Management of congenital diaphragmatic hernia: A systematic review from the APSA outcomes and evidence based practice committee

Pramod S. Puligandla, Julia Grabowski, Mary Austin, Holly Hedrick, Elizabeth Renaud, Meghan Arnold, Regan F. Williams, Kathleen Graziano, Roshni Dasgupta, Milissa McKee, Monica E. Lopez, Tim Jancelewicz, Adam Goldin, Cynthia D. Downard, Saleem Islam

Research output: Contribution to journalReview articlepeer-review

122 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)1958-1970
Number of pages13
JournalJournal of Pediatric Surgery
Issue number11
Publication statusPublished - 1 Nov 2015
Externally publishedYes


  • CDH
  • ECMO
  • Pulmonary hypertension
  • Repair
  • Systematic review


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