[Congenital lobar emphysema in childhood].
Over a 20-year period, thirty-two children with infantile (congenital) lobar emphysema (ILE) are subjected to treatment in the department of pediatric chest surgery--Emergency Medicine Institute "N. I. Pirogov". The age distribution of the patients is as follows: neonates--7 (21.8 per cent), sucklings--17 (53.8 per cent), 1 y.--5 (15.6 per cent), 2 y.--2 (6.2 per cent), 12 y.--1 (3.1 per cent). In seventeen children (53.3 per cent) the lobar emphysema is located in the left upper lobe. The onset of the clinical picture is in the neonatal period (17 cases, 53.3 per cent) and in the suckling age (13 cases, 40.6 per cent), and becomes manifest with: dyspnea--16 children (50.0 per cent), tachypnea--26 (81.2 per cent) and cyanosis--18 (56.2 per cent). Twenty-seven children are admitted with varying degree respiratory insufficiency (84.4 per cent). Diagnosis is made on the ground of conventional roentgen examinations mainly. Stenosis of the respective lobar bronchus is discovered in 8 children by CAT and bronchoscopy. Scarce vascular pattern is established angiopulmographically in 87.5 per cent of children, and fan-like displacement of the vessels-in 75.0 per cent. All children undergo operation--removal of the emphysematous lobe. Morphological study of the resected lung shows cartilage deficit in the bronchial wall in 14 children (34.5 per cent), bronchial stenosis in 26.2 per cent whereas in 43.8 per cent the underlying cause of ILE is unclear. In 4 children (12.5 per cent) the outcome is lethal because of inflammatory pulmonary process in the postoperative period.
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D001999:Bronchoscopy; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D011013:Pneumonectomy; D011183:Postoperative Complications / Q000453:epidemiology; D011656:Pulmonary Emphysema / Q000150:complications / Q000151:congenital* / Q000175:diagnosis / Q000601:surgery; D014057:Tomography, X-Ray Computed