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[Diagnosis and course of congenital lobar emphysema]
Insights
Congenital lobar emphysema in children often presents later than previously thought, suggesting a need to re-evaluate surgical urgency and age structure. Bronchological examination is crucial for diagnosis.
Area of Science:
- Pediatric Pulmonology
- Thoracic Surgery
- Diagnostic Imaging
Background:
- Congenital lobar emphysema (CLE) is a rare congenital lung malformation.
- Literature typically reports CLE in infants, but this study observed a significant number of older children.
- Understanding the typical age distribution and diagnostic challenges is important for effective management.
Purpose of the Study:
- To analyze clinical data of 22 children diagnosed with congenital lobar emphysema over 20 years.
- To investigate the age structure, affected lobes, and diagnostic findings in CLE patients.
- To evaluate the role of bronchoscopy and bronchography in diagnosis and management, and to reassess surgical indications.
Main Methods:
- Retrospective analysis of 22 pediatric cases of congenital lobar emphysema.
- Review of patient age at diagnosis, clinical presentation, and affected lung lobes.
- Evaluation of diagnostic procedures including bronchoscopy and bronchography.
- Assessment of surgical outcomes and spontaneous regression of emphysema.
Main Results:
- Observed 22 children with CLE, with 9 patients older than one year, deviating from reported age structures.
- The upper lobes were most frequently affected (11 cases), followed by the right upper lobe (6) and right middle lobe (4).
- Bronchoscopy revealed tracheal (2) and main bronchial stenosis (7) in a surprising number of cases; bronchography showed significant bronchial compression.
Conclusions:
- The observed age distribution suggests CLE may be diagnosed later than commonly reported.
- Bronchological examination is vital for differential diagnosis, especially in distinguishing CLE from cystic lung changes.
- Findings support a revised understanding of CLE's age structure and the urgency of surgical intervention.
Abstract:
Within 20 years 22 children with congenital lobar emphysema were observed. 13 children were infants, 9 were already older than a year, thus, clearly deviating from the age structure reported in literature. 15 children underwent an operation, in 2 children the alterations disappeared completely respectively partially. One child prematurely born died before operation. The upper lobe was concerned most frequently (11 times), followed by the right upper lobe (6 times) and the right middle lobe (4 times). Bronchoscopy was showing in surprising frequency a stenosis of the trachea (2 times) respectively of the main bronchus (7 times). In 13 of 15 cases bronchographically investigated the displacement and compression of the adjacent lobar bronchi were predominant. The regression of the emphysema observed in a x-ray picture series initially with an "opaque lung" and later on with spontaneous disappearance of the lobar emphysema is more fully described. In the opinion of the authors the analysis of their cases is justifying a change in the conception concerning age structure and urgency of surgery. Bronchological examination is considered of importance for differential diagnostics especially to delimit cystic pulmonary alteration.