CONGENITAL LUNG MALFORMATIONS

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CONGENITAL LUNG MALFORMATIONS Congenital cystic adenomatoid malformation (CCAM)

Congenital cystic adenomatoid malformation (CCAM) 25% of all congenital lung malformations 90% of congenital cystic lesions Anomalous fetal development of terminal respiratory structures, that results in a multicystic, displastic mass that interferes in the normal alveolar development. Arterial supply is from the pulmonary artery (not sistemic like sequestration). CCAAM can be divided in mostly three types according Stocker classification, but a fourth type has been described.

Congenital cystic adenomatoid malformation (CCAM) Clinical notes CCAM associates to several malformations CCAM type II: renal agenesia/displasia diaphragmatic hernia cardiovascular CCAM type III: polyhidramnios, hydrops CCAM type IV: respiratory distess, tension neumothorax

Congenital cystic adenomatoid malformation (CCAM) Stocker classification (1977) of CCAM based on size of the cysts at imaging & pathology CCAM TYPE I (50%): 1 or more large (2-10 cm) cysts CCAM TYPE II (40%): numerous small cysts of uniform size CCAM TYPE III (<10%): solid on macroscopic examination but have mycrocysts CCAM type 4: large cysts, periferal distribution CCAM type 0letal, panlobular. II III I

Congenital cystic adenomatoid malformation: type I Multicystic mass with air filled 1 or more large (2-10 cm) cysts Most common type (50%) Mediastinal shift (mass effect) Normal abdominal air distribution

Congenital cystic adenomatoid malformation type I 1 or more large (2-10 cm) cysts Most common type (50%)

Congenital cystic adenomatoid malformation type II Numerous small cysts of uniform size Mediastinal shift Normal distribution of abdominal air

Congenital cystic adenomatoid malformation type III Solid mass appearance in the upper left lobe, but have microcysts.

Congenital cystic adenomatoid malformation: CT features Cysts of variable size, contain air or fluid No evidence of systemic arterial supply Mass effect (mediastinal shift or adjacent lung compression) Usually unilateral, lower lobes, solitary lesions MAQ. Suelen ser solitarias, unilaterales, bases pulmonares... Congenital cystic adenomatoid malformation: CT features

Most lesions confined to a single lobe…. but can be multilobar

Or affect one hemithorax….