Abstract

Background and Aim: Congenital diaphragmatic hernia (CDH) is a rare defect often associated with pulmonary hypoplasia and abnormal pulmonary vascular development. Even after successful hernia repair, pulmonary disease may persist into adulthood. Impaired diaphragmatic motility may lead to compromised respiratory function long after index repair. This study investigates whether a novel ultrasound measurement, the diaphragmatic excursion ratio, can be a simple and non-invasive method to evaluate routine diaphragmatic motion after CDH repair, and whether it correlates with adverse surgical and respiratory outcomes.Materials and Methods: A cross-sectional study was conducted in consecutive patients who presented at medium-term follow-up visit between December 2017 and December 2018 after CDH repair at single pediatric hospital. Transthoracic ultrasound was performed with craniocaudal diaphragmatic excursion measured bilaterally during routine breathing. Diaphragmatic excursion ratios (diaphragmatic excursion of repaired vs. unrepaired side) were calculated and retrospectively compared with clinical data including demographics, length of stay, respiratory adjuncts, oral feeding, and need for gastrostomy.Results: Thirty-eight patients (median age at ultrasound, 24 months, interquartile range 11–60) were evaluated. Nine patients underwent primary repair, 29 had non-primary repair (internal oblique muscle flap or mesh patch). Patients with a diaphragmatic excursion ratio below the median (0.54) had longer hospital stays (median 77 vs. 28 days, p = 0.0007) more ventilator days (median 16 vs. 9 days, p =0.004), and were more likely to have been discharged on oxygen (68 vs. 16%, p = 0.001). They were also less likely to be exclusively taking oral feeds at 1-year post-surgery (37 vs. 74%, p = 0.02) and more likely to require a gastrostomy tube in the first year of life (74 vs. 21%, p = 0.003).Conclusions: Transthoracic ultrasound after CDH repair is practical method to assess diaphragm motion, and decreased diaphragm excursion ratio is associated with worse respiratory outcomes, a longer length of stay, and dependence on gastrostomy tube feeding within 1 year. Further prospective studies may help validate this novel ultrasound measurement and offer prognostic value.

Highlights

  • Congenital diaphragmatic hernia (CDH) is a rare congenital defect of the diaphragm that is often associated with abnormal pulmonary development

  • In order to create a metric that could possibly be compared between children of different sizes, the diaphragmatic excursion ratio (DER) was defined as ratio of the repaired-side diaphragmatic excursion (RDE) to the contralateral-side diaphragmatic excursion (CDE) during quiet breathing: DER = RDE/CDE

  • Our study demonstrated a novel application of transthoracic ultrasound to assess diaphragmatic excursion during quiet breathing in infants and children after CDH repair

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Summary

Introduction

Congenital diaphragmatic hernia (CDH) is a rare congenital defect of the diaphragm that is often associated with abnormal pulmonary development. Pulmonary hypoplasia contributes to significant respiratory morbidity after surgical repair of CDH and obstructive pulmonary disease may persist into adulthood [1]. As mechanical forces are critical to normal lung development [2], there has been considerable interest in studying the respiratory mechanics of patients after CDH repair to identify patients at risk of respiratory complications, and to understand the changes in diaphragm function after repair. Either as a result of disease severity or of some feature of the repair, mesh repair is an independent predictor of poor outcomes in patients with CDH [3, 4]. Congenital diaphragmatic hernia (CDH) is a rare defect often associated with pulmonary hypoplasia and abnormal pulmonary vascular development. This study investigates whether a novel ultrasound measurement, the diaphragmatic excursion ratio, can be a simple and non-invasive method to evaluate routine diaphragmatic motion after CDH repair, and whether it correlates with adverse surgical and respiratory outcomes

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