Annals of Thoracic Medicine Official publication of the Saudi Thoracic Society, affiliated to King Saud University
 
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CASE REPORT
Year : 2008  |  Volume : 3  |  Issue : 4  |  Page : 152-153

Ectopia cordis associated with Cantrell's pentalogy


1 Department of Pediatric Surgery, Sir Padampat Mother and Child Health Institute (JayKayLon Hospital), SMS Medical College, Jaipur, Rajasthan, India
2 Community Health Center, Jalalabad, Shahjahanpur, (UP), India

Correspondence Address:
Basant Kumar
Room No.-189, Resident Doctors` Hostel, JLN Marg, SMS Medical College, Jaipur-302 004
India
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DOI: 10.4103/1817-1737.43084

PMID: 19561899

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Cantrell's pentalogy with ectopia cordis is an extremely rare and lethal congenital anomaly, with a reported incidence of 1:100000 births in developed countries. We report a neonate who presented with ectopia cordis along with cleft lower sternum, upper abdominal wall defect, ectopic umbilicus, diaphragmatic defect, and interventricular septal defect. The neonate had respiratory distress with peripheral cyanosis and died because of acidosis and electrolyte imbalance before surgical intervention could be undertaken. We discuss the case and present a brief review of literature and of embryogenesis


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