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Infantile lobar emphysema (ILE) in 32 children was treated surgically. Most cases presented in infancy with respiratory distress, often involving the left upper lobe, with surgical removal of the affected lobe being the standard treatment.
Area of Science:
- Pediatric Surgery
- Pulmonology
- Congenital Lung Abnormalities
Background:
- Infantile (congenital) lobar emphysema (ILE) is a rare but serious condition affecting newborns and infants.
- Early diagnosis and surgical intervention are crucial for managing ILE and improving patient outcomes.
Purpose of the Study:
- To analyze the clinical characteristics, diagnostic methods, and surgical outcomes of 32 children treated for ILE over a 20-year period.
- To identify potential causes and contributing factors to ILE in pediatric patients.
Main Methods:
- Retrospective review of 32 pediatric patients diagnosed with ILE and treated surgically.
- Analysis of patient demographics, clinical presentation, diagnostic imaging (X-ray, CT, bronchoscopy, angiopulmography), operative procedures, and postoperative outcomes.
- Morphological examination of resected lung tissue to identify underlying pathological changes.
Main Results:
- The majority of patients (53.8%) were infants, with onset typically in the neonatal or suckling period, presenting with dyspnea, tachypnea, and cyanosis.
- Left upper lobe was the most common site (53.3%). Respiratory insufficiency was present in 84.4% of cases.
- Surgical resection of the emphysematous lobe was performed on all patients. Morphological studies revealed bronchial wall cartilage deficit in 34.5% and stenosis in 26.2%, with unclear etiology in 43.8%.
- A 12.5% mortality rate was observed, primarily due to postoperative inflammatory pulmonary processes.
Conclusions:
- Surgical lobectomy remains the primary treatment for infantile lobar emphysema.
- While imaging and bronchoscopy aid diagnosis, the etiology of ILE is often multifactorial or unclear.
- Postoperative inflammatory complications pose a significant risk and require careful management.
Abstract:
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.