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 1:100,000 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.
Kumar
Basant
B
Department of Pediatric Surgery, Sir Padampat Mother and Child Health Institute (JayKayLon Hospital), SMS Medical College, Jaipur, Rajasthan, India. drbkpg75@rediff.com
Sharma
Chetan
C
Sinha
Devendra D
DD
Sumanlata