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Short-Term Neurodevelopmental Outcome in Congenital Diaphragmatic Hernia: The Impact of Extracorporeal Membrane Oxygenation and Timing of Repair

Danzer, Enrico MD; Hoffman, Casey PhD; D’Agostino, Jo Ann DNP, CRNP; Connelly, James T. BS, RRT-NPS; Waqar, Lindsay N. MPH; Gerdes, Marsha PhD; Bernbaum, Judy MD; Rintoul, Natalie E. MD; Herkert, Lisa M. CRNP; Peranteau, William H. MD; Flake, Alan W. MD; Adzick, N. Scott MD; Hedrick, Holly L. MD

Pediatric Critical Care Medicine: January 2018 - Volume 19 - Issue 1 - p 64-74
doi: 10.1097/PCC.0000000000001406
Extracorporeal Support

Objectives: The purpose of this study was to assess the need and timing of extracorporeal membrane oxygenation in relation to congenital diaphragmatic hernia repair as modifiers of short-term neurodevelopmental outcomes.

Design: Retrospective study.

Setting: A specialized tertiary care center.

Patients: Between June 2004 and February 2016, a total of 212 congenital diaphragmatic hernia survivors enrolled in our follow-up program. Neurodevelopmental outcome was assessed at a median age of 22 months (range, 5–37) using the Bayley Scales of Infant Development, third edition. Fifty patients (24%) required extracorporeal membrane oxygenation support. Four patients (8%) were repaired prior to cannulation, 25 (50%) were repaired on extracorporeal membrane oxygenation, and 21 (42%) were repaired after decannulation.

Interventions: None.

Measurements and Main Results: Children with congenital diaphragmatic hernia, who required extracorporeal membrane oxygenation scored on average 4.6 points lower on cognitive composite (p = 0.031) and 9.2 points lower on the motor composite (p < 0.001). Language scores were similar between groups. Mean scores for children with congenital diaphragmatic hernia repaired on extracorporeal membrane oxygenation were significantly lower for cognition (p = 0.021) and motor (p = 0.0005) outcome. Language scores were also lower, but did not reach significance. A total of 40% of children repaired on extracorporeal membrane oxygenation scored below average in all composites, whereas only 9% of the non–extracorporeal membrane oxygenation, 4% of the repaired post–extracorporeal membrane oxygenation, and 25% of the repaired pre–extracorporeal membrane oxygenation patients scored below average across all domains. Only 20% of congenital diaphragmatic hernia survivors repaired on extracorporeal membrane oxygenation support scored within the average range for all composite domains. Duration of extracorporeal membrane oxygenation support was not associated with a higher likelihood of adverse cognitive (p = 0.641), language (p = 0.147), or motor (p = 0.720) outcome.

Conclusions: Need for extracorporeal membrane oxygenation in congenital diaphragmatic hernia survivors is associated with worse neurocognitive and neuromotor outcome. Need for congenital diaphragmatic hernia repair while on extracorporeal membrane oxygenation is associated with deficits in multiple domains. Overall time on extracorporeal membrane oxygenation did not impact neurodevelopmental outcome

All authors: The Center for Fetal Diagnosis and Treatment, The Children’s Hospital of Philadelphia, Philadelphia, PA.

The authors have disclosed that they do not have any potential conflicts of interest.

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Copyright © 2018 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies