Congenital parenchymatous malformations of the lung

Congenital lung malformations, primary and secondary, contribute to an impo rtant portion of pediatric thoracic surgery. One purpose of this report is to outline the close relation in terms of embryology and clinical presentat ion of congenital parenchymatous pulmonary malformations. In a retrospectiv e study we also aim to evaluate our experience with the diagnosis and surgi cal management of congenital parenchymatous bronchopulmonary malformations and to compare our data with the literature. From January 1979 to December 1996 a series of 48 patients, 30 males (62.5%) and 18 females (37.5%), were operated on for congenital bronchopulmonary malformations. Pulmonary seque stration, bronchogenic cysts, congenital lobar emphysema, and congenital cy stic adenomatoid malformation were seen in 16, 13, 5, and 14 patients, resp ectively. The first clinical symptoms occurred at a mean age of 8.8 years ( 1 day to 62 years), and the mean age at the time of surgical intervention w as 9.3 years (1 day to 62 years). The maximum time between first symptoms a nd surgical treatment was 27 years. A lobectomy was performed in 22 cases; in the other patients more lung-preserving surgery such as enucleation or s equestrectomy was performed. Only one postoperative death occurred followin g lobectomy for pulmonary sequestration, and it was due to pulmonary hypopl asia and pulmonary hypertension. Eleven other patients presented with posts urgical complications: pneumothorax (n = 5), pleural effusion (n = 3), prol onged air leak (n = 2), portal vein thrombosis (n = 1), and hemorrhage requ iring reintervention (n = 1). We conclude that any thoracic cystic lesion e xpanding on chest radiography should be an indication for surgical resectio n, even if asymptomatic, because of the risk of pulmonary compression, infe ction, or malignant degeneration. In the few cases of a fetal intrathoracic mass, prenatal diagnosis and intrauterine intervention may be indicated, a nd these indications are also discussed.