Incomplete Cantrell's Pentalogy-A Case Report

Area of Science:

• Pediatric Surgery
• Congenital Malformations
• Developmental Biology

Background:

• Pentalogy of Cantrell is a rare congenital condition involving defects of the diaphragm, abdominal wall, pericardium, sternum, and heart.
• It presents a spectrum of anomalies with varying severity, posing diagnostic and therapeutic challenges.

Purpose of the Study:

• To report a complex case of Pentalogy of Cantrell in an 11-year-old boy.
• To highlight the diagnostic and surgical challenges associated with this rare syndrome.

Main Methods:

• Case presentation of an 11-year-old male with congenital ventral hernia.
• Detailed clinical examination and description of associated anomalies including sternal defects, liver malformations, and diaphragmatic/pericardial defects.

Main Results:

• The patient presented with a large ventral midline defect from umbilicus to xiphisternum, a bifid sternum, a three-lobed liver, and a diaphragmatic defect continuous with a pericardial defect.
• The heart was visible pulsating directly through the skin due to the extensive anterior midline defects.

Conclusions:

• Pentalogy of Cantrell, despite modern surgical advancements, remains a significant challenge due to the wide range of anomalies and high mortality.
• Early recognition and comprehensive management strategies are crucial for improving outcomes in affected children.