Abstract

BackgroundTo evaluate the long-term functional and structural pulmonary development in children with repaired congenital diaphragmatic hernia (CDH) and to identify the associated perinatal-neonatal risk factors.MethodsChildren with repaired CDH through corrective surgery who were born at gestational age ≥ 35 weeks were included in this analysis. Those who were followed for at least 5 years were subjected to spirometry and chest computed tomography for evaluation of their functional and structural growth. Main bronchus diameters and lung volumes (total, left/right) were measured. According to total lung volume (TLV) relative to body surface area, children were grouped into TLV ≥ 50 group and TLV < 50 group and the associations with perinatal-neonatal factors were analyzed.ResultsOf the 28 children (mean age, 6.2 ± 0.2 years) with left-sided CDH, 7 (25%) had abnormal pulmonary function, of whom 6 (87%) showed restrictive patterns. All pulmonary functions except FEF25–75% were worse than those in matched healthy control group. Worse pulmonary function was significantly associated with small head and abdominal circumferences at birth. The mean TLV was 1339.1 ± 363.9 mL and LLV/TLV was 47.9 ± 2.5 mL. Children with abnormal pulmonary function were more likely to have smaller lung volumes. In multivariate analysis, abdominal circumference at birth was significantly associated with abnormal lung volume.ConclusionsA quarter of children with repaired CDH showed abnormal pulmonary function. Small abdominal circumference at birth was associated with abnormal pulmonary function and lower TLV.

Highlights

  • To evaluate the long-term functional and structural pulmonary development in children with repaired congenital diaphragmatic hernia (CDH) and to identify the associated perinatal-neonatal risk factors

  • Subjects We identified neonates with CDH born between January 1, 1994 and December 31, 2012 at Asan Medical Center and underwent corrective surgery in the neonatal intensive care unit; among them, we included those whose follow-up was possible after 5 years in the analysis

  • Neonatal characteristics A total of 30 children with repaired CDH were included in the analysis (Supplementary Table 1)

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Summary

Introduction

To evaluate the long-term functional and structural pulmonary development in children with repaired congenital diaphragmatic hernia (CDH) and to identify the associated perinatal-neonatal risk factors. Congenital diaphragmatic hernia (CDH) is a rare disease that affects approximately 1 in 2500 newborns [1, 2]. Multiple studies have analyzed the growth of lung parenchyma in children with repaired CDH and reported that the disease-involved lungs show catch-up growth [12, 13], albeit to a lesser degree than the contralateral side with findings with such as air trapping and architectural distortion. No studies to date have investigated lung development in such children after the neonatal period or analyzed the associations between functional impairment and structural underdevelopment

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