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Congenital cystic diseases of the lung.

Journal of the Korean Pediatric Society 1991;34(12):1671-1677.
Published online December 31, 1991.
Congenital cystic diseases of the lung.
Won Soon Park1, Young Pyo Chang1, Hee Ju Kim1, Young Yull Koh1, Jung Hwan Choi1, Chong Ku Yun1, Joo Hyun Kim2
1Departments of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
2Department of Thoracic Surgery, Seoul National University College of Medicine, Seoul, Korea
선천성 폐 낭종성 질환
박원순1, 장영표1, 김희주1, 고영률1, 최중환1, 윤종구1, 김주현2
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 흉부외과교실
Received: 17 April 1991   • Accepted: 10 July 1991
Congenital cystic diseases of the lung (CCDL) is a clinical group of disorders characterized by a cystic appearance of pulmonary tissue. It can be classified into four distinct categories: 1) Congenital lobar emphysema (CLE), 2) Congenital cystic adenomatoid malformation (CCAM), 3) Pulmonary sequeestration (PS), and ^Bronchogenic cyst (BC). 24 patients with CCDL admitted to the Seoul National University Children’s Hospital from January 1984 to June 1989, were investigated for their pathologic classificataion, clinical characteristics, diagnostic measures and treatment outcome. The results were as follows: 1) 24 cases of CCDL were classified pathologically into four distinct categories: CLE —4 cases, CCAM —5 cases (type 1: 4 casese, type II: 1 cases), PS (intralobar) —10 cases, BC — 5 cases. 2) CCAM and PS were seen more commonly in females, CLE and BC were more common in males. 3) In PS, left lower lobe was the most frequent site affected. 4) In age distribution, most cases of CLE and CCAM were diagnosed during the first 6 postnatal months. PS and BC were generally seen later in life. 5) The most frequent presenting symptoms were respiratory distress in CLE and CCAM, and recurrent respiratory infection in PS and BC. 6) Chest radiographic findings were quite effective in differentiating cystic lesions of the lung from other conditions. 7) Associated anomalies were observed in 6 cases (25%). 8) In the symptomatic patient, prompt surgical treatment was recommended. 9) Surgical treatment was quite effective and safe (92% of survival rate).
Key Words: Congenital cystic diseases of the lung, Congenital lobar emphysema, congenital cystic adenomatoid malformation, Pulmonary sequestration, Bronchogenic cyst

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