|Ectopia Cordis and Pentalogy of Cantrell: Report of Two Cases|
|Murat Bakacak1, Mehmet Sühha Bostancı2, Salih Serin1, Cemil Goya3, Zeyneb Bakacak4, Fazlı Avcı1|
|1Kahramanmaras Sutcu Imam University, Department of Obstetrics and Gynecology, Kahramanmaras, Turkey
2Sakarya University Research and Education Hospital, Department of Obstetrics and Gynecology, Sakarya, Turkey
3Dicle University, Department of Radiology and Diagnostic Imaging, Diyarbakır, Turkey
4Specialist of Obstetrics and Gynecology, Kahramanmaras, Turkey
IJWHR 2014; 2: 323–325
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Keywords : Pentalogy of Cantrell, Ectopia Cordis, Congenital Heart Defect
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Ectopia cordis (EC) is the presence of a live, beating heart outside the thorax and is one of the most unique congenital anomalies. EC has been a weak association noted with trisomy 18 and a few cases have been associated with other chromosomal abnormalities. Pentalogy of cantrell (PC) is a rare congenital syndrome of abdominal wall defect, lower sternal defect, diaphragmatic pericardial defect, anterior diaphragmatic defect, and intracardiac abnormalities. It has a rare frequency of about 1/100,000 births. The hallmark of this syndrome is an omphalocele associated with EC. Only a few patients with the full spectrum of the pentalogy have been described. Only very few patients survive attempts at surgical repair, the main causes of death being tachyarrhythmias, bradycardia, low blood pressure, rupture of the diverticulum, and heart failure. Early diagnosis is important about discuss the option of elective termination with the parents if intracardiac anomalies of incompatible with life are noted. We report two cases of PC diagnosed in early second trimester.
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