Bilateral congenital pulmonary airway malformation presenting in adulthood with a review of the literature

Abstract We report a rare case of congenital pulmonary airway malformation (CPAM) in a middle-aged female patient with bilateral involvement. In view of its presentation in adulthood, it presents a rare picture of a disease often diagnosed in early childhood. Until now, 65 cases of this disease have been reported in adults, including 3 cases with bilateral involvement. A classical radiological picture of CPAM can often be confused with bronchiectasis or pulmonary sequestration. A definite treatment with surgical resection poses a challenge in bilateral involvement. We highlight a rare case with its challenges in diagnosis and management. A review of literature of cases of CPAM with bilateral involvement is conducted in an effort to better understand this entity in adulthood.