A case of congenital cystic adenomatoid malformation of the lung

ABSTRACT

The congenital cystic adenomatoid malformation (CCMA) of the lung is a rare variant of congenital cystic lung disease consisted of one or usually multiple interconnecting cysts in the pulmonary parenchyma lined by cuboidal and columnar epithelium. This diease present clinically in three ways 1) stillborn or perinatal death, 2)progress respiratory distress in the newborn, and 3)acute and chronic pulmonary infections in the older infant and child. The onset of symptoms, which are cyanosis, tachypnea, and other forms of respiratory distress, usually occurs at or shortly after birth, This manifestations are related to compression of the remained normal ung by expansion of the cysts. We have experienced a case of congenital cystic adenomatoid malformation of the lung in a 1-day-old male infant who had tachypnea. A right upper lobectomy was done with satisfactory postoperative courses clinically and radiologically at 8 months of age. A brief review of the related literature is presented.