Perinatal Natural History and Treatment of Congenital Lung Malformations in Prenatally Diagnosed Neonates

Abstract

Congenital lung malformations comprise a spectrum of disease entities, including congenital pulmonary airway malformation, bronchial atresia, and intra- and extralobar sequestration. With the progress in prenatal diagnosis, fetuses diagnosed as CLM has been increasing. However, the perinatal clinical features of each disease have not yet been described. They comprise a broad spectrum of symptom ranging from complete regression of the lesion to rapid growth resulting in hydrops fetalis. In addition, the indication and the timing for surgery are still controversial. Thus, strategy to evaluate patients’ degree of severity and customize treatment to each patient’s particular need is vital. We have three types of surgical timings; standby emergency surgery for respiratory failure just after planned cesarean section, emergency surgery for respiratory distress in neonatal period and elective surgery for asymptomatic children around 1 year of age. The prognosis is quite excellent in non-hydrotropic fetuses. The outcome is still poor in spite of planned fetal interventions and standby emergency surgery in neonates with hydrops fetalis. The alternative strategy is needed for the better outcome of these severer hydrotropic fetuses.