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Pulmonary sequelae in survivors of congenital diaphragmatic hernia
A R Falconer1, R A Brown, P Helms
1Hospital for Sick Children, London.
Insights
Congenital diaphragmatic hernia repair survivors show lasting lung abnormalities, including reduced airflow and ventilation-perfusion mismatch. These pulmonary issues may impact long-term health and function.
Area of Science:
- Pediatric Surgery
- Pulmonary Medicine
- Radiology
Background:
- Congenital diaphragmatic hernia (CDH) is a serious birth defect.
- Surgical repair is standard, but long-term pulmonary sequelae are common.
- Understanding post-repair lung function is crucial for patient outcomes.
Purpose of the Study:
- To assess long-term pulmonary function in CDH survivors.
- To identify specific lung abnormalities after CDH repair.
- To compare outcomes based on repair side and ventilation duration.
Main Methods:
- Clinical examination and spirometry in 19 CDH survivors and controls.
- Radiographic assessment of lung volumes.
- Radionuclide ventilation-perfusion (V/Q) lung scans.
Main Results:
- CDH survivors had lower spirometric measurements, notably peak expiratory flow.
- Left lung volumes were enlarged post-left repair, suggesting overdistension.
- V/Q scans revealed ipsilateral lung perfusion deficits, especially with longer ventilation.
Conclusions:
- CDH survivors exhibit significant residual pulmonary abnormalities.
- These abnormalities, including V/Q mismatch, may lead to adult functional impairment.
- Right-sided repair appears to have a better long-term pulmonary outcome.
Abstract:
Nineteen survivors of congenital diaphragmatic hernia repair were compared with age and sex matched control children six to 11 years after repair. All subjects were examined clinically and underwent lung function testing. The patients also had individual lung volumes assessed radiographically and had radionuclide (krypton-81 m, technetium-99 m macroaggregates) ventilation-perfusion (V/Q) lung scans. Four patients had pectus excavatum and two had mild scoliosis. Spirometric measurements were lower in the patients than in the control subjects but only the differences in peak expiratory flow and flow at 50% of expired vital capacity were significant. The radiographic left lung volumes in patients surviving left diaphragmatic repair were larger than expected at 49.3% (SD 2%), suggesting alveolar overdistension. V/Q scans showed a mismatch in the ipsilateral lung, mean Q (40% (7%] being significantly lower than mean V (47% (6%)). In seven patients who had required ventilation for four days or more perfusion to the ipsilateral lung was significantly lower (34% (6%)) than values for the 12 patients ventilated for less than four days (43% (6%)). Survivors of right diaphragmatic repair had a better outcome in terms of relative radiographic lung volumes and V/Q distribution. More severely affected children are now surviving repair of congenital diaphragmatic herniation, with residual pulmonary abnormalities that could produce functional impairment in adult life.