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Pediatric Imaging

E3073. Congenital Lung Malformations: A Pictorial Essay

Kaur N1,  Nepal P2,  Adhikari N2,  Nagar A3,  Ojili V.1 1. The University of Texas Health Science Center at San Antonio, San Antonio, United States; 2. Hamad Medical Corporation, Doha, Qatar; 3. Ohio State University Wexner Medical Center, Columbus, United States

Address correspondence to P. Nepal (

Background Information: Congenital malformations of the lung encompass a wide spectrum with varied clinical presentations. For simplicity and adherence to basic pathophysiology, lung malformations can be classified as bronchopulmonary anomalies, combined lung and vascular abnormalities, and sole vascular anomalies. The purpose of this exhibit is to get acquainted with typical and atypical presentation of congenital lung malformations, judicious diagnostic workup, associated anomalies or syndromes, and treatment options for surgically correctable abnormalities.

Educational Goals/Teaching Points: This exhibit will list congenital abnormalities of the lungs by classifying the spectrum into bronchopulmonary, combined lung and vascular abnormalities, and solely vascular anomalies. A brief illustration of developmental anatomy of foregut and lungs that helps in understanding disease spectrum will be provided. We will describe common and uncommon clinical imaging features, judicious use and sequencing of diagnostic imaging modalities, complications, and associated anomalies and syndromes. Finally, we emphasize the radiologist’s role in differentiating the diagnosis and guiding surgeons and physicians with knowledge of the spectrum of lung malformations.

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques: The spectrum of lung malformations that we wish to exhibit includes bronchopulmonary anomalies (pulmonary hypoplasia, congenital pulmonary airway malformation, congenital lobar emphysema, bronchial atresia and agenesis, and congenital cysts), combined lung and vascular abnormalities (scimitar syndrome and pulmonary sequestration), and vascular anomalies (pulmonary artery agenesis, pulmonary arterial sling, pulmonary arteriovenous malformations [AVMs], and AVM syndromes). Imaging modalities presented include chest radiography, antenatal ultrasound, chest CT, and contrast and barium studies. We will discuss various imaging techniques such as chest CT and CT angiography as well as modified protocols and respiratory and ECG gating for vascular malformations.

Conclusion: This exhibit highlights the common lung malformations and important associated anomalies and describes the radiologic findings to offer some clues for the differential diagnosis.