Akciğerin Konjenital Malformasyonları
Özet
Konjenital akciğer malformasyonları, asemptomatik seyirden doğum sonrası ileri derecede solunum problemlerine veya ölü doğuma kadar değişken klinik tablolara yol açabilen, nadir görülen anomalilerdir. Bu lezyonlar kistik adenomatoid malformasyon, pulmoner sekestrasyon, konjenital lober amfizem, bronkojenik kist ve bronşiyal atrezi gibi yapıları kapsar. Prenatal ultrasonografi ve fetal MRG teknolojilerindeki gelişmeler sayesinde anomaliler genellikle gebeliğin 18-24. haftalarında intrauterin olarak saptanabilmektedir. Çoğu malformasyon, akciğer gelişiminin özellikle psödoglandüler evresindeki normal işleyişin bozulmasından kaynaklanır. Hidrops fetalis veya kalp yetmezliği gelişen kötü prognozlu olgularda torakoamniyotik şant veya açık fetal cerrahi gibi prenatal girişimler %50’nin üzerinde sağkalım sağlamaktadır. Doğum sonrası dönemde semptomatik lezyonlar için temel tanı yöntemi bilgisayarlı tomografidir. Klinik olarak enfeksiyon, apse ve malignite odağı oluşturabilen bu anomalilerin tedavisinde çoğunlukla lobektomi veya segmentektomi gibi cerrahi rezeksiyon yöntemleri uygulanır. Son yıllarda açık cerrahinin yanı sıra video yardımlı göğüs cerrahisi (VATS) kullanımı da yaygınlaşmıştır.
Congenital lung malformations are rare anomalies that cause a broad spectrum of clinical symptoms, ranging from asymptomatic courses to severe neonatal respiratory distress or stillbirth. These disorders include congenital cystic adenomatoid malformation, pulmonary sequestration, congenital lobar emphysema, bronchogenic cyst, and bronchial atresia. Due to advancements in prenatal ultrasonography and fetal MRI, these lesions are mostly detected intrauterinely between the 18th and 24th weeks of gestation. Many malformations originate from disruptions during the pseudoglandular phase of embryonic lung development. In severe cases with poor prognosis, such as those developing hydrops fetalis or heart failure, prenatal interventions like thoracoamniotic shunting or open fetal surgery provide survival rates exceeding 50%. Postnatally, computed tomography is the gold standard for radiological evaluation. Since these lesions can lead to recurrent pneumonia, lung abscesses, or malignant transformation, surgical resection via lobectomy or segmentectomy is generally required. In recent years, video-assisted thoracic surgery (VATS) has increasingly become a preferred, safer alternative to open thoracotomy.
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