Konjenital Kistik Akciğer Hastalıkları

Yazarlar

Şamil Amirjanov

Özet

Konjenital kistik akciğer hastalıkları (KKAH), bronkopulmoner sistemin embriyolojik gelişimindeki bozukluklar sonucu ortaya çıkan ve trakeobronşiyal ağacın gelişimindeki zamanlama ile lokasyon farklarına göre farklı histolojik özellikler gösteren lezyonlardır. Akciğerlerin gelişimi embriyonik, psödoglandüler, kanaliküler, sakküler, alveoler ve mikrovasküler olmak üzere altı aşamada gerçekleşir. Bu süreçlerdeki gelişimsel kusurlar konjenital pulmoner hava yolu malformasyonu (KPHM), intralober veya ekstralober bronkopulmoner sekestrasyon (BPS), bronş atrezisi (BA) ve konjenital lober amfizem gibi farklı malformasyonlara neden olur. Tanı ve klasifikasyonda Langston ile yeni Japonya sınıflamaları esas alınmakta; lezyonların hava yolu ile ilişkisi, histolojik yapıları ve kanlanma kaynakları (pulmoner dolaşım veya aorttan besleyen aberan sistemik arter) ayırıcı tanıda belirleyici rol oynamaktadır. Prenatal dönemde ultrasonografi ve manyetik rezonans görüntüleme kullanılarak lezyon çapı, KHO (KPHM hacim oranı) ve hidrops fetalis gelişimi incelenerek fetal prognoz ve yönetim planlanır. Postnatal dönem patofizyolojisinde persistan pulmoner hipertansiyon, solunum yetmezliği ve hava hapsi riski öne çıkmaktadır. Hastalıkların temel tedavisi cerrahi rezeksiyon olup acil cerrahi gerektiren semptomatik yenidoğanların yanı sıra, asemptomatik hastalarda tekrarlayan akciğer enfeksiyonları ve malignite transformasyon riskini önlemek amacıyla genellikle 3-12 ay arasında elektif lobektomi veya torakoskopik rezeksiyon önerilmektedir.

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