PP‐10 CHARACTERIZATION OF ESOPHAGEAL MOTILITY IN INFANTS BORN WITH CONGENITAL DIAPHRAGMATIC HERNIA USING HIGH RESOLUTION MANOMETRY

Abstract

Purpose:Congenital diaphragmatic hernia (CDH) is a rare condition characterized by a congenital defect in the diaphragm. Abdominal organs are displaced into the thorax through the diaphragmatic defect, hereby causing lung hypoplasia. Survivors also present with long‐term functional gastro‐intestinal morbidity such as gastro‐esophageal reflux, esophageal dysfunction and poor nutritional status. Congenital esophageal dysmotility and surgical disruption of the esophago‐gastric junction (EGJ) may contribute to this esophageal dysfunction. The aim of this study was to characterize esophageal function in young infants born with CDH.Methods:High resolution solid state manometry was used to investigate the esophageal function in 9 postoperative infants born with CDH (6 M, median age 23 days). A total of 416 liquid swallows were analyzed (range 27–70 swallows per patient; mean 46 swallows). All data are presented as median (IQR).Results:We included 9 patients: 8 with a left diaphragmatic defect, 5 with a “liver up” defect, 4 underwent a fetal tracheal occlusion procedure because of severe lung hypoplasia, 8 needed a patch to close a large diaphragmatic defect. Across patients, length of peristaltic defects was 0.4 (0.3,0.6) cm. Distal contractile integral (DCI) was 357 (249,428) mmHg.sec.cm. Distal Latency (DL) was 4.9 (3.6,5.6) sec, whereby 5/9 patients had a DL less than 4.5 sec. EGJ resting pressure was 37 (17,60) mmHg. The integrated relaxation pressure (IRP4) was 14 (9,19) mmHg with IRP4 over 15 mmHg in 4 patients.Conclusion:We investigated the esophageal body and EGJ function in 9 patients with CDH using high resolution manometry. This pilot study shows that the distal contractile integral is reduced in all infants with CDH. This reflects weak esophageal contractile strength and supports the congenital foregut dysmotility hypothesis. Surprisingly, deglutitive relaxation pressures were increased in only 4/9 patients suggesting that EGJ outflow obstruction post hernia repair of the crural diaphragm, is only partially contributing to the esophageal motor dysfunction observed in patients with CDH.