomes Research, The Research Institute at Nationwide Children's Hospital, Department of Surgery, University of Ohio, Columbus, OH, United States.
authors.forename: Katherine J
authors.initials: KJ
authors.lastname: Deans
authors.affiliation: Division of Pediatric Surgery, Hiram C. Polk, Jr., M.D. Department of Surgery, University of Louisville, Louisville, KY, United States.
authors.forename: Mary E
authors.initials: ME
authors.lastname: Fallat
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Indiana University School of Medicine, Indianapolis, IN, United States.
authors.forename: S Maria E
authors.initials: SME
authors.lastname: Finnell
authors.affiliation: Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.
authors.forename: Michael A
authors.initials: MA
authors.lastname: Helmrath
authors.affiliation: Section of Pediatric Surgery, Department of Surgery, University of Michigan, Ann Arbor, MI, United States.
authors.forename: Ronald B
authors.initials: RB
authors.lastname: Hirschl
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Feinberg School of Medicine of Northwestern University, Chicago, IL, United States.
authors.forename: Rashmi S
authors.initials: RS
authors.lastname: Kabre
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, University of Wisconsin, Madison, WI, United States.
authors.forename: Charles M
authors.initials: CM
authors.lastname: Leys
authors.affiliation: Section of Pediatric Surgery, Department of Surgery, The University of Chicago Medicine and Biologic Sciences, Chicago, IL, United States.
authors.forename: Grace
authors.initials: G
authors.lastname: Mak
authors.affiliation: Division of Pediatric Surgery, Hiram C. Polk, Jr., M.D. Department of Surgery, University of Louisville, Louisville, KY, United States.
authors.forename: Jessica
authors.initials: J
authors.lastname: Raque
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Indiana University School of Medicine, Indianapolis, IN, United States.
authors.forename: Frederick J
authors.initials: FJ
authors.lastname: Rescorla
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Washington University School of Medicine, St. Louis, MO, United States.
authors.forename: Jacqueline M
authors.initials: JM
authors.lastname: Saito
authors.affiliation: Department of Surgery, Children's Mercy Hospital, Kansas City, MO, United States.
authors.forename: Shawn D
authors.initials: SD
authors.lastname: St Peter
authors.affiliation: Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.
authors.forename: Daniel
authors.initials: D
authors.lastname: von Allmen
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Washington University School of Medicine, St. Louis, MO, United States.
authors.forename: Brad W
authors.initials: BW
authors.lastname: Warner
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI, United States.
authors.forename: Thomas T
authors.initials: TT
authors.lastname: Sato
mesh_terms: D000015:Abnormalities, Multiple / Q000601:surgery; D057868:Anastomotic Leak; D001999:Bronchoscopy; D004933:Esophageal Atresia / Q000601:surgery*; D004944:Esophagoplasty; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D019990:Perioperative Care; D011183:Postoperative Complications; D012189:Retrospective Studies; D014132:Trachea / Q000601:surgery; D014138:Tracheoesophageal Fistula / Q000601:surgery*; D016896:Treatment Outcome
keywords: Anastomotic leak; Anastomotic stricture; Esophageal atresia; Tracheoesophageal stricture; Variability in care