Pediatr Allergy Respir Dis.
2008 Jun;18(2):129-137.
Clinical Features of Congenital Cystic Adenomatoid Malformation of the Lung
- Affiliations
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- 1Department of Pediatrics, College of Medicine, Pusan National University, Busan, Korea. phj7294@hanmail.net
Abstract
- PURPOSE
Congenital cystic adenomatoid malformation (CCAM) is a rare variant of congenital pulmonary cystic disease, which is characterized by a proliferation of the dilated bronchiolar-like structure of varying size and/or distribution. In this study, we described the clinical manifestations of CCAM in a single institution during a 12-year period.
METHODS
Twenty patients diagnosed with CCAM who were admitted at Busan National University Hospital between January 1995 and December 2007, were investigated for their presenting characteristics, age at diagnosis, locations, histopathologic types, combined anomalies, treatments and outcomes.
RESULTS
Twenty patients diagnosed with CCAM were reviewed. Twelve males and eight females, the mean age of the patients was 7.5 years. The main symptoms were coughing, sputum, recurrent infection, chest pain, cyanosis and tachypnea. Seven patients were antenatally diagnosed and two patients were incidentally diagnosed. Radiologic findings were multiple cysts in 15 cases, solitary cyst in 2, consolidation in 3, pleural effusion in 4, tracheal deviation in 2, and abscess in 1 case. The most common location of cystic lesion was the right lower lobe, which includes 9 cases (45%). According to Stocker's histologic classification of CCAM, type I was found in 11 cases (73%), type II in 2 cases and type III in 1 case. In 5 cases, there were combined anomalies including ipsilateral pulmonary sequestration, dextrocardia, pectus excavatum, patent ductus arteriosus, accessory bronchus, and bronchogenic cyst. Fifteen patients underwent lobectomy, two patients are going to have operation and other 3 patients were transferred from regional hospital after diagnosis. All patients who underwent surgical resection improved.
CONCLUSION
CCAM is a rare but potentially life-threatening pulmonary anomaly, which can be found at different ages and presents with various clinical findings. The possibility of an underlying CCAM should be considered in children with recurrent pulmonary infections or persistent abnormalities on chest X-rays following an acute infection.