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Peripheral Airway Obstruction in Children with Congenital Diaphragmatic Hernia

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A3658 - Peripheral Airway Obstruction in Children with Congenital Diaphragmatic Hernia
Author Block: H. M. Emanuel; The University of Texas Health Science Center, Houston, TX, United States.
Background:
Approximately 30-50% of children surviving congenital diaphragmatic hernia (CDH) develop long-term pulmonary sequelae including pulmonary hypertension, intermittent/exertional dyspnea, and/or asthma-like symptoms. Measurement of pulmonary function is essential to monitor respiratory status in children with CDH. Standard spirometry is the gold standard in clinical assessment of lung function. However, it requires patient effort and cooperation, limiting accurate evaluation of preschool children. Additionally, spirometry doesn’t detect early obstructive changes in smaller airways. Impulse oscillometry (IOS) is a fast, noninvasive, validated technique, even in preschool children, that measures respiratory impedance, detects early changes in obstructive small airway disease, and can be an indicator of lung function. The objective of this case series was to assess usefulness of long-term measurement of IOS in childhood survivors of CDH.
Methods:
A case series of ten children seen in the high-risk, multidisciplinary clinic for CDH at The University of Texas Health Science Center at Houston (UTHealth) from August 2016 through November 2017 with CDH. At the time of enrollment, demographic information including stage defect size, ECMO history and current respiratory symptoms were collected. Impulse oscillometry was performed in accordance with European Respiratory society. Spirometry was also obtained as per ATS guidelines.
Results:
Our cohort consisted of 2 girls and 8 boys aged 2 - 9 years (mean 5.7). 50% were Caucasian, 40% Hispanics, and 10% African-American. CDH study group stage distribution was 20% type A (smallest defect), 30% type B, 40% type C, 10% D (largest defect, most severe). Average NICU length of stay was 45 days. Two patients (20%) required ECMO. While one patient reported chronic respiratory symptoms; 50% reported intermittent episodes of wheezing associated with upper respiratory infections and/or exercise intolerance. Seven patients (70%) of the study subjects had pathological reactance either at Xrs5 or Xrs20 (peripheral airway obstruction). Airway resistance (R) was normal in all subjects. Five patients were able to perform spirometry: 4 were normal and one patient had an obstructive pattern
Conclusion:
Despite few documented chronic respiratory symptoms and normal spirometry, peripheral airway obstruction was noted in 70% of patients, as evidenced by abnormal reactance in IOS. Airway resistance (IOS) and spirometry may demonstrate normal readings even in patients with significant peripheral airway disease since these airways account for only 20% of total airway resistance. IOS may prove to be a useful tool to aid evaluation of pulmonary functions and detect early changes in CDH patients.
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