CPAM type 0
Thursday 5 April 2012
CCAM type 0 (congenital acinar dysplasia, congenital acinar aplasia)
CCAM type 0 (congenital acinar dysplasia, congenital acinar aplasia) (1-3%) (neonates, other malformations, poor prognosis)
small and firm lungs
bronchial-type airways with cartilage, smooth muscle and glands separated by abundant mesenchymal tissue
CPAM, type 0, also known as "acinar dysplasia" or "acinar agenesis", is a rarely occurring, infrequently described malformation that is largely incompatible with life.
CPAM, type 0 is seen in term and premature infants who are cyanotic at birth and sur-vive only a few hours and is associated with cardiovascular abnormalities and dermal hypoplasia.
Grossly, the lungs are small and ﬁrm and have a diffusely granular surface.
Microscopically, tissue consists of bronchus-like structures with muscle, glands, and numerous cartilage plates.
Prominent mesenchymal tissue separates these structures and contains extramedullary hematopoiesis, large thin-walled vascular channels, and collections of amor-phic basophilic debris.
Rarely, structures resembling proximal bronchioles are present, along with a few scattered acini at the periphery of the lesion.
CPAM, Congenital Pulmonary Airway Malformation (CPAM)