pulmonary malformations
Image Gallery
[ (||image_reduire{0,60}|inserer_attribut{alt,Intrapulmonary bronchogenic cyst}) ] [ (||image_reduire{0,60}|inserer_attribut{alt,Intrapulmonary bronchogenic cyst}) ] [ (||image_reduire{0,60}|inserer_attribut{alt,Intralobar sequestration}) ] [ (||image_reduire{0,60}|inserer_attribut{alt,Intralobar sequestration}) ] [ (||image_reduire{0,60}|inserer_attribut{alt,Intralobar sequestration}) ]
Focal developmental malformations of the lung are a heterogeneous group of lesions that display a variety of features. They often are thought to be quite distinct entities, but there is considerable overlap among these lesions.
Both standard nomenclature and common usage do not identify clearly the components of these lesions in a distinctive fashion. A classification based on pathologic features is presented.
A malformation sequence based on airway obstruction during development is proposed as a unifying pathogenetic mechanism for some seemingly unrelated lesions. (#12520470#)
Types (Exemples)
pulmonary agenesis
bronchopulmonary foregut malformations
pulmonary cystic malfomations
- congenital pulmonary airway malformations (CPAMs or CCAMs)
- CPAM type 0
- CPAM type 1
- CPAM type 2
- CPAM type 3
- CPAM type 4
- pleuropulmonary blastoma
congenital lobar emphysema
polyalveolar lobe
pulmonary sequestrations
- extralobar pulmonary sequestration
- intralobar pulmonary sequestration
pulmonary vascular malformations
- pulmonary arteriovenous malformations
- congenital alveolar capillary dysplasia (misalignment of pulmonary veins)
- congenital pulmonary lymphangiectasis
References
Groenman F, Unger S, Post M. The molecular basis for abnormal human lung development. Biol Neonate. 2005;87(3):164-77. PMID: #15591817#
New concepts in the pathology of congenital lung malformations. Langston C. Semin Pediatr Surg. 2003 Feb;12(1):17-37. PMID: #12520470#