Unilateral Multicystic Pulmonary Disease in a Young Adult: An Unusual Diagnosis

Abstract

Congenital pulmonary airway malformation (CPAM) is an uncommon developmental hamartomatous anomaly caused by abnormal branching of immature bronchioles with replacement of the lung by cysts/disorganized tissue. CPAMs are usually lobar or sublobar in extent, and ∼90% of lesions are identified within the first 2 years of life. CPAM occupying an entire lung, with a delayed adulthood presentation, is extremely rare; only 4 such occurrences have been previously described. We present a case of a 20-year-old woman diagnosed with unilateral entire lung CPAM on the basis of radiologic findings that was surgically resected via video-assisted thoracoscopic surgery.