Congenital Pulmonary Malformations in Pediatric Patients: Review and Update on Etiology, Classification, and Imaging Findings

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Complexities and uncertainties regarding the etiology of congenital pulmonary malformations

Understanding congenital pulmonary malformations is often difficult and confusing, primarily because the etiology of these malformations has not been clearly established. Among many potential etiologic factors proposed so far for the development of congenital pulmonary malformations, there are 4 main theories: (1) defective foregut budding, differentiation, and separation13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25; (2) airway obstruction26, 27; (3) vascular abnormality13; and (4) genetic

Imaging algorithm and techniques

A detailed discussion of imaging techniques for the currently available imaging modalities in the evaluation of congenital pulmonary malformations is beyond the scope of this article. The focus here is on reviewing practical imaging algorithms and basic techniques for evaluating congenital pulmonary malformations in pediatric patients.

Congenital pulmonary malformations are increasingly being detected with prenatal ultrasonography (US), often supplemented by magnetic resonance (MR) imaging.3, 4,

Spectrum of congenital pulmonary malformations in pediatric patients

Classification of congenital pulmonary malformations is both challenging and often controversial from embryologic, radiologic, pathologic, and clinical viewpoints. There have been several ways of classifying congenital pulmonary malformations in the past.40, 41 Some investigators used embryology as their base and classified congenital pulmonary malformations according to the stage of intrauterine development in which the event giving rise to the malformation occurs.41 Other investigators

Summary

Congenital pulmonary malformations are a group of various anomalies involving lung parenchyma, pulmonary artery, and pulmonary vein, which can be the source for important morbidity and mortality in infants and children. The ability to diagnose congenital pulmonary malformations in children has advanced tremendously over the past decade. Traditional methods of chest radiographs, barium swallow study, bronchography, and conventional pulmonary angiography for evaluating these lesions are now being

Acknowledgments

The authors thank Harry Kozakewich, MD, for providing gross photographs of lungs demonstrating bronchial atresia.

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