Congenital Pulmonary Airway Malformation. Issue 5 (September 2016)

• Record Type: Journal Article
• Authors: Wu, Zhi J.; Narula, Navneet; Beneck, Debra; Nguyen, Andrew B.; Lee, Paul C.
• Is Part Of: Clinical pulmonary medicine. Volume 23:Issue 5(2016)
• Publication Date: 2016-09
• Subjects: congenital pulmonary airway malformation -- congenital lung malformation -- cystic lung lesion; Lungs -- Diseases -- Periodicals; 616.24005
• DOI: 10.1097/CPM.0000000000000168
• Languages: English
• ISSNs: 1068-0640

Abstract

Abstract : Congenital pulmonary airway malformation, previously known as congenital cystic adenomatoid malformation, is a rare congenital defect and is classified into 5 types. The exact mechanism is not well established, but studies have shown that bronchial atresia plays an important role in the pathogenesis. Molecular studies provided additional information about the disease. Most patients with congenital pulmonary airway malformation survive through pregnancy. Some of them present with respiratory distress after birth, with the rest remaining asymptomatic for years. The management of the asymptomatic patients is controversial. We present a review of the epidemiology, the pathophysiology, radiographic findings, treatment modalities, and recently published studies on the subject.