Pramod S Puligandla1, Julia Grabowski2, Mary Austin3, Holly Hedrick4, Elizabeth Renaud5, Meghan Arnold6, Regan F Williams7, Kathleen Graziano8, Roshni Dasgupta9, Milissa McKee10, Monica E Lopez11, Tim Jancelewicz7, Adam Goldin12, Cynthia D Downard13, Saleem Islam14. 1. Montreal Children's Hospital, McGill University Health Centre. 2. Ann and Robert Lurie Children's Hospital of Chicago. 3. The University of Texas Medical School at Houston. 4. Children's Hospital of Philadelphia. 5. Albany Medical Center. 6. University of Michigan, CS Mott Children's Hospital. 7. University of Tennessee Health Science Center, Le Bonheur Children's Hospital. 8. Phoenix Children's Hospital. 9. Cinncinati Children's Hospital. 10. University of Kansas. 11. Baylor College of Medicine. 12. Seattle Children's Hospital, University of Washington. 13. Kosair Children's Hospital, University of Louisville, Louisville, KY. 14. University of Florida. Electronic address: saleem.islam@surgery.ufl.edu.
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.
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.
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