Congenital Malformations and Developmental Anomalies of the Lung

Abstract

Congenital malformations of the lung are a group of diverse, yet related, abnormalities which may involve the lung parenchyma, pulmonary vasculature, or a combination of both. They may be detected in fetal life, produce severe symptoms during infancy, or may not manifest symptomatically until adulthood. The goal of imaging is to demonstrate the various components of the malformation, to facilitate appropriate management. This article discusses the spectrum of congenital and developmental lung anomalies, their etio-pathogenesis, role of various imaging modalities, and characteristic radiological findings.

Congenital lung malformations: can we avoid computed tomography? A five-year study

“…CLMs consist of a continuum of pulmonary development abnormalities that can affect all structures of the respiratory system (lung parenchyma, trachea and bronchi, pulmonary or systemic arteries and veins) as isolated or combined abnormalities, resulting in a large variety of clinical and imaging features. CLMs are generally diagnosed prenatally with ultrasound (US), which is the primary imaging modality for foetal screening [ 3 ] due to its low invasiveness. It provides valuable information about the presence and size of a focal lung lesion [ 11 ], lung hypo-plasia, mass effect, hydrops fetalis, and any other organ malformations, all of which affect the prognosis and mana-gement [ 12 , 13 ].…”

“…Other malformations show different degrees of vascular and pulmonary alterations (bronchopulmonary sequestration), and in some abnormalities, including arteriovenous malformation (AVM), there is an altered vascularity with a regular lung parenchyma [ 2 ]. In many cases, radiological features show a combination of multiple malformations, resulting in hybrid lesions [ 3 ].…”

Prenatal diagnosis of pulmonary artery sling associated with tracheal agenesis: A case report

“…The furrows become progressively deeper and eventually join with the craniad, thereby separating the ventral laryngotracheal tube from the dorsal esophagus 5 . To date, there are four main theories for the development of a congenital trachea and pulmonary malformations 6,7 : (1) defective foregut budding, differentiation, and separation; (2) airway obstruction; (3) vascular abnormality; and (4) genetic causes. These proposed mechanisms may act alone or in combination.…”