Sequestrations, congenital cystic adenomatoid malformations, and congenital lobar emphysema.
The respiratory system begins to develop at 3 weeks gestation, and aberrations in developmental processes may give rise to a group of structural abnormalities collectively referred to as bronchopulmonary foregut malformations (BPFMs). These lesions or anomalies may subsequently present in the newborn period as pulmonary parenchymal abnormalities in association with significant respiratory compromise. This article briefly reviews fetal lung development and then proceeds to delineate the characteristics, presentation, and surgical treatment of 3 of the more common BPFMs, those being sequestrations, congenital cystic adenomatoid malformations, and infantile lobar emphysema. The various types of BPFMs may occur in conjunction with one another or in association with other congenital anomalies, and these lesions should be managed with a thoughtful and aggressive surgical approach.
Mendeloff
Eric N
EN
Congenital Medical City Children's Hospital, Dallas, TX 75230, USA. emendeloff@casant.com
D001998:Bronchopulmonary Sequestration / Q000175:diagnosis / Q000601:surgery; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D011656:Pulmonary Emphysema / Q000151:congenital* / Q000175:diagnosis / Q000601:surgery