Article:

Late onset congenital cystic adenomatoid pulmonary malformation

Abstract: Congenital cystic adenomatoid pulmonary malformations are defined as the presence of a cystic mass of undifferentiated lung tissue; a third of the cases affect one of the lower lobes, and multilobar or bilateral affection is rare. The entity is a rare congenital malformation in the lungs, with an incidence of one in 11,000 to one in 35,000 live births. It is mainly discovered during the fetal period, although a low percentage of patients will be diagnosed postnatally. The disease has a wide spectrum of clinical manifestations, from asymptomatic to life-threatening respiratory symptoms. The diagnosis is made by imaging studies, and the treatment depends on the patient’s clinical status. We reported the case of an infant with a rare congenital pulmonary airway malformation and a late diagnosis, which makes the case even rarer, who required open lobectomy as treatment.