Meltem Bingöl KOLOĞLU, Aydın YAĞMURLU, İsmail Haluk GÖKÇORA, Bilal ALPER, Hülya Özkan ULU

A severe form of cantrell' s pentalogy with complete ectopia cordis

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 fi kir ayrılıkları rapor edilmektedir.

Komplet ektopiya kordisli cantrell pentalojisi olgusu

Cantrell’s Pentalogy (CP) is a rare congenital syndrome characterized by defects of lower sternum, anterior diaphragm and pericardium with complete or partial ectopia cordis, an omphalocele and congenital intracardiac defects. Few cases manifesting all the defects have been reported, however, only a small number have survived as the outcome mainly depends on the cardiac malformation. Herein, we report a case of most severe form of CP manifesting all defects and associated with complete thoracoabdominal ectopia cordis to discuss the challenges in management of this uncommon entity.

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