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Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 859-860
Congenital cystic adenomatoid malformation: A case report

Department of Radiodiagnosis & Imaging, Gandhi Medical College & Associated Hamidia Hospital, Bhopal, M.P, India

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Date of Submission28-Jun-2006
Date of Acceptance10-Oct-2006

Keywords: Congenital Cystic Adenomatoid Malformation (CCAM)

How to cite this article:
Malik R, Pandya V K, Agrawal G, Jain M. Congenital cystic adenomatoid malformation: A case report. Indian J Radiol Imaging 2006;16:859-60

How to cite this URL:
Malik R, Pandya V K, Agrawal G, Jain M. Congenital cystic adenomatoid malformation: A case report. Indian J Radiol Imaging [serial online] 2006 [cited 2020 Dec 1];16:859-60. Available from:

   Introduction Top

Congenital cystic adenomatoid malformation (CCAM) is a rare pulmonary anomaly. It is a hamartomatous lesion characterized by a cessation of normal bronchiolar maturation, resulting in cystic overgrowth of the terminal bronchioles. We report one such case of Type 1 CCAM.

   Case repoprt Top

3 year male child was referred for x-ray chest with 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)

   Discussion Top

CCAM is unusual condition characterized by immature, malformed lung tissue with cystic appearance, which results from an abnormality of branching morphogenesis of the lungs. An adenomatous overgrowth of terminal bronchioles and alveoli leads ultimately to large masses, which are communicating with the tracheobronchial tree, and have feeding vascularisation from the pulmonary (bronchial) circulation [1]. In 1977 Stocker et al. originally described the findings and classified CCAM [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 as
" Type I - This is the most common type and composed of variable cysts with at least one dominant cyst greater 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.

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 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].

   References Top

1.O. C. Ioachimescu and A. C. Mehta, From cystic pulmonary airway malformation, to bronchioloalveolar carcinoma and adenocarcinoma of the lung. Eur Respir J 2005; 26:1181-1187.  Back to cited text no. 1    
2.Stocker JT, Madewell JE, Drake RM, Congenital cystic adenomatoid malformation of the lung, Classification and morphologic spectrum, Hum Pathol 1977; Mar; 8(2): 155-171  Back to cited text no. 2    
3.D Boon, T Llewellyn and P Rushton, A strange case of a tension pneumothorax.. Emerg Med J 2002; 19:470-471.  Back to cited text no. 3    
4.Catherine M. Owens and Karen E.Thomas. The Paediatric Chest. In: David sutton, Textbook of Radiology and Imaging, 7th ed. London: Churchill Livingstone, 2003: 247-264.   Back to cited text no. 4    
5.Adzick NS, Harrison MR, Glick PL, Golbus MS, Anderson RL, Mahony BS, et al Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history..J Pediatr Surg 1985 Oct; 20(5): 483-488   Back to cited text no. 5    
6.Conran RM, Stocker JT, Extralobar sequestration with frequently associated congenital cystic adenomatoid malformation, type 2: report of 50 cases. Pediatr Dev Pathol 1999 Sep-Oct; 2(5): 454-63   Back to cited text no. 6    
7.Cystic Adenomatoid Malformation Inc. , Date last updated: November 15, 2002; Date assessed: May 12, 2006.  Back to cited text no. 7    

Correspondence Address:
R Malik
Department of Radiodiagnosis & Imaging, Gandhi Medical College & Associated Hamidia Hospital, Bhopal, M.P
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-3026.32368

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  [Figure - 1], [Figure - 2], [Figure - 3]

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