Congenital Lung Malformations, Update and Treatment

There is a broad spectrum of bronchopulmonary malformations which present in early infancy and childhood. These include Bronchogenic cysts, Bronchopulmonary sequestrations, congenital cystic adenomatoid malformation (CCAM), and congenital lobar emphysema. These lesions maybe detected by prenatal diagnosis, present as acute respiratory distress in the newborn period, or may remain undiagnosed and asymptomatic until late in life. Pulmonary sequestration (PS) is a rare congenital malformation of the lower respiratory tract. It consists of a non-functioning mass of lung tissue that lacks normal communication with the tracheobronchial tree and receives its’ arterial blood supply from the systemic circulation. CCAMs are extremely rare with the reported incidence being between 1/25,000 and 1/35,000 . The pathogenesis is uncertain but appears to result from a abnormality of the branching morphogenesis of the lung and represents a maturation defect. Congenital Lobar Emphysema (CLE) is a rare anomaly of lung development which often presents in the neonatal period with hyperinflation of one or more pulmonary lobes. For the last decade we have preferred to use minimally invasive techniques to perform lobectomies in all of these infants with congenital cystic lesions. The benefits of avoiding a formal thoracotomy and the morbidity associated with it greatly out way the disadvantages of the increased technical difficulty and operative time. CONGENITAL LUNG MALFORMATIONS, UPDATE AND TREATMENT