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2016, Volume 32, Number 3, Page(s) 200-204     
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DOI: 10.5146/tjpath.2013.01208
Congenital Pulmonary Airway Malformation Type 2: A Case Report with Review of the Literature
Ebru TAŞTEKİN1, Ufuk USTA1, Ayşegül KAYNAR2, Çiğdem ÖZDEMİR3, Ömer YALÇIN1, Filiz ÖZYILMAZ1, Ali Kemal KUTLU1
1Department of Pathology, Trakya University, Faculty of Medicine, EDİRNE, TURKEY
2Iskenderun State Hospital, HATAY, TURKEY
3Tekirdag State Hospital, TEKİRDAĞ, TURKEY
Keywords: Cystic adenomatoid malformation of lung, Congenital, Lung diseases, Hydrops fetalis

A congenital pulmonary airway malformation is a rare disorder of the pulmonary airway and a hamartomatous mass of disorganized lung tissues with various degrees of cystic change. A 20-year-old pregnant woman who did not have previous clinical follow-up during her pregnancy visited the gynecology department for her first check on the 19th week of gestation. The sonogram, showed severe hydrops fetalis. Laboratory findings were consistent with non-immune hydrops fetalis. Medical abortion was performed and the fetus was sent to our department for a complete fetal autopsy. Macroscopically, whole parts of the fetus had striking oedema. Massive pleural and peritoneal effusions were seen on dissection. The left lung filled the whole thoracic cavity. The heart was displaced to the right and the right lung was compressed. Microscopically, the left lung mass showed dilated bronchiole-like structures (1-20 mm) that were lined with ciliated columnar cells without any intervening mucinous cells. The subepithelial stroma contained thin, interrupted smooth muscle fibers and elastic connective tissue without cartilage plates. Our case is a very good example of non-immune hydrops fetalis associated with congenital pulmonary airway malformation type 2. Prenatal clinical and ultrasonographic follow-ups during pregnancy are very important for early diagnosis of congenital malformations.

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