Objective: To evaluate clinical features of malformations of lung and mediastinum. Methods: Retrospective analysis of 51 patients with malformations identified in the author's division between 1982 and 1997. They verified the clinical manifestations, diagnostic procedures, and treatment. Results: The most frequent anomaly was esophageal atresia/tracheoesophageal fistula (12), followed by vascular ring (11), congenital lobar emphysema (7), diaphragmatic hernia/eventration (7), bronchogenic cyst (5), pulmonary hypoplasia (4), pulmonary sequestration (3), and arterial-venous fistula (2). Age at diagnosis varied from birth to 10 years. Most of them exhibited symptoms at first month, some even at birth. The main respiratory symptoms were tachypnea, coughing, retractions, dyspnea, wheezing, pneumonia and rales. Chest X-rays with barium swallow were most important for diagnosis. When chest radiology was inconclusive, CT scans were next in achieving diagnosis. Surgical intervention was the effective treatment in 86% of cases. Conclusions: Lung and mediastinal congenital malformations with respiratory symptoms require adequate diagnostic evaluation and immediate intervention. The most frequent respiratory symptoms were coughing and tachypnea and chest X-rays with barium swallow was the main diagnostic method. Survival is good and mortality is secondary to associated malformations.

Keywords: Bronchopulmonary malformations. Respiratory distress. Retrospective studies.