Background Congenital malformations of the lung are rare and vary widely in their presentation and severity. The evaluation of affected patients frequently requires multiple imaging modalities to diagnose the anomaly and plan surgical correction.

Materials and Methods From January 1987 to March 2008, a total of 18 patients from birth to 5 years of age median, 5.76months with congenital pulmonary malformation were included in this study. Profiles of clinical manifestations, chest radiographs, echocardiographs, computed tomography CT, and magnetic resonance imaging MRI were analyzed to confirm the diagnosis of congenital pulmonary malformations.

Results During 21 years, 18 patients were diagnosed with congenital malformations of the lung, which included bronchogenic cyst 1 case, cystic adenomatoid malformation 3 cases, congenital lobar emphysema 8 cases, pulmonary sequestration 1 case, pulmonary agenesis 2 cases, and pulmonary hypoplasia 2 cases. One patient had three simultaneous abnormalities pulmonary sequestration, bronchogenic cyst and cystic adenomatoid malformation.

Common clinical presentations were respiratory distress 2 cases, respiratory infections 5 cases, and dyspnoea 11 cases. Diagnostic modalities included chest radiography 18 cases, CT scan 14 cases, MRI scan 1 case, and bronchoscopy 3 cases. Thoracotomy with excision of the lesion by lobectomy or pneumonectomy resulted in survival of 14 patients 77. Four deaths 23 were due to pulmonary sequestration 1 case, nosocomial infection 1 case and chronic respiratory insufficiency 2 cases.

Conclusion These data demonstrate that congenital bronchopulmonary malformations usually can be diagnosed by plain chest xray films. Computed tomography may occasionally be necessary. All lesions, including symptomatic lesions in neonates, can be managed surgically soon after diagnosis.