title: High incidence of fracture events in patients with Long-Gap Esophageal Atresia (LGEA): A retrospective review prompting implementation of standardized protocol.
abstract: OBJECTIVE
  To identify factors associated with an increased risk of fractures in Long-Gap Esophageal Atresia (LGEA) patients. Following implementation of a risk-stratified program, we hypothesized a reduction in fracture incidence within this potentially high-risk population.
  
  
  METHODS
  A retrospective review of LGEA-patients admitted between 2005 and 2014 was conducted. Symptomatic fractures with radiographic confirmation were defined as events. Univariate and multivariable analysis evaluated factors including admission weight-for-age z-score, primary versus secondary Foker process (FP), weight at Foker Stage I, days and episodes of paralysis, number of parenteral nutrition (PN) days, cumulative dose of loop diuretics adjusted for body weight and days exposed, and exposure to non-loop diuretics. A fracture-prevention protocol was initiated in 2012; incidence was evaluated pre and post-intervention.
  
  
  RESULTS
  Fifty-nine patients met inclusion criteria. Twenty-three (39%) patients in the entire cohort incurred at least one fracture during their hospitalization utilizing the Foker process. Given this high percentage, a targeted fracture-prevention protocol was initiated in 2012. Fracture incidence decreased from 48% prior to the protocol to 21% following the protocol (P= 0.046). Several variables that were associated with an increased risk of fractures on univariate analysis included prior esophageal anastomosis attempt (P= 0.008), number of separate episodes of paralysis (P= 0.002), exposure to non-loop diuretics (P= 0.006), cumulative loop diuretic dose (P< 0.001), as well as cumulative loop diuretic over days exposed (P< 0.001). Intensive care unit (ICU) stay (P= 0.002) and total length of hospitalization (P< 0.001) were also significantly longer among patients with a fracture. Number of separate episodes of paralysis was the only independent risk factor for the development of a fracture; patients having more than 3 episodes of paralysis had an estimated risk of fracture 15 times higher than those patients paralyzed only once or twice (O.R. 15.87, 95% C.I.: 1.47-171.23,P= 0.008).
  
  
  CONCLUSIONS
  Episodes of paralysis appeared to be the most significant risk factor for fractures in patients with LGEA who underwent the Foker procedure. The incidence of symptomatic fractures decreased significantly following implementation of a standardized protocol in this series of LGEA patients with continued prospective evaluation.
authors.affiliation: Department of Pediatric Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States.
authors.forename: Sigrid
authors.initials: S
authors.lastname: Bairdain
authors.affiliation: Department of Pharmacy, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States.
authors.forename: Brenda
authors.initials: B
authors.lastname: Dodson
authors.affiliation: Department of Pediatric Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States; Division of Critical Care Medicine, Department of Anesthesia, Perioperative and Pain Medicine, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States.
authors.forename: David
authors.initials: D
authors.lastname: Zurakowski
authors.affiliation: Department of Pulmonology, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States.
authors.forename: Lawrence
authors.initials: L
authors.lastname: Rhein
authors.affiliation: Department of Orthopedics, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States.
authors.forename: Brian D
authors.initials: BD
authors.lastname: Snyder
authors.affiliation: Division of Endocrinology, Department of Medicine, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States.
authors.forename: Melissa
authors.initials: M
authors.lastname: Putman
authors.affiliation: Department of Pediatric Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States.
authors.forename: Russell W
authors.initials: RW
authors.lastname: Jennings