LIBRO + ANEXOS NEUMOLOGÍA PEDIÁTRICA

Libro Blanco de las ACES Pediátricas 2024 112 ❚ a. Know that congenital pulmonary lymphangiectasia may be primary and associated with generalized lymphangiectasia or syndromes such as Noonan syndrome and anatomic abnormalities such as pulmonary vein stenosis or lymphatic obstruction 2. Etiology/genetics a. Know that unilateral lung agenesis most likely represents defective development during the embryonic stage b. Know that in most cases of congenital lobar emphysema a cause cannot be found, but identified etiologies may include central and lung hyperplasia c. Know that congenital diaphragmatic hernia involves an abnormality of early embryologic development d. Know that sequestration involves an interruption in orderly early lung development and a persistence of the primitive perfusion of the sequestered lung tissue from the systemic circulation e. Know that absent fetal breathing, oligohydramnios, or diaphragmatic hernia may be associated with lung hypoplasia f. Know that tracheomalacia may be idiopathic or associated with bronchopulmonary dysplasia, tracheoesophageal fistula, vascular ring, or other lesions g. Know that a bronchogenic cyst represents an abnormality of early airway development 3. Pathophysiology a. Pathology 1. Know that characteristic histologic findings in congenital lobar emphysema vary but may include decreased bronchial wall cartilage 2. Know that congenital lobar emphysema rarely involves the lower lobes 3. Know the usual sites of diaphragmatic hernias 4. Know the anatomic characteristics of intralobar and extralobar sequestrations 5. Know the common location of pulmonary sequestration (ie, left-sided, lower lobe, posterior, and medial) 6. Know that sequestered lobes are usually perfused by an abnormal systemic artery that may arise from below the diaphragm 7. Know which congenital malformations are supplied by a systemic artery 8. Know that hypoplastic lungs are initially small, with decreased numbers of alveoli and decreased caliber of airways and vessels 9. Recognize the association between lung hypoplasia and the other manifestations of Potter syndrome 10. Know that a tracheal bronchus most commonly arises on the right from the midintrathoracic region of the trachea 11. Recognize the patterns of tracheoesophageal fistula 12. Recognize the association between right or double aortic arch and compression of the trachea due to the vascular ring that is made up in part by these vessels 13. Know that most closed vascular rings include the esophagus and trachea, while pulmonary sling is posterior to the trachea but anterior to the esophagus 14. Know that the scimitar syndrome includes abnormal pulmonary venous drainage and hypoplastic lung 15. Know the usual anatomic location of bronchogenic cysts in the middle or posterior mediastinum