Clinical spectrum of congenital cystic disease of the lung in children

Objectives: Congenital cystic lesions of the lung are uncommon but share si milar embryologic and clinical characteristics. The purpose of this study i s to review our institutional experience of congenital cystic lung disease, emphasizing the clinical spectrum of the disease related to age, and prese nt some cases with unusual clinical manifestations. Patients: Between 1962 and 1996, 26 patients (9 females and 17 males) under 15 years old underwent evaluation and surgical treatment for congenital cystic lung disease. Seve n patients were under 1 year old, and 19 were in over 1 year old. There wer e 13 bronchogenic pulmonary cysts, 6 pulmonary sequestrations, 4 congenital cystic adenomatoid malformations (CCAM), and 3 congenital lobar ennphysema s. Results: All patients under I year old showed respiratory distress with mediastinal shift but no episodes of infection. In contrast, 13 of the 19 p atients over 1 year old had symptoms of recurrent infection without respira tory distress. Five patients over 1 year old were entirely asymtomatic from birth. There were significant differences (P < 0.05) in the frequencies of respiratory distress and infection between the two groups (chi(2)-test). L obectomy was performed in 21 patients, excision in 3 patients, segmentectom y in one patient, and exploration in one patient. There was no incident of postoperative mortality or morbidity except for one patient with CCAM compl icated by reexpansion lung edema. Twenty-one patients at long-term follow-u p from 2 to 30 years after surgery are doing well with no subsequent limita tion of physical activities due to lung resection. Conclusions: In patients under 1 year old, cystic lesions were discovered by respiratory distress; and in patients over 1 year old signs of infection were the most important clinical features. Early recognition of these relatively rare congenital cy stic lung lesions would lead to the immediate, proper surgical intervention . (C) 1999 Elsevier Science B.V. All rights reserved.