Congenital cystic adenomatoid malformatlon (CCAM) of the lung is a rate, potentially
lethal form of congenital pulmonary cystic disease and was introduced in the English
literature by Chin and Tang in 1949.
According to Daroca (1979), there have been approximately 70 cases reported In the
world's literature up to 1979.
Recently we have experienced a case of CCAM from a 2,880 gm female infant, who
was born by a matter of G4P2 with repeated cesarian section at 37 weeks. The infant
appeared normal at birth, but 4 hours later cyanosis was developed.
On physical examination, markedly decreased breath sound at the left chest with coarse
breath sound at the right chest was noted.
Chest roentgenogram revealed a large round cavity filling the left lower hemithorax
with increased radiolucency and displacing the heart and mediastinum to the right. By
varium study, diaphragmatic hernia was excluded.
Because of no improvement in symptoms and findings, left lower olobectomy with open
thoracotomy was performed at 3 days of age under the diagnosis of congenital lung
cyst. No main bronchial or vascular anomalies were seen.
The resected specimen was enlarged, spongic d purplish lung weighing around 50gm.
The lung was largely replaced by a large and many small cystic spaces traversed by
remaining collapsed parenchyme on cut sections.
The largest space measure about 4cm in diameter and the inner surface was not
smooth. The peripheral portion of the specimen were rather atelectatic and seemed to be
uninvolved.
The microscopic finding is characterized by numerous cystic spaces that are lined by
bronchial type epithelium, and gland-like spaces or alveolar spaces that are lined by
simple cuboidal or columnar epithelium with occasional mucus producing cells.
Bronchial seromucinous glands and cartilages are totally lacking. One day after
operation, the patient expired.
CCAM occurs in premature infants or still borns in many instances, but rarely in
new-borns and older children.
Operation should be performed as soon as the diagnosis is established, because it is a
surgically curable condition.
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