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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 pulmonary airway malformation, formerly called congenital cystic adenomatoid malformation, is a benign tumor the results in the formation of single or multiple cysts. [2] Pulmonary sequestration refers to abnormal lung tissue that gets its blood supply from the systemic circulation instead of the pulmonary circulation, like the rest ...
Causes of pulmonary hypoplasia include a wide variety of congenital malformations and other conditions in which pulmonary hypoplasia is a complication. [1] These include congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, fetal hydronephrosis, caudal regression syndrome, mediastinal tumor, and sacrococcygeal teratoma with a large component inside the fetus.
Bronchogenic cysts are usually found in the middle mediastinum. Chest x-rays show a smooth density just in front of the trachea or main stem bronchi at the carinal level. When the cyst communicates with the tracheobronchial tree, the air-fluid level may be seen within the cyst. CT scanning is useful in localizing these cysts.
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.
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.
However, lung biopsy has several complications and is not always diagnostic. [citation needed] Considering its non-invasive methodology, facility of execution, and good patient tolerance, multi-slice spiral CT or CT bronchoscopy should be the test of choice to study cystic lung diseases in particular WCS. [3]
Tracheobronchomegaly is a very rare congenital disorder of the lung primarily characterized by an abnormal widening of the upper airways. [2] The abnormally widened trachea and mainstem bronchi are associated with recurrent lower respiratory tract infection and copious purulent sputum production, eventually leading to bronchiectasis and other respiratory complications.