All issues > Volume 34(12); 1991

Original Article

J Korean Pediatr Soc. 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

Received: April 17, 1991;  Accepted: July 10, 1991.

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: 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).

Keywords :Congenital cystic diseases of the lung;Congenital lobar emphysema;congenital cystic adenomatoid malformation;Pulmonary sequestration;Bronchogenic cyst