title: Management of congenital diaphragmatic hernia: A systematic review from the APSA outcomes and evidence based practice committee.
abstract: OBJECTIVE
  Variable management practices complicate the identification of optimal strategies for infants with congenital diaphragmatic hernia (CDH). This review critically appraises the available evidence to provide recommendations.
  
  
  METHODS
  Six questions regarding CDH management were generated. English language articles published between 1980 and 2014 were compiled after searching Medline, Cochrane, Embase and Web of Science. Given the paucity of literature on the subject, all studies irrespective of their rank in the levels of evidence hierarchy were included.
  
  
  RESULTS
  Gentle ventilation with permissive hypercapnia provides the best outcomes. Initial high frequency ventilation may be considered but its overall efficacy is unproven. Routine inhaled nitric oxide (iNO) or other medical adjuncts for acute, severe pulmonary hypertension demonstrate no benefit. Evidence does not support routine administration of pre- or postnatal glucocorticoids. Mode of extracorporeal membrane oxygenation (ECMO) has little bearing on outcomes. While the overall timing of repair does not impact outcomes, early repair on ECMO has benefits. Open repair leads to significantly fewer recurrences. Polytetrafluoroethylene (PTFE) is the most durable patch repair material.
  
  
  CONCLUSIONS
  Limited high-level evidence prevents the development of robust management guidelines for CDH. Prospective, multi-institutional studies are needed to identify best practices and optimize outcomes.
authors.affiliation: Montreal Children's Hospital, McGill University Health Centre.
authors.forename: Pramod S
authors.initials: PS
authors.lastname: Puligandla
authors.affiliation: Ann and Robert Lurie Children's Hospital of Chicago.
authors.forename: Julia
authors.initials: J
authors.lastname: Grabowski
authors.affiliation: The University of Texas Medical School at Houston.
authors.forename: Mary
authors.initials: M
authors.lastname: Austin
authors.affiliation: Children's Hospital of Philadelphia.
authors.forename: Holly
authors.initials: H
authors.lastname: Hedrick
authors.affiliation: Albany Medical Center.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Renaud
authors.affiliation: University of Michigan, CS Mott Children's Hospital.
authors.forename: Meghan
authors.initials: M
authors.lastname: Arnold
authors.affiliation: University of Tennessee Health Science Center, Le Bonheur Children's Hospital.
authors.forename: Regan F
authors.initials: RF
authors.lastname: Williams
authors.affiliation: Phoenix Children's Hospital.
authors.forename: Kathleen
authors.initials: K
authors.lastname: Graziano
authors.affiliation: Cinncinati Children's Hospital.
authors.forename: Roshni
authors.initials: R
authors.lastname: Dasgupta
authors.affiliation: University of Kansas.
authors.forename: Milissa
authors.initials: M
authors.lastname: McKee
authors.affiliation: Baylor College of Medicine.
authors.forename: Monica E
authors.initials: ME
authors.lastname: Lopez
authors.affiliation: University of Tennessee Health Science Center, Le Bonheur Children's Hospital.
authors.forename: Tim
authors.initials: T
authors.lastname: Jancelewicz
authors.affiliation: Seattle Children's Hospital, University of Washington.
authors.forename: Adam
authors.initials: A
authors.lastname: Goldin
authors.affiliation: Kosair Children's Hospital, University of Louisville, Louisville, KY.
authors.forename: Cynthia D
authors.initials: CD
authors.lastname: Downard
authors.affiliation: University of Florida. Electronic address: saleem.islam@surgery.ufl.edu.
authors.forename: Saleem
authors.initials: S
authors.lastname: Islam
mesh_terms: D055317:Evidence-Based Practice; D015199:Extracorporeal Membrane Oxygenation; D005938:Glucocorticoids / Q000008:administration & dosage; D065630:Hernias, Diaphragmatic, Congenital / Q000628:therapy*; D006612:High-Frequency Ventilation; D006801:Humans; D006935:Hypercapnia / Q000209:etiology; D006976:Hypertension, Pulmonary / Q000188:drug therapy; D007231:Infant, Newborn; D009569:Nitric Oxide / Q000008:administration & dosage; D011446:Prospective Studies; D012121:Respiration, Artificial
chemical_list: D005938:Glucocorticoids; D009569:Nitric Oxide
keywords: CDH; ECMO; Pulmonary hypertension; Repair; Systematic review