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Prenatal diagnosis and management of congenital pulmonary airway malformation

Alexia Egloff, MD
Dorothy I Bulas, MD
Section Editors
Deborah Levine, MD
Louise Wilkins-Haug, MD, PhD
Deputy Editor
Vanessa A Barss, MD, FACOG


Congenital lung masses include congenital pulmonary airway malformations (CPAMs), bronchopulmonary sequestration (BPS), and congenital lobar emphysema with bronchial atresia. Hybrid lesions have also been described, suggesting these masses represent a spectrum of abnormalities [1].

Prenatal diagnosis of these disorders has increased with widespread use of prenatal ultrasonography [2]. In addition, use of prenatal ultrasound and magnetic resonance (MR) imaging has improved understanding of the natural history of fetal lung lesions. Ultrasound evaluation is sensitive for the diagnosis of many prenatal lung lesions, but has low specificity [3]. Fetal MR imaging improves evaluation of pulmonary anatomy, which can help to confirm or exclude the suspected diagnosis, and provides additional information useful for counseling and obstetrical management [4-6]. A definitive diagnosis is usually not possible without surgical resection and histopathological evaluation [1,7,8].


Ultrasound is the primary modality for imaging the fetal chest and detecting fetal chest masses [9], but MR imaging can be a useful adjunct for confirming the presence of a mass, further characterizing normal and abnormal anatomy, and providing assessment of residual lung volume. Assessment of lung parenchyma sonographically can be limited due to maternal obesity, oligohydramnios, overlying ribs, and fetal lie; MR is less limited by these factors.

Ultrasound — On ultrasound, the normal fetal chest is oval or round, with the heart positioned in the anterior half of the left chest and bordered by lung parenchyma (image 1A-B). Inferiorly, the diaphragms are dome-shaped and hypoechoic. The fetal airway is not well delineated by ultrasound. Lung parenchyma appears homogeneous and slightly hyperechogenic compared to the fetal liver, and its echogenicity increases with gestational age [10].

Fetal lung volume increases with gestational age with the right lung typically measuring 56 percent of total lung volume. Three-dimensional (3D) and four-dimensional (4D) ultrasound imaging appear to be more useful than two-dimensional ultrasound for estimating lung volume [11].


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Literature review current through: Sep 2016. | This topic last updated: Oct 12, 2016.
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