Congenital cystic adenomatoid malformation diagnosed during adolescence

Congenital cystic adenomatoid malformation (CCAM) of lungs is a rare congenital lesion that is associated with abnormal proliferation of terminal bronchi and has an incidence of 1/4000 to 1/35000. Most of the cases (90%) are seen in the first two years of life and it is rarely seen in adolescents and the older age groups. Respiratory distress related to pulmonary compression and lung hypoplasia occur during the neonatal period, whereas recurrent and persistant pneumonia may occur at the older ages. The lesion is classified into five types by Stocker et al. Herein, we present a fourteen-year-old case who had no symptoms previously and diagnosed as type 2 cystic adenomatoid malformation with video-assisted thoracic surgery (VATS) due to persistent and treatment-resistant pneumonia

Adolesan yaþta taný alan konjenital kistik adenomatoid malformasyon

Akciðerlerin konjenital kistik adenomatoid malformasyonu (KKAM) terminal bronþiollerin anormal proliferasyonu sonucu geliþen nadir bir geliþimsel anomali olup insidansý 1/4000 ila 1/35000’dir. Olgularýn büyük çoðunluðu (%90) hayatýn ilk iki yýlý içinde tespit edilmekte ve ileri yaþlarda, özellikle adolesanlarda çok nadir görülmektedir. Yenidoðan döneminde pulmoner kompresyon ve hipoplaziye baðlý solunum sýkýntýsý geliþirken daha büyük yaþtaki olgularda rekürren ve persistan pnömoniler görülebilir. Lezyon Stocker ve arkadaþlarý tarafýndan 5 tipe ayrýlmýþtýr. Burada daha önce hiçbir yakýnmasý olmayan, tedaviye yanýtsýz persistan pnömonisi nedeniyle video yardýmlý torasik cerrahi (VATS) yapýlarak tip II kistik adenomatoid malformasyon tanýsý alan 14 yaþýnda bir olgu sunulmuþtur.