Abstract
Lung function in survivors of congenital diaphragmatic hernia at school age
Background: New therapeutics modalities in the last decades have increased survival rate of patients with severe forms of congenital diaphragmatic hernia (CDH) resulting in increased late morbidity. Surviving children remain at high risk of developing long-term pulmonary consequences. There are limited data regarding lung function (LF) of CDH survivors at school age.
Aims and objectives: to assess LF in CDH survivors at school age.
Methods: CDH survivors treated in University Medical Centre Ljubljana from 2002 to 2010 were recruited in the study. Children underwent spirometry and lung volume measurements. Results were compared with those from a healthy control group matched for sex, age and height.
Results: Data from 13 CDH survivors (8 girls, mean age 11,9 years, SD 3,09) and 11 children in control group (7 girls, mean age 11,9 years, SD 2,98), was eligible for analysis. In CDH group 11 (85%) were early presenters. Forced expiratory volume in 1 s (FEV1) <80% was seen in 6 (45%) of children in CDH group compared to zero in the control group. CDH survivors had significantly lower mean forced vital capacity (FVC) (81% vs 103%; p= 0,01), FEV1 (83% vs 113%; p=0.005), and a significantly higher residual volume/ total lung capacity ratio (RV/TLC) ( 154,3 % vs 107,5%) ; p<0.001). There was no significant difference in the mean FEV1/FVC neither in mean total lung capacity.
Conclusions: CDH survivors have abnormalities in lung function at school age, They have lower percent predicted FEV1 and FVC and higher RV/TLC. The improved survival of patients with more severe forms of diaphragmatic hernia stresses the need for long-term follow-up of lung function.