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Current Pediatric Research

Congenital cystic adenomatoid malformation lung - A case report

Author(s): Mangla Sood and Dr. Seema Sharm

Vol. 15, No. 1 (2011-01 - 2011-06)

Mangla Sood and Dr. Seema Sharma

Department of Pediatrics, Indra Gandhi Medicl College, Shimla, India
Department of Pediatrics, RPMC, Tanda, India


Congenital cystic adenomatoid malformations (CCAM) of the lung are rare congenital cys-tic lung lesions that arise from excessive disorganized proliferation of tubular bronchial structures. The prenatal rate of detecting lung cysts at the routine 1820-week scan is almost 100%.However as gestation progresses the tracheobronchial tree becomes patent and the fluid within the cysts exits into the amniotic fluid and the cysts collapse, allowing the other lobes of the lung to develop normally. Only at birth do the cysts then expand and present in the newborn period with respiratory distress. In late childhood or in adult life, it can present as recurrent chest infections or even undergo malignant transformation. We report a case of Type II CCAM in newborn with brief review of literature.

Keywords: Congenital cystic adenomatoid malformations lung, congenital, disorganized proliferation, tubular bronchial structures, respiratory distress, malignant transformation
Accepted October 27 2010

Case Report

Full term male baby with birth weight 3.0kg was born to a 24 years primigravida by normal vaginal delivery at hos-pital. Antenatal period was supervised and uncompli-cated. The only USG done at second trimester was re-ported as normal. Baby cried soon after birth but devel-oped respiratory distress at 6 hours of life for which he was shifted to nursery. First examination revealed normal vitals except for RR 74/min. Baby was maintaining satu-ration above 89% on room air. Systemic examination was normal. In chest, air entry was decreased on right side with few crepts. Chest X-ray was done which showed opacification of right lung. Though there was no history to suggest sepsis or meconium aspiration, sepsis screen was sent and baby was started on intravenous antibiotics. Sepsis screen was negative, blood culture sterile and anti-biotics were stopped after 7 days. Baby continued to have tachypnoea. ECHO was normal and ruled out congenital cardiac disease. Repeat chest x-ray (Fig 1) on day 9 showed mass containing air-filled cysts with mediastinal shift and herniation of right lung to opposite side. CT-chest (Fig 2) done on day 14 of life revealed multilocular cystic lesions with thin walls surrounded by normal lung parenchyma in right lung with herniation of lung towards left side. Baby underwent successful surgery on day 16 of life, remained on ventilator for 4 days post-op. He re-mained well for 15 days, but developed pneumothorax on right side after respiratory infection. It was treated suc

Fig 1

Fig: 1 Chest X-ray of the baby

Fig 2

Figure 2. CT chest of the baby

congenital malformation. He is under our follow-up since last seven months and is healthy and well thriving without any lung dysfunction. Histopathological examination of the excised mass showed many normal-sized to dilated alveoli lined by flattened or low-cuboidal epithileum and containing blood and alveolar macrophages in the lumen. There were numerous relatively uniform cysts measuring 0.5-2 cm in diameter resembling bronchioles, lined by stratified co-lumnar epithileum and having a thin fibromuscular wall that showed the focal presence of skeletal muscles sug-gestive of CCAM Type II malformation. affected children leading normal lives with only slight decrease in lung volume.[7]

Table 1. Pathologic Features of Congenital Cystic Adenomatoid Malformation lung (according to Stocker)
Classification Type 0 Type 1 Type 2 Type 3 Type 4
Frequency (%) 1-3 >65 20-25 8 10
Cyst size (maxi-mum) 0.5 cm 0.5-10 cm 0.5-2 cm 0.3-0.5 cm 7 cm
Epithelial lining Ciliated, pseu-dostratified columnar Cuboidal cells, flattened, co-lumnar Ciliated cuboidal, ciliated pseudostrati-fied Ciliated cuboi-dal, columnar Type 1& 2 al-veolar lining cells
Muscular wall thickness (mm) 100-500 100-300 50-100 0-50 25-100
Mucus cells Present in all cases Present (33% of cases) Absent Absent Absent
Cartilage Present in all cases Present (5-10% of cases) Absent Absent Rare
Skeletal muscle Absent Absent Present (5% of cases) Absent Absent


