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.

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.

목 적:본 연구는 선천성 낭성 선종양 폐기형으로 진단된 환자들의 임상 양상, 방사선 소견, 호발부위, 조직학적 분류, 동반기형, 치료 및 경과를 알아보고자 시행되었다. 방 법:1995년 1월부터 2007년 12월까지 12년간 부산대학교병원에서 선천성 낭성 선종양 폐기형으로 진단된 20례를 대상으로 병력지 검토를 통해 후향적으로 조사하였다. 결 과:총 20례 중 남자 12례, 여자 8례로 산전 초음파 검사에서 발견된 7례를 제외한 나머지 환자의 진단시 평균 연령은 7.5세였다. 진단 및 임상 양상은 산전 초음파 검사에서 발견된 7례(35%), 기침, 가래 등의 호흡기 증상이 10례(50%), 발열이 8례(40%), 그 외에도 흉통 4례(20%), 청색증 2례(10%), 빈호흡과 호흡곤란이 각각 1례씩 있었으며 우연히 단순 X-선 검사에서 발견된 2례가 있었다. 단순 흉부 촬영과 흉부 전산화 단층 촬영을 모든 환자에서 시행하였고 다발성 낭성 병변이 15례(75%)로 가장 많았으며, 단일 낭종 2례(10%), 폐경화 3례(15%), 흉수 4례(20%), 기도 편위 2례(10%), 폐농양 1례가 있었다. 병변의 위치는 우엽에 12례(60%), 좌엽에 8례(40%), 상엽에 6례(30%), 하엽이 14례(70%)로 우하엽에서 9례(45%)로 가장 호발하였다. Stocker의 조직학적 분류에 따른 형태로는 제 I형이 11례(73%)로 가장 많았으며 제 II형이 3례, 제 III형이 1례 있었다. 5례에서 동반 기형이 있었으며, 그 동반 기형으로는 동측 폐격리증, 우심증과 누두흉, 동맥관 개존증, 부기관지, 기관지 원성 낭종 등이 있었다. 수술적 치료로는 15례에서 모두 폐엽 절제술을 시행하였고, 1례가 수술 예정이며, 1례는 선천성 낭성 선종양 폐기형 동반된 결핵성 흉막염의 치료 후 수술을 시행할 예정이다. 산전 진찰에서 진단되어 출생 직후 임상 증상이 없었던 환자의 경우는 대부분 생후 1년 이후에 수술하였으며, 임상 증상이 나타나 진단받은 환자들의 진단 후 수술까지의 평균 기간은 12일이었다. 수술 후 환자들은 모두 생존하여 수술 전 보이던 반복적인 호흡기 감염, 흉수, 흉통, 호흡곤란, 청색증 등의 임상 증상들은 모두 소실되었다.