Congenital Cystic Adenomatoid Malformation of the Lung: Analysis of 17 Cases / 소아알레르기및호흡기학회지

ABSTRACT

PURPOSE: Congenital cystic adenomatoid malformation of the lung(CCAM) is characterized by an excessive overgrowth of terminal bronchiolar-type structures with a lack of mature alveoli. This study was performed to evaluate the manifestation among different pathologic classification. METHODS: Seventeen patients admitted to the Samsung Medical Center from January 1995 to June 2000, were investigated for their clinical characteristics, radiologic findings, timing of treatment and post-operative outcome in each type. RESULTS: 1) Seven patients have type 1, while 9 cases were type 2. One case was diagnosed as having mixed type. Mean ages at diagnosis are 7 years 4 months. 2) 4 cases had experienced respiratory diseases that are pneumonia(3 cases), empyema(2 cases) and lung abscess(2 cases). 3) The most common clinical manifestations are fever and cough, followed by chest pain, dyspnea, cyanosis, weak crying and moaning. 4) The left lung(10 cases), especially left lower lobe(8 cases) is involved more frequently than right lung. 5) Associated anomalies are pulmonary sequestration, pectus excavatum, diaphragmatic hernia. 6) All lesions were surgically removed and all of patients survived. In our cases, there was no difference in outcome by age at operation. CONCLUSION: In this study, CCAM show various clinical manifestations, and the outcome is good irrespective of their pathologic type and age at operation. Probably this is due to few associated anomalies in our cases, but it is not clear whether this is common finding in Korea. Our findings suggest that CCAM is treated successfully by surgical removal which can be delayed unless respiratory symptoms are evident.