Prenatal diagnosis and management of congenital lobar emphysema

Abstract

Background: Congenital lobar emphysema (CLE) is a rare anomaly of lung development that usually presents in the neonatal period with respiratory distress and pulmonary lobar hyperinflation. The routine use of prenatal ultrasonography has resulted in the early identification and serial evaluation of congenital lung lesions. CLE can be distinguished from other congenital lung lesions on ultrasonography by the differences in echogenicity and reflectivity.

Methods: Two cases of CLE diagnosed at midgestation by ultrasonography and ultrafast fetal magnetic resonance imaging (MRI), along with serial sonographic 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 adenomatoid malformation and bronchopulmonary sequestration. However, these neonates with CLE showed marked air-trapping and respiratory distress requiring 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 those in whom the lesions appear to have resolved in utero. These patients should have ready access to postnatal surgical intervention.