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Long-term pulmonary sequelae in children with congenital diaphragmatic hernia
H Ijsselstijn1, D Tibboel, W J Hop
1Department of Pediatrics, Erasmus University Rotterdam, the Netherlands.
Insights
Congenital diaphragmatic hernia (CDH) survivors show mild airway obstruction and increased responsiveness to methacholine, indicating long-term lung function impairment. Neonatal ventilation and lung hypoplasia contribute to these persistent pulmonary issues.
Area of Science:
- Pediatric Pulmonology
- Neonatal Respiratory Medicine
- Thoracic Surgery
Background:
- Congenital diaphragmatic hernia (CDH) frequently causes neonatal respiratory insufficiency, lung hypoplasia, and pulmonary hypertension.
- Artificial ventilation is often necessary, increasing the risk of bronchopulmonary dysplasia and long-term airway obstruction.
Purpose of the Study:
- To evaluate the long-term pulmonary sequelae in patients with congenital diaphragmatic hernia (CDH).
- To assess residual lung function and airway characteristics in CDH survivors compared to controls.
Main Methods:
- Studied 40 CDH patients (age 7-18 years) and 65 age-matched controls.
- Performed pulmonary function tests including spirometry, lung volumes, and diffusing capacity.
- Assessed airway responsiveness to methacholine (MCH) and metabisulfite (MBS).
Main Results:
- CDH patients exhibited mild peripheral airway obstruction and increased residual volume (RV) and RV/TLC ratio compared to controls.
- Both groups had normal total lung capacity (TLC) and diffusing capacity for carbon monoxide (DLCO).
- Increased airway responsiveness to MCH was common in CDH patients, but bronchoconstriction to MBS was rare.
Conclusions:
- CDH patients demonstrate minor residual lung function impairment, characterized by mild airway obstruction and heightened MCH responsiveness.
- Findings suggest structural changes in distal airways, not autonomic dysfunction, are responsible for persistent abnormalities.
- Neonatal artificial ventilation and residual lung hypoplasia are key factors in long-term pulmonary sequelae in CDH.
Abstract:
Neonates with congenital diaphragmatic hernia (CDH) often suffer from respiratory insufficiency due to lung hypoplasia and pulmonary hypertension. Artificial ventilation is frequently required, and this leads to a high incidence of bronchopulmonary dysplasia. Long-term follow-up studies have shown persisting airway obstruction. To evaluate the long-term pulmonary sequelae in CDH, we studied 40 CDH patients of age 7 to 18 yr (median 11.7 yr) and 65 age-matched controls without CDH and lung hypoplasia who underwent similar neonatal treatment. Mild airway obstruction was found in both groups with more peripheral airway obstruction in CDH patients than in control subjects. Both groups had normal TLC and single-breath carbon monoxide diffusion capacity (DLCO). CDH patients had increased residual volume (RV) and RV/TLC compared with controls. Increased airway responsiveness to methacholine (MCH) was common but bronchoconstriction to inhaled metabisulfite (MBS) was rare both in CDH and control subjects. We conclude that this group of CDH patients has minor residual lung function impairment. Mild airway obstruction and increased airway responsiveness to inhaled MCH but not to MBS suggest that structural changes in distal airways are involved and not autonomic nerve dysfunction. Both artificial ventilation in the neonatal period and residual lung hypoplasia seem important determinants of persistent lung function abnormalities in CDH patients.