Download Congenital cystic adenomatoid malformation type I

Survey
yes no Was this document useful for you?
   Thank you for your participation!

* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project

page
1
page
2
page
3
page
4
page
5
page
6
page
7
page
8
page
9
page
10
page
11
Document related concepts
no text concepts found
Transcript 
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.
I
II
III
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.
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
Congenital cystic adenomatoid malformation: CT features
Most lesions confined to a single lobe…. but can be multilobar
Or affect one hemithorax….
Related documents