Congenital pulmonary airway malformation

Dr Alexandra Stanislavsky and Dr Frank Gaillard et al.

A congenital pulmonary airways malformation (CPAM) is a multi-cystic mass of
segmental lung tissue with abnormal bronchial proliferation

. !"A#s are considered part of the

spectrum of bronchopulmonary foregut malformations and until recently were described as
a congenital cystic adenomatoid malformation (CCAM).
Epidemiology
$hey account for approximately %& of congenital lung lesions. $he estimated incidence is
approximately '('%))-*))) live births and there is a male predominance.
Clinical presentation
$he diagnosis is usually either made on antenatal ultrasound+ or in the neonatal period on
investigation of progressive respiratory distress
,-*
. -f large+ they may cause pulmonary
hypoplasia+ with resultant poor prognosis.
-n cases where the abnormality is small+ the diagnosis may not be made for many years or even
until adulthood. .hen it does become apparent it is usually as a result of recurrent chest
infection
,-*
.
Pathophysiology
$he condition results from failure of normal broncho-alveolar development with hamartomatous
proliferation of terminal respiratory units in a gland-like pattern /adenomatoid0 without proper
alveolar formation.
1istologically they are characterised by adenomatoid proliferation of bronchiole-like structures
and macro- or microcysts lined by columnar or cuboidal epithelium and absence of cartilage and
bronchial glands.
$hese lesions have intracystic communications and+ unlike bronchogenic cysts+ can also have a
connection to the tracheobronchial tree.
Sub types
At least four sub types are currently classified mainly according to cyst si2e.
type I
o most common( 3)& of cases
,

o large cysts
o one or more dominant cysts( -') cm in si2e
o may be surrounded by smaller cysts
type II
o '%-)& of cases
,

o cysts are less than cm in diameter
o associated with other abnormalities
renal agenesis or dysgenesis
pulmonary se4uestration
congenital cardiac anomalies
type III
o 5')& of cases
o microcysts( 6 % mm in diameter
o typically involves an entire lobe
o has a poorer prognosis
type IV
o unlined cyst
o typically affects a single lobe
ocation
7esions are usually unilateral and involve a single lobe. Although there isn8t a well documented
lobar predilection+ they appear less fre4uently in the right middle lobe
,
.
Associations
hybrid lesion( e.g. !"A# 9 pulmonary se4uestration
renal agenesis
3

!adiographic features
$he appearance of !"A#s will vary depending on the type.
Antenatal ultrasound
$hese lesions appear as an isolated cystic or solid intrathoracic mass. A solid thoracic mass is
usually indicative of a type --- !"A# and is typically hyperechoic. $here can be mass effect
where the heart may appear displaced to the opposite side. 1ydrops fetalis or polyhydramnios
may develop
,
and may be detected on ultrasound as ancillary sonographic features. Alternatively
the lesion may remain stable in si2e+ or even regress
%
.
Plain film
!hest radiographs in type - and -- !"A#8s may demonstrate a multicystic /air-filled0 lesion.
7arge lesions may cause mass effect with resultant+ mediastinal shift+ and depression and even
inversion of the diaphragm. -n the early neonatal period the cysts may be completely or partially
fluid filled+ in which case the lesion may appear solid or with air fluid levels. 7esions may
change in si2e on interval imaging /expand from collateral ventilation via pores of :ohn0. $ype
--- lesions appear solid.
C"
!$ has a number of roles in the management of !"A#s. Firstly it more accurately delineates the
location and extent of the lesion. Secondly+ and most important in surgical candidates+ !$
angiography is able to identify systemic arterial supply if present.
Appearances once more reflect the underlying type where a type --- lesion can appear as a
consolidative area.
Complications
"otential post natal complications include
recurrent pneumothorax
haemopneumothorax
pyopneumothorax
possible incidence of certain malignancies+ which include including
,
(
o bronchoalveolar carcinoma
o bronchogenic carcinoma
o pleuropulmonary blastoma
o rhabdomyosarcoma
"otenetial in utero complications include
development of hydrops fetalis may rarely result when there is severe compression of the
fetal heart or great vessels
a significant compression of the normal fetal lung can also rarely cause pulmonary
hypoplasia

"reatment and prognosis

$here can be wide spectrum in prognosis.
Surgery /elective lobectomy0 is the treatment of choice in symptomatic patients+ both in those
presenting early with respiratory compromise and in those presenting later with recurrent
infections
,
. $ype - lesions generally have the best prognosis.
-n the setting of a small stable asymptomatic lesion+ surgical excision is more controversial.
Advocates for excision 4uote the reported risk of developing malignancies within the lesion /see
above0. An alternative approach is to watch and wait. $here are reports of spontaneous
regression particularly in those serially followed up on antenatal ultrasound
3+')
.
#ifferential diagnosis
General imaging differential considerations include
bronchogenic cyst
pulmonary se4uestration
congenital diaphragmatic herniation
congenital lobar emphysema
localised congenital cystic bronchiectasis
For type I lesions on !$ also consider
cicatri2ation collapse with scarring and traction bronchiectasis
CPAM tipe I
CPAM tipe I
Congenital diaphragmatic herniation Pulmonary sequestration Cicatrization
collapse