Ectopia cordis associated with Cantrell′s pentalogy

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 umbi...

Full description

Bibliographic Details
Main Authors: Kumar Basant, Sharma Chetan, Sinha Devendra, Sumanlata
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2008-01-01
Series:Annals of Thoracic Medicine
Subjects:
Online Access:http://www.thoracicmedicine.org/article.asp?issn=1817-1737;year=2008;volume=3;issue=4;spage=152;epage=153;aulast=Kumar
Description
Summary: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
ISSN:1817-1737
1998-3557