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Class II pentalogy of Cantrell
Helga Naburi1, Evelyne Assenga2, Sonal Patel3
1Department of Paediatrics and Child Health, Muhimbili University of Health and Allied Sciences, Dar es Salaam, Tanzania. hnaburi2000@yahoo.com.
BMC Research Notes
|July 30, 2015
Summary
Pentalogy of Cantrell, a rare congenital defect, was diagnosed in a Tanzanian infant. Early surgical intervention is crucial for survival, but resource limitations in Tanzania contributed to a poor outcome.
Area of Science:
- Pediatric Surgery
- Congenital Anomalies
- Neonatal Care
Background:
- Pentalogy of Cantrell is a rare congenital syndrome characterized by five major defects.
- It involves the diaphragm, abdominal wall, pericardium, sternum, and heart.
- The syndrome has a low survival rate, emphasizing the need for early surgical repair.
Observation:
- A neonate in Tanzania presented with omphalocele and ectopia cordis.
- Diagnostic imaging revealed absence of the lower sternum and intra-cardiac defects.
- The infant developed cardiac failure within the first week of life.
Findings:
- The infant was diagnosed with Pentalogy of Cantrell.
- Inadequate local facilities hindered complex corrective surgery.
- The infant succumbed to death at 9 months due to medication and transport challenges.
Implications:
- Resource-limited settings present significant challenges for managing complex congenital anomalies.
- Timely surgical intervention is critical for improving survival rates in infants with Pentalogy of Cantrell.
- The establishment of cardiac surgery facilities in Tanzania offers hope for better management of such cases in the future.
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