CCAM lung are rare development anomaly with inci-dence of 1:25,000 to 1:35,000 in literature with males and females equally affected.[1] The lesions are benign ha-martomatous or dysplastic lung tumor characterized by overgrowth of terminal bronchioles with reduction in al-veoli.[1] 80% cases present in newborn period with al-most equal frequency in premature and term infants. It is usually unilateral and restricted to a single lobe.[2] In, up to 10% of the cases additional extra-pulmonary abnor-malities can be found, such as renal, central nervous, gas-trointestinal, and cardiac defects.[3] The development of the vertebrate lung has been subdi-vided into five distinct periods based on the anatomical changes that occur in lung architecture: embryonic (3-7 weeks), pseudoglandular (7-17 weeks), canalicular (17-29 weeks), saccular (24-36 weeks), and alveolar (36 weeks to maturity). The CCAM develops during the pseudoglandu-lar and saccular period (7-35weeks). [4]

Stocker, Madewell, and Drake using clinical and patho-logic features based on site of origin of the malformation (e.g., tracheal, bronchial, bronchiolar, bronchiolar/ alveo-lar duct and alveolar/distal acinar) divided CCAM into three subtypes in 19774 and later added two more sub-types in 2002.[5]: (i) type 0 – acinar dysplasia, (ii) type I – multiple large cysts or a single dominate cyst, (iii) type II – multiple evenly spaced cysts, (iv) type III – bulky firm mass, (v) type IV – peripheral cyst type.(Table 1) Stocker’s classification does not accurately describe CCAMs detected antenatally. Adzick et al classifies ante-natally detected cystic lung lesions into two types: macro-cystic with size >5mm (type 1) and microcystic with size <5mm (type 2). [6]

The differential diagnosis includes lobar sequestration, bronchogenic cyst, congenital lobar emphysema, or con-genital diaphragmatic hernia. Antenatally it can lead to fetal hydrops, and maternal po-lyhydramnios, with anticipated mortality 100% with fetal hydrops without lung decompression in utero using resec-tion of lobe or thoracoamniotic shunts.[7] Fifteen to 50% of CCAMs decrease in size significantly before birth.[8] Complete postnatal resolution is rare, and apparent spon-taneous ‘disappearance’’ of antenatally diagnosed lesions should be followed carefully, as nearly half of these cases subsequently require surgery.[9] Ultrasound, CT and MRI are used to identify the location and appearance of the lung abnormality, and determine any changes in thoracic position of other lung lobes, mediastinum, renal (renal agenesis) and gastrointestinal (diaphragmatic hernia and bowel atresia) systems and the cardiac structures. The blood supply and venous drainage is evaluated by Doppler ultrasound. CCAM has both its arterial and venous blood flow from the pulmonary system, whereas in lung sequestration the aorta, rather than the pulmonary artery is the source of blood supply, and there is no communica-tion with the bronchial tree. [10]

Delivery should be conducted at specialised centre. Respi-ratory distress including cyanosis, retractions, and grunt-ing is the most common presentation in the newborn pe-riod due to cysts expanding and compressing their sur-rounding structures leading to pulmonary hypoplasia, me-diastinal shift, spontaneous pneumothorax, or pleural ef-fusion due to hydrops.[9,11]. Symptomatic lesions require urgent radiological evaluation with chest radiography and ideally a CT scan, followed by surgical excision. Later in life morbidity from infection resulting in recurrent pneu-monia, lung abscess, empyema, pneumothorax, or, more rarely, malignancy has been reported.[8,12,13,15] For asymptomatic cases some centre advocate conservative management.[14,16] Surgery is postponed if the patient is asymptomatic but CT chest is done within 1 month post-natally in all cases to delineate the thoracic lesion, dem-onstrate any connection with the tracheobronchial tree, and most importantly evaluate the blood supply so that surgery can be performed in a planned manner due to long term risks. Long term outcome is very good for surgically managed asymptomatic patients,


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  16. Ribet ME, Copin MC, Soots JG, Gosselin BH. Bron-choalveolar carcinoma and congenital cystic adenoma-toid malformation. Ann Thorac Surg 1995; 60: 1126-1128.
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Correspondence to:
Mangla Sood

Pratasha, North Oak Area
Sanjauli, Shimla 171006
Himachal Pradesh, India

Curr 64 Pediatr Res 2011 Volume 15 Issue 1

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