1. CONGENITAL LUNG MALFORMATIONS
Congenital cystic adenomatoid malformation (CCAM)

2. 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.

3. 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

4. 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

5. 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

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

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

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

9. 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

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

11. Or affect one hemithorax….