Long-Term Follow-Up of Children with Diaphragmatic Hernia

Abstract

From 1962 to 1988, 147 neonates were admitted for operative repair of CDH to the Division of Pediatric Surgery in the Children's Hospital of Cologne. Follow-up studies were performed on 45 patients ages 1 to 25 years representing 54.2% of the 83 survivors. 18 patients (40%) were entirely without any complaints, 11 patients (24.4%) had increased rates of respiratory infections. Especially in early childhood they suffered from obstructive alterations in the respiratory tract. No restrictive pulmonary changes were observed. Scintigraphic ventilation tests were performed on 44 cases. 34 (77%) tests indicated absolutely normal results. Some rare local deficits of lung ventilation were based on adhesive anatomic alterations of the thoracic skeleton and the diaphragm. The lung function tests conducted in 29 persons ages 6 to 25 years revealed that the vital capacity and the forced expiratory volume were all normal. We found an increase of the intrathoracic gas volume in 11 infants (disposition to pulmonary inflation). In 9 cases we observed a decrease in the mid expiratory flow curves and in 6 patients we measured an increase in the specific resistance of airways (tendency towards obstructive airway alterations). Nevertheless even former hypoplastic lungs revealed a good extensibility.

The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity

“…In the study from Marven et al [66], CDH patients perceived their own fitness to be worse than the healthy control group. Other studies demonstrate that around 20% of interviewed CDH patients consider themselves less fit than their healthy peers [59,65,73]. It seems most likely that the decreased V 0 O 2 max found by Zaccara et al is due to a lower degree of physical fitness.…”

“…FEV1 and/or FEV1/FVC are decreased in patients with airway obstruction. Wischerman et al [59] reported normal FEV 1 in CDH patients, but found a tendency to obstruction of the smaller airways illustrated by reduction in the mid-expiratory flow rate and raised specific airway resistance. There was no correlation between the extent of pulmonary hypoplasia, assessed during the operation and by pre-and postoperative chest Xrays, and reduction of airflow rate [59].…”

“…Wischerman et al [59] reported normal FEV 1 in CDH patients, but found a tendency to obstruction of the smaller airways illustrated by reduction in the mid-expiratory flow rate and raised specific airway resistance. There was no correlation between the extent of pulmonary hypoplasia, assessed during the operation and by pre-and postoperative chest Xrays, and reduction of airflow rate [59]. Although FEV 1 improves in approximately half of the patients after bronchodilator therapy [68,69], it remains significantly lower when compared to healthy subjects [69].…”

“…Effects on pulmonary function of CDH patients treated with fetal TO are preliminary. Keller et al [43] [48,[59][60][61]. Bronchodilators and inhaled steroids were, at least transiently, used by the majority of CDH patients, during the first year of life and thereafter [61,62].…”

“…It is hypothesized that reduced-lung tissue and consequent alveolar distension may result in early airway closure as found in the aging emphysematous lung, which could be an additional risk factor in the development of chronic obstructive disease on the longer term [48,71]. Some longterm follow-up studies indicate a higher incidence of restrictive lung function impairment among CDH patients compared to the healthy population [63,65] especially when there was a large diaphragmatic defect [65], while others cannot confirm this finding [59,66]. Overall CDH patients appear not to have an important reduction of total lung capacity in their school-and adolescence years [66,68,69].…”