선천성 폐 낭종성 질환의 임상적 고찰

Abstract

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:Congenital lobar emphysema(CLE), Congenital cystic adenomatoid malformation(CCAM), Pulmonary sequestration(PS), and Bronchogenic cyst(BC). 17 patients with congenital cystic diseases of the lung admitted to the Department of Pediatirics, Keimyung University, Dongsan Hospital from January, 1986 to December, 1995 were investigated for their pathologic classification, clinical characteristics, diagnostic measures and treatment outcome. The results were as follows: 1) 17 cases of CCDL were classified pathologically into four distinct categories: BC 8 cases, PS 5 cases(intralobar 4 cases, extralobar 1 case), CCAM 4 cases. 2) CCDL were seen more commonly in males. 3) In age distribution, 64.7% of CCDL were diagnosed at over 6 years. 4) In BC and PS, right lower lobe was the most frequent site affected. 5) The most frequent presenting symptoms were respiratory distress and recurrent respiratory infection. 6) Chest radiographic findings were quite effective in differentiating cystic lesions of the lung from other conditions. 7) Associated anomalies were obserbed in 4 cases (23.5%). 8) In the symptomatic patient, prompt surgical treatment was recommended. 9) Surgical treatment was quite effective and safe.