Pediatric Congenital Lung Malformation: Advanced Imaging Techniques in Pre- and Neonatal Evaluation

Abstract

Pediatric congenital lung malformations (CLMs) comprise a spectrum of developmental anomalies of lung parenchyma, airways, and vasculature. CLMs are increasingly diagnosed prenatally but remain best characterized by postnatal cross-sectional imaging. During pregnancy, ultrasound (US) and fetal magnetic resonance imaging (MRI) are commonly used to monitor lung lesions. Management of CLMs, including imaging, in infants and young children depends on associated symptoms and institutional standards. Chest CT angiography (CTA) is usually the most appropriate initial postnatal imaging modality for assessing prenatally diagnosed or clinically suspected CLMs in asymptomatic infants and children. Magnetic resonance (MR) imaging/magnetic resonance angiography (MRA) may be considered as a complementary, problem-solving, imaging modality for evaluation of CLMs during fetal and neonatal periods. This article presents contemporary perspectives on the imaging approach to pediatric patients with suspected CLMs and reviews up-to-date radiologic findings and clinical characteristics of CLMs.

Keywords: CT; MRI; children; congenital malformation; fetal; imaging; lung; pediatric.

Figure 1

CPAM type I. HASTE oblique sagittal (a) and True FISP (b) MRI scans taken at 28 weeks of gestation reveal a large fluid-filled mass with bright T2 signal in the right chest (arrows). This abnormality results in a shift of the heart toward the left side of the mediastinum.

Figure 2

CPAM type II. Axial HASTE T2 MR images show a hyperintense lesion characterized by at least six cystic images (red arrow) of 2–6 mm at the lung base, with extension to the right posterior lung (orange arrow). No compression on the diaphragm or mediastinum. Minimal pleural effusion layer (white arrow).

Figure 3

CPAM type III. Coronal T2 HASTE image shows, diffuse hyperintensity of the entire left lung (white arrow), without evidence of recognizable cystic structures. Note the normal intensity of the right lung (red arrow).

Figure 4

Sagittal T1w FLASH MRI sequence shows increased signal intensity in the left abnormal pulmonary parenchyma (white arrow) and the compressed signal of the right lung (red arrow). A differential diagnosis to consider is congenital lobar overinflation (CLO), where a more homogeneous signal intensity would typically be observed.

Figure 5

T2 HASTE axial plane (a), coronal (b) and sagittal (c): shows a multicystic brilliant formation (white arrow), at the level of the right and middle lower lobes. The lesion represents 55% of the right lung volume.

Figure 6

Hybrid congenital malformation consisting of lung seizure and CCAM. CECT MPR reconstruction (a), VR (b), and axial (c,d) images show the presence of a lobulated-margin formation located in the lower lobe of the left lung, which is consists of multiple cystic formations (diameters ranging between a few millimeters and 1.5 cm) and a consolidation zone corresponding the costodiaframmatic recess, which is supplied by a voluminous arterial branch originating from the left lateral wall of the aorta in the thoraco-thoracic passage.

Figure 7

BPS at 27 weeks gestation. The coronal image shows a mass with higher signal intensity than the normal lung but lower signal intensity than amniotic fluid. The consolidation shifts the heart to the right (black arrow). There is a feeding artery from the aorta to suggest the diagnosis of sequestration (blue arrow).

Figure 8

Intra-lobar bronchopulmonary sequestration. The Contrast-enhanced computed tomography (CECT) axial (a), coronal MPR reconstruction (b), and volume rendering reconstruction (c) images show a parenchymal consolidation located in the lower left lobe supplied by an afferent artery originating from the suprarenal tract of the abdominal aorta. Intraoperative findings confirmed the bronchopulmonary sequestration (d).

Figure 8

Intra-lobar bronchopulmonary sequestration. The Contrast-enhanced computed tomography (CECT) axial (a), coronal MPR reconstruction (b), and volume rendering reconstruction (c) images show a parenchymal consolidation located in the lower left lobe supplied by an afferent artery originating from the suprarenal tract of the abdominal aorta. Intraoperative findings confirmed the bronchopulmonary sequestration (d).

Figure 9

Bridging bronchus type 2. Non-contrast CT coronal (a) and VR (b) images reveal the RMB ending in a diverticulum. The right lung is ventilated by a displaced RMB arising from the LMB, forming a pseudo-carina, that is at the T6–T7 level and has an inverted T appearance.