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Ectopia cordis in a Nigerian child

O A Adebo1, F A Arowolo, O Osinowo

  • 1Department of Surgery, University College Hospital, University of Ibadan, Nigeria.

West African Journal of Medicine
|October 1, 1993
PubMed
Summary
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This case study describes a rare congenital condition in a newborn with complete ectopia cordis and a single atrium, presenting with multiple severe heart defects and omphalocele. The infant did not survive beyond 45 hours due to the complex anomalies.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Neonatal Surgery

Background:

  • Ectopia cordis is a rare congenital anomaly where the heart is abnormally positioned outside the chest.
  • Complete ectopia cordis involves severe defects of the sternum and pericardium.
  • Single atrium is a rare congenital heart defect characterized by a single ventricle and atrium.

Observation:

  • A female infant presented with complete ectopia cordis, a single atrium, and complete sternal deficiency four hours after birth.
  • The infant had an omphalocele containing an enlarged liver and absence of pericardium.
  • Intracardiac anomalies included a ventricular septal defect, overriding aorta, and total anomalous venous drainage.

Findings:

  • The infant exhibited a complex spectrum of congenital anomalies, including severe cardiac malformations and an omphalocele.

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  • Despite medical intervention, the infant succumbed to the condition within 45 hours of birth.
  • The combination of complete ectopia cordis, single atrium, and other intracardiac defects represents a critical congenital condition.
  • Implications:

    • This case highlights the severe challenges in managing neonates with complex ectopia cordis and associated anomalies.
    • Early recognition and multidisciplinary management are crucial for improving outcomes in such critical cases.
    • Further research into the genetic and environmental factors contributing to these complex congenital defects is warranted.