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Congenital Cystic Adenomatoid Malformation: A Case Report

R MALIK, VK PANDYA, G AGRAWAL, M JAIN

Ind J Radiol Imag 2006 16:4:859-860

Key words : Congenital Cystic Adenomatoid Malformation (CCAM)

INTRODUCTION- DISCUSSION­

Congenital cystic adenomatoid malformation (CCAM) is CCAM is unusual condition characterized by immature,
a rare pulmonary anomaly. It is a hamartomatous lesion malformed lung tissue with cystic appearance, which
characterized by a cessation of normal bronchiolar results from an abnormality of branching morphogenesis
maturation, resulting in cystic overgrowth of the terminal of the lungs. An adenomatous overgrowth of terminal
bronchioles. We report one such case of Type 1 CCAM. bronchioles and alveoli leads ultimately to large masses,
which are communicating with the tracheobronchial tree,
CASE REPOPRT and have feeding vascularisation from the pulmonary
(bronchial) circulation [1]. In 1977 Stocker et al. originally
3 year male child was referred for x-ray chest with described the findings and classified CCAM [2].
complains of recurrent chest infection and respiratory
distress since birth. X-ray chest showed multiple cystic
lucencies in right lower lung field (fig. 1). CT Scan chest
revealed, multilocular, thin walled, variable sized (ranging
from 3mm to 22mm) cystic lesions, surrounded by normal
lung parenchyma (fig. 2). Few cysts showed air fluid level
with contralateral herniation of right lung. Findings were
suggestive of type 1 Congenital Cystic Adenomatoid
Malformation (CCAM). Patient was treated with
lobectomy. Post operative x-ray shows normalization of
herniated lung (fig.3)

Fig 2

CCAM is rare and usually presents before the age of 3

years. The exact incidence is now known. It is more
common in boys than girls, and it is usually unilateral.
Patients can present with life threatening respiratory
distress or a history of recurrent chest infections. Some
cases are asymptomatic and are discovered as an
incidental finding on radiography. Congenital lobar
emphysema can present in a similar way and should be
considered in the differential diagnosis. CCAM is best
diagnosed with computed tomography [3] and classified
Fig 1 as
" Type I - This is the most common type and composed
of variable cysts with at least one dominant cyst greater
From the Department of Radiodiagnosis & Imaging, Gandhi Medical College & Associated Hamidia Hospital, Bhopal (MP)

Request for Reprints: Dr R. Malik, Department of Radiodiagnosis & Imaging, Gandhi Medical College & Associated Hamidia
Hospital, Bhopal (MP)

Received 28 June 2006; Accepted 10 October 2006

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860 R Malik et al
than 2 cm. Prognosis is excellent.
" Type II -Composed of smaller uniform cysts up to 2
cm. This form is commonly associated with anomalies
(especially renal, cardiac, intestinal and skeletal).
" Type III - Least common types and is composed of
microcysts appearing solid upon visual inspection. Poor
prognosis is secondary to respiratory compromise and
associated congenital abnormalities. [4]
Classification based on prenatal ultrasound findings: ­
" Microcystic lesions (< 5 mm): usually associated with
fetal hydrops and has poor prognosis.
" Macrocystic lesions (> 5 mm): not usually associated
with hydrops and has favorable prognosis [5]
Differential Diagnosis includes congenital lobar
emphysema, Bronchogenic cyst, Bronchopulmonary
sequestration, prior infection with pneumatocele formation
and congenital Diaphragmatic hernia.
Fig 3
The coexistence of CCAM in Extra Lobar Sequestration
has been reported. [6]
Chest radiography almost invariably identifies CCAM of
sufficient size to cause clinical problems. The usual
appearance is of a mass containing air-filled cysts. Other
radiological signs that may be evident include mediastinal
shift, pleural and pericardial effusions, and
pneumothoraces; however, the diagnosis may not be clear
from chest radiography alone. Particularly in the case of
type III lesions, chest radiography may demonstrate a
mass without any evidence of cysts. In cases in which
the cystic lesion involutes, chest radiography may not
allow sufficient definition to determine whether the CCAM
has disappeared completely [7]. Computerized chest
tomography reveals the typical appearance of multilocular
cystic lesions with thin walls surrounded by normal lung
parenchyma; however, the presence of superimposed
infection with in the lesion may complicate the
appearance, resulting in air fluid levels. The definition of
high-resolution chest tomography (HRCT) is sufficient to
differentiate between micro cystic and macro cystic
IJRI, 16:4, November 2006
lesions [7]
On antenatal ultrasonography, Type I lesions appear as
multiple large cystic areas in the lung. In type II lesions,
multiple small cysts are evident on ultrasonography.
Because of the extremely small size of the cysts in type
III lesions, the antenatal ultrasonographic appearance often
is one of a homogenous mass. Ultrasonography may
demonstrate evidence of hydrops, such as fetal ascites
or pleural effusion [7].
On Magnetic resonance imaging, the appearance of
CCAM is determined by the size of the lesion as well as
the number and size of the cysts. In contrast to pulmonary
sequestrations, which typically have a homogeneous
appearance, CCAM usually demonstrates some degree
of inhomogeneity because of the multiple cysts. In the
postnatal period, the use of MRI may reveal evidence of
CCAM that, by ultrasonography and chest radiography,
appeared to involutes completely; however, whether or
not any benefit exists in using MRI in the postnatal period
when compared to chest CT scanning is unclear [7].
CCAM is treated with lobectomy. This may have to be
done as an emergency depending on clinical state, and
some cases have been operated on prenatally. Treatment
can be postponed if the patient is asymptomatic and the
cyst is resolving. Long term outcome is very good, affected
children leading normal lives with only slight decrease in
lung volume [3].
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