Congenital lobar emphysema

This paper reviews three cases of congenital lobar emphysema from Saudi Arabia, seen over a period of three years. All were boys aged 2.5, 7 and 8 months, and recurrent attacks of chest infection, which started during the early neonatal period, were the main presentation. In all cases, diagnosis was confirmed by chest x-ray. Two of them had a lobectomy and made a good recovery. Operation was refused in the third. The clinicopathological features, diagnosis and management are discussed. An outline of the anaesthetic management is also discussed briefly.     

Congenital lobar emphysema —A report of 5 cases

Five infants with congenital lobar emphysema whose main symptoms included dyspnea, cyanosis and recurrent respiratory infections, are presented herein. The most reliable diagnostic tool was plain chest X-ray films with antero-posterior and lateral views, while radio-isotopic investigation of the lung perfusion state took second place in the diagnosis of this disease. The affected lobes were the left upper lobe in four patients and the right middle lobe in one. Lobectomy was performed with good results in 4 patients, however, 1 infant was lost following an emergency thoracotomy.     

Congenital lobar emphysema

Congenital lobar emphysema is a cause of respiratory distress during infancy that may present a diagnostic problem. Recognition of this entity is rewarding, because excisional therapy is fairly specific and the results are satisfactory. We present two infants aged 7 weeks and 2 days with congenital lobar emphysema who were diagnosed and treated in our institution for the past two years. Published work is reviewed; diagnostic and treatment issues are discussed.     

Congenital lobar emphysema in adults

Due to the severity of respiratory symptoms, congenital lobar emphysema often requires surgical treatment in the early stages of life. Diagnosis of congenital lobar emphysema in adult life is therefore unusual, often presenting with mild symptoms. Diagnostic assessment is therefore of great importance in the treatment of these patients. A case of congenital lobar emphysema in an adult is reported and the diagnostic and therapeutical approach are discussed. The use of new diagnostic techniques such as dynamic RMN and SPET V/Q lung scan may improve the accuracy of the diagnostic evaluation. Pulmonary lobectomy led to marked improvement in respiratory function. Accurate diagnosis and appropriate management of congenital lobar emphysema in adult patients can lead to favourable results.     

Surgical treatment of infantile lobar emphysema in cardiovascular disease with left-to-right shunts

Serious symptoms associated with the lobar emphysema in congenital heart diseases with left-to-right shunt disappeared after radical operation for cardiac lesion. The study of seven autopsied cases revealed that the lobar emphysema resulted from check valve mechanism created by compression of the bronchi by distended pulmonary artery. Eight cases of our experience of lobar emphysema disappeared shortly after the operation forccongenital heart disease with left-to-right shunt seems to indicate infantile lobar emphysema in congenital heart diseases with left-to-right shunt should not be treated by resection but rather by radical operation for cardiac lesion as soon as possible even in infancy.     

Congenital bilobar emphysema

Congenital bilobar emphysema is reported to be extremely rare. We describe 3 cases, and we review the diagnosis problems and treatment methods of this childhood respiratory tract pathologic condition.     

Congenital lobar emphysema: diagnostic and therapeutic considerations.

BACKGROUND/PURPOSE: Congenital lobar emphysema (CLE) is characterized by overinflation of a pulmonary lobe and may present as a diagnostic and therapeutic dilemma. The authors have reviewed their experience to clarify the controversies about the diagnosis and management of CLE in children. METHODS: Children who had CLE and were treated at our department between 1991 and 1998, inclusive, were reviewed. RESULTS: Fourteen children consisting of 8 boys and 6 girls (age range, 25 days to 2.5 years) had CLE. Eight children (57%) were symptomatic at birth. Major presenting symptoms were dyspnea (n = 8, 57%), cyanosis (n = 6, 43%) and recurrent respiratory tract infection (n = 4, 28.5%). Chest x-rays and computerized tomography scans showed hyperaeration of the affected lobes in 14 and 12 patients, respectively. Pulmonary perfusion scan was performed in 8 patients and showed loss of perfusion in the affected lobe in 7 cases. The affected sites were left upper lobe (n = 8, 57%), right middle lobe (n = 4, 28.5%) and right upper lobe (n = 2, 14.5%). Congenital cardiac anomalies were encountered in 2 patients (14.5%). Ten children underwent lobectomy, and postoperative course was uneventful. Nonoperative management was performed in 4 cases (28.5%) presenting at older age with milder symptoms. In the latter, although symptoms subsided, radiological abnormalities persisted during the follow-up period of 3 months to 4 years. No mortality was encountered in the series. CONCLUSIONS: The diagnosis of CLE is established by combined radiological and scintigraphic imaging methods. Surgical excision of the affected lobe is the appropriate treatment in all infants under 2 months of age and in infants older than 2 months presenting with severe respiratory symptoms. Infants older than 2 months presenting with mild to moderate respiratory symptoms associated with normal bronchoscopic findings can be treated conservatively. In conservative management, the children should be under close follow-up, and the family should be alarmed about the disease. Further studies are required to determine the long-term outcome of conservative management.     

Congenital lobar emphysema: Like father, like son

Congenital lobar emphysema (CLE) is an uncommon cause of progressive respiratory distress that typically presents in the first few days of life. There has been a previous report of CLE in a mother and daughter. The authors describe 2 cases involving the right upper and middle lobes in a father and son secondary to relative deficiency of the bronchial cartilage. This provides additional evidence for inherited factors in the etiology of CLE.     

Congenital lobar emphysema of left upper lobe. A case report

In a 2 1/2-year-old boy chest X-ray examination because of dyspnoea revealed hyperlucency of the left upper lobe with herniation of the hyperinflated lobe into the right hemithorax. Surgical exploration showed the upper lobe of the trilobed lung to be the site of congenital lobar emphysema, while the lingula and left lower lobe were collapsed. Left upper lobectomy was successfully performed.     

Use of urine drainage bags with unidirectional valve for intercostal drainage

In order to reduce the inconvenience of cumbersome chest drainage bottles and allow greater mobility to the patient, we describe the use of ordinary urinary drainage bags with unidirectional valve as an alternative method of intercostal drainage.     

"Acquired" lobar emphysema: a complication of respiratory distress in premature infants

Ten premature infants with respiratory distress developed a type of pulmonary lobar emphysema that is clinically and histologically distinct from interstitial or congenital lobar emphysema. In eight patients emphysema was noted in the right lower lobe, while in two the left upper lobe was affected. Five infants exhibited nonresolution of the emphysema and an inability to be weaned from ventilator support. Lung scans in these five infants showed absent or markedly decreased perfusion of the involved lobe. Lobectomy was necessary. Postoperatively, their respiratory status improved and they were successfully weaned from the ventilator. Five premature infants with identical radiographic emphysematous changes had normal lung scans. These infants did not require lobectomy and were also successfully weaned from the ventilator. Radiographic changes of lobar emphysema in these five infants completely resolved. Pathologic changes in the excised lobes were characteristic of bronchopulmonary dysplasia and obstructive intraalveolar emphysema. The long-term effects of positive pressure ventilation, oxygen, and suctioning techniques directed toward the right lower lobe may play an important role in the etiology of "acquired" lobar emphysema.