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Congenital pulmonary airway malformation (CPAM), formerly known as congenital cystic adenomatoid malformation (CCAM), is a congenital disorder of the lung similar to bronchopulmonary sequestration. In CPAM, usually an entire lobe of lung is replaced by a non-working cystic piece of abnormal lung tissue.
Congenital lobar emphysema (CLE), also known as congenital lobar overinflation and infantile lobar emphysema, [37] is a neonatal condition associated with enlarged air spaces in the lungs of newborn infants. It is diagnosed around the time of birth or in the first 6 months of life, occurring more often in boys than girls.
Chest x-ray of infant showing CPAM in the left lung causing a mediastinal shift towards the right. The cysts appear as bubbles in the left lung. Congenital pulmonary airway malformation (CPAM) is a rare disease in which the lung airways develop abnormally in the fetus. This leads to infants having pockets of air and cystic masses in their lungs.
Massive lobar emphysema may result from the compression of bronchi by dilated pulmonary arteries, which typically causes respiratory distress. The pulmonary artery's infundibulum's orientation regulates the pulmonary arteries' preferred dilatation as well. Infundibulum is typically vertical and brief.
There is still much debate to whether pulmonary sequestration is a congenital problem or acquired through recurrent pulmonary infection. It is widely believed that extralobar pulmonary sequestrations are a result of prenatal pulmonary malformation while intralobar pulmonary sequestrations can develop due to recurrent pulmonary infections in adolescents and young adults.
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This category reflects the organization of International Statistical Classification of Diseases and Related Health Problems, 10th Revision. Generally, diseases outlined within the ICD-10 codes Q30-Q34 within Chapter XVII: Congenital malformations, deformations and chromosomal abnormalities should be included in this category.