Galen Ven Malformasyonu
Özet
Galen ven malformasyonu (GVM), çocukluk çağı vasküler anomalilerinin %30'unu oluşturan, yüksek debili arteriovenöz şantlara bağlı gelişen nadir ve ciddi bir konjenital damar hastalığıdır. Yenidoğan döneminde kalp yetmezliği ve nörolojik komplikasyonlarla kendini gösteren bu tablo, tedavi edilmediğinde yüksek mortalite riski taşımaktadır. Tanı genellikle ultrasonografi ve MRI ile konulmakta, tedavi stratejisi ise hastanın klinik tablosuna göre medikal destek, endovasküler girişim veya cerrahi yöntemlerle multidisipliner bir yaklaşımla belirlenmektedir. Günümüzde erken teşhis ve gelişen endovasküler teknikler, hastaların mortalite oranlarını düşürerek nörolojik gelişimlerinin korunmasına olanak sağlamaktadır.
Vein of Galen malformation (VGM) is a rare and severe congenital vascular anomaly responsible for approximately 30% of pediatric vascular lesions, characterized by high-flow arteriovenous fistulas. It typically presents in neonates with life-threatening heart failure or neurological complications, necessitating prompt intervention to prevent high mortality rates. Diagnosis is primarily achieved through ultrasonography and MRI, while management requires a multidisciplinary approach involving medical stabilization, endovascular embolization, or surgical options depending on the clinical severity. Recent advancements in endovascular techniques and early detection strategies have significantly improved survival outcomes and helped preserve neurological development in affected pediatric patients.
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