Neurodevelopmental outcomes in CDH survivors: A single institution's experience
Joseph T Church1, Rodrigo Mon1, Tiffany Wright1
1Section of Pediatric Surgery, Department of Surgery, Michigan Medicine, Ann Arbor, MI.
Insights
Congenital diaphragmatic hernia (CDH) survivors often experience neurodevelopmental delays. Associated anomalies and extracorporeal membrane oxygenation (ECMO) use are key predictors of these outcomes in CDH patients.
Area of Science:
- Pediatric Surgery
- Neonatal Medicine
- Developmental Pediatrics
Background:
- Congenital diaphragmatic hernia (CDH) is a severe condition associated with significant morbidity in survivors.
- Long-term neurodevelopmental outcomes in CDH survivors require further investigation to identify predictive factors.
Purpose of the Study:
- To evaluate the neurodevelopmental outcomes of congenital diaphragmatic hernia (CDH) survivors.
- To identify prenatal and perinatal factors associated with neurodevelopmental outcomes in CDH survivors.
Main Methods:
- Retrospective observational study of 68 CDH survivors born between July 2006 and March 2016.
- Neurodevelopment assessed using Peabody Developmental Motor Scales (PDMS-2) for gross, fine, and total motor quotients.
- Analysis included prenatal variables (liver herniation, lung volume), birth demographics, and therapies (ECMO, HFOV/HFJV).
Main Results:
- Mean motor quotients indicated below-average gross and total motor function, and average fine motor function.
- Observed:expected total fetal lung volume (o:eTFLV) predicted fine motor quotient.
- Associated anomalies and extracorporeal membrane oxygenation (ECMO) use were significant predictors for all motor quotients.
Conclusions:
- Associated anomalies and ECMO use are significant predictors of neurodevelopmental delay in congenital diaphragmatic hernia (CDH) survivors.
- These findings highlight the importance of monitoring and early intervention for CDH survivors with these risk factors.
Purpose:
Survivors of congenital diaphragmatic hernia (CDH) face high morbidity. We studied the neurodevelopmental outcomes of CDH survivors at a single institution.
Methods:
CDH survivors born July 2006-March 2016 at a free-standing children's hospital were reviewed. Neurodevelopment was assessed using the Peabody Developmental Motor Scales (PDMS-2) broken into gross, fine, and total motor quotients. Data collected included prenatal variables (liver herniation, defect laterality, observed:expected total fetal lung volume (o:eTFLV) on MRI), birth demographics (sex, race, estimated gestational age (EGA), birth weight (BtWt), 5 min APGAR, associated anomalies), and therapies/hospital course (HFOV/HFJV, ECMO, timing of repair, pulmonary hypertension (PHTN) severity, length of stay, ventilator days). Variables were analyzed using mixed linear modeling.
Results:
Sixty-eight children were included. Most patients had left-sided CDH (55/68, 81%) without liver herniation (42/68, 62%). ECMO utilization was 25/68 (37%). The mean [95% confidence interval] gross motor quotient for the entire cohort was 87 [84-91], fine motor quotient was 92 [88-96], and total motor quotient was 88 [84-93], representing below average, average, and below average functioning, respectively. o:eTFLV predicted fine motor quotient among prenatal variables. Associated anomalies and ECMO use predicted all quotients in the final model.
Conclusions:
Associated anomalies and ECMO use predict neurodevelopmental delay in CDH survivors.
Type Of Study:
Retrospective observational study; Prognostic.
Level Of Evidence:
II.
