Seventeen patients were diagnosed as having congenital cystic adenomatoid malformation of the lung during 1970-88. One case was associated with congenital diaphragmatic hernia and the child died before operation. The other 16 children underwent successful surgery. The patients presented in one of three ways--with neonatal respiratory impairment (12 cases) requiring urgent diagnosis and treatment, with recurrent respiratory tract infections (3), and with pneumothorax (1). Diagnostic problems arose with the neonatal presentation. In four of the children laparotomy was performed for presumed diaphragmatic hernia; three of these children had right sided lesions. Congenital diaphragmatic hernia was excluded by contrast studies in a further two patients. Histological examination confirmed the diagnosis in all cases. The postoperative course was uneventful in all 16 patients. No long term impairment of pulmonary function was noted.
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