Congenital cystic adenomatoid malformation (CCAM).
Pathogenetically, congenital cystic adenomatoid malformation has been attributed to an overgrowth of bronchioles, with almost complete suppression of alveolar development between the 7th and 10th weeks of embryonic life
CCAM is a congenital anomaly of the terminal brochioles which results in a dysplastic, multicystic mass. These cysts usually communicate with the bronchial tree. At birth, these cysts are fluid-filled. Ultimately, communication with the airway allows for the fluid-filled mass to become air-filled. Whether fluid-filled or air-filled, the cysts become increasing larger, exert mass effect, compress mediastinal structures and lung parenchyma.
CCAM consists of a multicystic intralobar mass of disorganized lung tissue, derived primarily from bronchioles. It can involve an entire lobe. Lower lobes are most often involved, but any lobe can be affected. The treatment of choice is excision of the affected lobe.
Infants less than one year old usually present with respiratory distress. Usually, the abnormality is confined to one lobe. There are three types of CCAM:
Type I: Single/multiple cysts > 2cm (most common).
Type II: Smaller cysts <2cm with mixed solid component.
Type III: Solitary solid mass.
Radiological differential diagnosis: Congenital diaphragmatic hernia, congenital lobar emphysema, localized persistent pulmonary interstitial emphysema and pneumatocele.
Treatment: Surgical lobectomy is curative with Type I. There is increased morbidity and mortality with Type II and II because the lesions are often larger at presentation.
http://www.radpod.org/2008/02/28/congenital-cystic-adenomatoid-malformation/
http://www.uhrad.com/pedsarc/peds069.htm
http://radiographics.rsnajnls.org/cgi/content/full/24/1/e17
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