Prenatal diagnosis and management of congenital lobar emphysema

Background: Congenital lobar emphysema (CLE) is a rare anomaly of lung deve lopment that usually presents in the neonatal period with respiratory distr ess and pulmonary lobar hyperinflation. The routine use of prenatal ultraso nography has resulted in the early identification and serial evaluation of congenital lung lesions. CLE can be distinguished from other congenital lun g lesions on ultrasonography by the differences in echogenicity and reflect ivity. Methods: Two cases of CLE diagnosed at midgestation by ultrasonography and ultrafast fetal magnetic resonance imaging (MRI), along with serial sonogra phic documentation of their prenatal course were reviewed. Results: The CLE lesions decreased in size over the course of the pregnancy , similar to that seen with other congenital lung lesions such as cystic ad enomatoid malformation and bronchopulmonary sequestration. However, these n eonates with CLE showed marked air-trapping and respiratory distress requir ing lobectomy in the early neonatal period. Conclusions These cases provide insight into the prenatal course of CLE and underscore the need for continued postnatal evaluation of fetuses even tho se in whom the Lesions appear to have resolved in utero. These patients sho uld have ready access to postnatal surgical intervention. Copyright (C) 200 0 by W.B. Saunders Company.