A rare disease; congenital pulmonary airway malformation in an adult

Abstract

Congenital pulmonary airway malformation (CPAM), is a rare disease known as a developmental abnormality of the lower respiratory tract. It may occur in the neonatal period due to respiratory distress and patients usually die in the first few months of life. Rarely, it may remain asymptomatic until adulthood. In this study we describe an adult case and present a review of the literature. A 19-year-old male with no relevant medical history was admitted to our clinic with cough and wheeze. The patient had a history of frequent lower respiratory tract infection during childhood. Chest radiograph revealed a diffuse opacity and volume loss in the right hemithorax. High resolution tomography showed hypoplasia of the right hemithorax, multiple cysts in all of the lobes and segments on the right side, ground glass opacity and interlobular septal thickening of the whole right lung parenchyma. Right pneumonectomy was performed with the pre-diagnosis of congenital pulmonary airway malformation and the pathological examination was compatible with CPAM. CPAM is a rare disease in adulthood. We should consider CPAM in the differential diagnosis of patients with frequent recurrent pulmonary infection and cystic lung lesions. In order to prevent infections and to eliminate the risk of malignancy, surgical treatment should be applied for definite diagnosis and treatment.