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Complete Ectopia Cordis: A Case Report and Literature Review
Simon Pius1, Halima Abubakar Ibrahim1, Mustapha Bello1
1Department of Paediatrics, University of Maiduguri Teaching Hospital, Maiduguri, Nigeria.
Insights
Ectopia cordis, a rare congenital heart defect, involves heart protrusion through the chest wall. Early prenatal diagnosis and prompt intervention are crucial for potentially salvaging affected infants.
Area of Science:
- Cardiology
- Congenital Anomalies
- Pediatric Surgery
Background:
- Ectopia cordis is a rare congenital condition characterized by the heart's abnormal positioning outside the chest cavity.
- It is often associated with pentalogy of Cantrell, involving defects in the thoracoabdominal wall and other visceral structures.
- The condition has a high mortality rate, with many infants succumbing within days of birth, even with surgical intervention.
Observation:
- A case of ectopia cordis in an infant born to an uneducated rural family is presented.
- The infant survived for seven days, exceeding the typical survival period often cited in medical literature.
- Diagnosis was established postnatally, highlighting potential delays in identification and management.
Findings:
- Prenatal diagnosis via antenatal ultrasound is possible in well-equipped facilities, enabling early management planning.
- The presented case demonstrates prolonged survival despite the rarity and severity of ectopia cordis.
- This case underscores the importance of proactive diagnosis and timely treatment initiation.
Implications:
- Increased awareness among pediatricians and obstetricians regarding ectopia cordis is essential for early detection.
- Prompt and proactive management strategies may improve outcomes for infants with this rare congenital heart defect.
- Further research into optimizing diagnostic and therapeutic approaches for ectopia cordis is warranted to improve salvage rates.
Abstract:
Ectopia cordis is a congenital heart exposure defined as complete or partial protrusion of heart through ventral defect in the thoracoabdominal wall alone or with other viscera in cases of pentalogy of Cantrell. This condition was first described by Haller et al. in 1706; since then many advances have been made. Diagnosis of ectopia cordis is done prenatally in well-equipped health facility by antenatal ultrasound scan so that early diagnosis and management plan can be initiated. The index case was delivered to uneducated rural family and admitted at 3 days of life and survived for seven days, even though most literatures state that majority died within four days even with surgery. So, in view of this, we presented this case report to deliberately draw the attention of paediatrician/obstetrician to the fact that even though this condition is rare, proactive search and diagnosis should be made and early treatment should be instituted, so that such a child may be salvaged.
