Pentalogy of Cantrell
نویسندگان
چکیده
Özet İlk olarak 1958 yılında Cantrell, Haller ve Ravitch tarafından tanımlanan Cantrell sendromu, ektopia kordis ile birlikte diyafragma, orta hat karın duvarı ve perikardı etkileyen nadir görülen bir anomalidir. Cantrell sendromu sıklıkla intrakardiyak defektler ile birliktelik göstermektedir. Kompleks kardiyak anomali prognozu belirlemede önemli bir role sahiptir. Çeşitli derecelerde birçok sistemi etkileyen bu sendromun tedavi süreci fetal tanı başlamaktadır. Bu aşama itibariyle, farklı disiplinlerden tarafından paylaşılan görüş ve deneyimler hasta yönetimini planlamasında önemlidir. Ancak, tüm bu çabalara rağmen, tedavi şansı bu sendromun ağır formları için düşüktür.
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امفالوسل و قلب بیرون از قفسه سینه درنوزاد با Pentalogy of Cantrell
Background : Pentalogy of Cantrell is a very rare congenital anomaly which is diagnosed with severe thoracoabdominal defect, omphalocele, lower sternal defect, anterior diaphragmatic defect anterior pericardial defect and ectopia cordis.Pathogenesis is unknown, mesodermal developmental defects in 14-18 days after conception maybe responsible . Case Report: The case is newborn with omphalocele ...
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INTRODUCTION The pentalogy of Cantrell is rare clustering of congenital defects, first described by Cantrell and colleagues in 1958. The exact pathogenesis for the pentalogy remains unknown and no specific genetic abnormalities have been correlated; however, a failure of embryogenesis has been suspected. The microduplication of chromosome 15q21.3 (57,529,846 to 58,949,448) found in our patient ...
متن کاملPentalogy of Cantrell: case report and review of the literature.
Pentalogy of Cantrell is a syndrome that consists of five anomalies: a midline, upper abdominal wall abnormality; lower sternal defect; anterior diaphragmatic defect; diaphragmatic pericardial defect, and congenital abnormalities of the heart. The pathogenesis of this condition is not fully known yet, associations are common with this condition and treatment is extremely challenging. Prognosis ...
متن کاملPentalogy of Cantrell: Is Echocardiography Sufficient in the Neonatal Period?
Pentalogy of Cantrell is a rare syndrome that is characterized by varying degrees of midline wall defects and congenital cardiac anomalies. A left ventricular diverticulum (LVD) is defined as partial ectopia cordis, can be part of the pentalogy of Cantrell, and can put the patient at risk of severe complications. Early diagnosis and ligation/resection of the LVD is important to prevent complica...
متن کاملPrenatal diagnosis of pentalogy of cantrell in a case with craniorachischisis
The pentalogy of Cantrell was first described by Cantrell et al in 1958, who reported 5 cases with this anomaly [1]. The findings included a lower sternum defect, a midline supraumblical thoracoabdominal wall defect, a defect of the anterior diaphragm, a defect of the diaphragmatic pericardium and congenital cardiac anomalies [1,2]. The pentalogy is associated with very poor prognosis and there...
متن کاملAntenatal Diagnosis of a Case of Pentalogy of Cantrell Associated with Phocomelia Cantrell Pentaloji ve Fokomelia Birlikteliği Saptanan Olgunun Antenatal Tanısı
Melek Çiçek, Kafkas Üniversitesi Tıp Fakültesi, Kadın Hastalıkları ve Doğum Anabilim Dalı, Tel. 0506 8609381 Email. [email protected] Geliş Tarihi: 05.12.2011 • Kabul Tarihi: 16.12.2011 ABSTRACT Pentalogy of Cantrell is a rare congenital syndrome; described as the association of partial or complete ectopia cordis, a supraumbilical abdominal wall defect, congenital heart defects, defects ...
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