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Ectopia cordis associated with Cantrell's pentalogy.

Basant Kumar¹, Chetan Sharma, Devendra D Sinha

¹Department of Pediatric Surgery, Sir Padampat Mother and Child Health Institute (JayKayLon Hospital), SMS Medical College, Jaipur, Rajasthan, India. drbkpg75@rediff.com

Annals of Thoracic Medicine

|June 30, 2009

Summary

Cantrell's pentalogy is a rare, lethal congenital anomaly involving ectopia cordis. This case report details a neonate with multiple defects who succumbed to acidosis and electrolyte imbalance.

Keywords:

Abdominal wall defect Cantrell's pentalogy ectopia cordis

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Area of Science:

Pediatric Cardiology
Congenital Anomalies
Neonatal Medicine

Background:

Cantrell's pentalogy, characterized by ectopia cordis, is a rare and often lethal congenital condition.
The incidence is approximately 1 in 100,000 births in developed nations.

Observation:

A neonate presented with a severe form of Cantrell's pentalogy.
Observed defects included ectopia cordis, cleft lower sternum, upper abdominal wall defect, ectopic umbilicus, diaphragmatic defect, and interventricular septal defect.

Findings:

The neonate experienced respiratory distress and peripheral cyanosis.
Death occurred due to acidosis and electrolyte imbalance prior to surgical intervention.

Implications:

This case highlights the critical and often fatal nature of Cantrell's pentalogy.
Understanding embryogenesis is crucial for comprehending these complex congenital defects.