A Severe Form of Cantrell` s Pentalogy with Complete Ectopia Cordis Komplet Ektopiya Kordisli Cantrell Pentalojisi Olgusu

Abstract

Keywords

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Komplet Ektopiya Kordisli Cantrell Pentalojisi Olgusu

Abstract

Cantrell pentalojisi, komplet veya parsiyel ektopia kordis ile birlikte alt sternum, anterior diyafragma ve perikard defektleri, omfalosel ve konjenital intrakardiyak defektlerle karakterize olan ve seyrek görülen bir konjenital sendromdur. Tüm bu defektlerin birlikte görüldüğü çok az sayıda vaka rapor edilmiştir. Bununla birlikte sonuçlar ana olarak kardiyak malformasyona bağlı olduğundan çok az sayıda hasta sağ kalabilmiştir. Burada, sözügeçen tüm defektlerin ve eşlik eden komplet torakoabdominal ektopia kordisin tespit edildiği Cantrell pentolojisinin en ağır formu ve bu nadir görülen durumun tedavisindeki fikir ayrılıkları rapor edilmektedir

Keywords

• Ektopia kordis, Cantrell pentalojisi, omfalosel

References

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