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Pentalogy of Cantrell: a case report
Mahadev Dixit1, Mohan Gan, Raghunath Mohapatra
1Department of Cardiovascular and Thoracic Surgery, JN Medical College.
Journal of the Indian Medical Association
|March 27, 2010
Summary
Pentalogy of Cantrell, a rare congenital anomaly, presents midline defects and heart issues. Surgical intervention for a six-year-old girl highlighted the need for combined cardiac and plastic surgery expertise.
Area of Science:
- Pediatric Surgery
- Congenital Anomalies
- Medical Genetics
Background:
- Pentalogy of Cantrell is a rare congenital anomaly characterized by midline mesodermal defects.
- It is frequently associated with congenital heart disease, posing complex treatment challenges.
Observation:
- A six-year-old girl presented with a significant supra-umbilical abdominal wall defect and herniating abdominal contents.
- The defect involved deficiency of the lower sternum and xiphoid, indicative of Cantrell's Pentalogy.
Findings:
- The case highlights the abdominal wall defect component of Pentalogy of Cantrell.
- Surgical management required addressing both the abdominal wall defect and underlying congenital heart disease.
Implications:
- Optimal treatment necessitates close collaboration between pediatric cardiac surgeons and plastic surgeons.
- Early diagnosis and multidisciplinary surgical planning are crucial for improved outcomes in Pentalogy of Cantrell patients.
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