Spontaneous pneumothorax: congenital cystic adenomatoid malformation of the lung

Abstract

Background. Congenital cystic adenomatoid malformation (CCAM) is a rare developmental abnormality of the lung with an incidence of 1 in 25,000 to 1 in 35,000 pregnancies and represents 25% of congenital lung malformations and 95% of congenital lung lesions, males and females being equally affected. It is characterized by multicystic mass arising in the lung parenchyma from abnormal proliferation of the immature terminal respiratory bronchioles. The clinical spectrum is wide and ranges from silent incidental lesions to severe congenital respiratory distress or stillbirths. The diagnosis of CCAM can be made in utero by prenatal ultrasongraphy and postnatally by imaging radiography and CT. We report a case of a CCAM, which provoked spontaneous pneumothorax.