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Virtual Pediatric Hospital: Paediapaedia: Cystic Adenomatoid Malformation (CAM) Paediapaedia: Neonatal Chest Diseases

Cystic Adenomatoid Malformation (CAM)

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
The most common presentation is acute respiratory distress in the newborn in the first few hours of life. Alternatively, it can present at several months are several years of age as recurrent pneumonias.

Etiology/Pathophysiology:
Congenital hamartomatous lesion of the lung.

Pathology:
There are 3 subtypes, all of which lack normal bronchial communications:

Type I - multiple large air or fluid filled cysts, usually greater than 2.0 cm in diameter icon gif.

Type II - variably sized less bulky lesion with smaller cysts icon gif.

Type III - bulky mass composed of multiple tiny, microscopic cysts resembling bronchi that involves the entire lobe icon gif. Adenomatous hyperplasia with an increase in terminal bronchiolar structures, as well as a polypoid arrangement of mucosal epithelium is seen.

Imaging Findings:
Seen with equal frequency in any lobe. Can rarely be bilateral icon gif.

Type I - multiple large air or fluid filled cysts that produces a mediastinal shift and compression of adjacent lung. icon gificon gif The most common type is Type I. It may have air fluid levels in its cysts. If infected, it may appear homogeneously opacified.

Type II - variable sized less bulky lesion with smaller cysts with less mediastinal shift and respiratory distress.

Type III - bulky radiographically solid mass composed of multiple tiny cysts that involves the entire lobe.

DDX:

References:

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