Congenital lung lesions—underlying molecular mechanisms
Section snippets
Normal lung development
The bronchial tree of the human lung has more than 105 conducting and 107 respiratory airways arrayed in an intricate pattern crucial for oxygen flow. Lung development is a highly orchestrated process directed by mesenchymal(vessels)-epithelial interactions, which control and coordinate the temporal and spatial expression of multiple regulatory factors required for proper lung formation. Many endogenous and exogenous biophysical and biochemical factors may disturb this delicate process leading
Congenital cystic adenomatoid malformation
CCAMs are relatively rare developmental abnormalities of the lung that can cause significant morbidity and mortality in infants as the result of associated fetal hydrops, lung hypoplasia, and respiratory distress. The lesions are described as hamartomatous lesions, ie, normal lung tissues in a disorganized spatial arrangement. The result of this process is a multicystic mass that replaces the normal lung structure. Human CCAMs are unilateral and usually confined to a single lobe. The exact
Bronchopulmonary sequestration
BPS are microscopic cystic masses of nonfunctioning pulmonary tissue that lack an obvious communication with the tracheobronchial tree. Typically, the lung tissue in BPS receives all or most of its blood supply from an anomalous systemic artery, with the origin of this artery being variable. Two forms of sequestration are recognized: intralobar and extralobar. Although rare, both forms can occur simultaneously. Intralobar sequestrations (ILS) are incorporated into the normal surrounding lung,
Congenital lobar emphysema
CLE is a term used to describe a distended, hyperlucent lobe on plain radiographs, usually the left upper or the right middle lobe.60 Pathologically, a distinction is made between a polyalveolar lobe, in which the number of alveoli is greatly increased, and congenital lobar overinflation, in which the alveoli are markedly distended. CLE is rarely diagnosed prenatally, possibly because of its low prevalence in utero or the increased echogenicity of the lungs, which could be too subtle to be
Bronchogenic cysts
BC result from abnormal budding of the foregut. As foregut duplication cysts, they share common features with esophageal duplication cysts but are characterized by the presence of cartilage, smooth muscle and glands in their wall. The majority is located in the mediastinum, usually adjacent to the distal trachea or proximal main stem bronchi, but they can also be found within the parenchyma of the lung.66 They are usually unilocular, filled with fluid or mucus and generally do not communicate
Concluding remark
The spectrum of congenital lung lesions likely results from disordered embryologic interactions, which occur during the course of fetal lung development. Although some molecular links are being suggested, it is still too early to establish any molecular pathway as the cause of these congenital lesions.
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Cited by (89)
Congenital lung malformations: Dysregulated lung developmental processes and altered signaling pathways
2022, Seminars in Pediatric SurgeryManagement of Congenital Lung Malformations
2022, Clinics in PerinatologyCitation Excerpt :Abnormalities in development in this stage greatly affect the ability to perform gas exchange. The saccular stage (24–38 weeks) is when alveolar saccules form and the alveolocapillary membrane is thin enough to perform gas exchange by 24 weeks.2 Abnormalities in development in this stage are thought to lead to the development of type IV CPAMs.3
Case report: Asymptomatic bronchopulmonary sequestration in an adult with dual celiac and aortic supply
2022, Radiology Case ReportsThe natural history of prenatally diagnosed congenital pulmonary airway malformations and bronchopulmonary sequestrations
2022, Journal of Pediatric SurgeryA clinical guideline for structured assessment of CT-imaging in congenital lung abnormalities
2021, Paediatric Respiratory ReviewsLung and Pleura
2021, Gattuso’s Differential Diagnosis in Surgical Pathology
S.G. was supported by FCT grant ref. SFRH/BD/15,260/2004; Y.H. is supported by the Sophia Foundation (SSWO project 482).