PT - JOURNAL ARTICLE AU - Khirani, Sonia AU - Amaddeo, Alessandro AU - Olmo-Arroyo, Jorge AU - Lapillonne, Alexandre AU - Becquet, Odile AU - Khen-Dunlop, Naziha AU - Delacourt, Christophe TI - Longitudinal evaluation of diaphragmatic function in children with congenital diaphragmatic hernia AID - 10.1183/13993003.congress-2016.OA4553 DP - 2016 Sep 01 TA - European Respiratory Journal PG - OA4553 VI - 48 IP - suppl 60 4099 - http://erj.ersjournals.com/content/48/suppl_60/OA4553.short 4100 - http://erj.ersjournals.com/content/48/suppl_60/OA4553.full SO - Eur Respir J2016 Sep 01; 48 AB - Background: Congenital diaphragmatic hernia (CDH) is a rare malformation, causing respiratory distress in newborns, and requiring surgical treatment. Few studies have evaluated diaphragmatic function, but none has performed a longitudinal evaluation.Aims and objectives: To assess the respiratory function in CDH children at different post-surgery periods, with emphasis on diaphragmatic function.Methods: Breathing pattern and respiratory muscle strength were measured by means of esophageal (Pes) and gastric (Pgas) pressures.Results: Twenty-eight patients with antenatal or birth day CDH diagnosis (24 left, 4 right) were included. A diaphragmatic patch was necessary in 6 patients. Twelve patients were assessed prior to NICU discharge (N), 6 about 1-2 years after surgery (S1), and 10 about 5-6 years after surgery (S5). Mean antenatal estimated pulmonary volume (VLA) was 41±10%pr. Gastric pressure during sniff (SniffPgas) was negative at N (-58±22 cmH2O) and S1 (-53±36 cmH2O), reflecting diaphragmatic dysfunction, and improved significantly at S5 (+3±9 cmH2O; p<.001). Transdiaphragmatic pressure during sniff was low at N (47±18 cmH2O) but improved significantly at S5 (81±15 cmH2O; p=.008). Expiratory muscle strength was normal and stable at any age. Mean diaphragmatic endurance (TTdi) was high at N (0.10±0.04), reflecting a reduced capacity to sustain a respiratory effort, and significantly reduced at S5 (0.03±0.01; p<.001). VLA highly correlated with neonatal TTdi (r=-0.980, p<.001) and SniffPgas (r=0.897, p<.001).Conclusions: Diaphragmatic dysfunction is present at neonatal period in CDH and improves slowly with age. VLA is predictive of diaphragmatic capacity in the neonatal period.