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Thoracoabdominoplasty with umbilicoplasty for Cantrell's syndrome
Mikio Kinoshita1, Susam Park, Tomohiro Shiraishi
1Department of Plastic Surgery, Shizuoka Children's Hospital, Shizuoka, Japan. kinosim-tky@umin.ac.jp
Insights
Cantrell syndrome patients often face challenges with thoracoabdominal wall reconstruction, leaving the heart vulnerable. This study presents a surgical technique using rib cartilage and rectus muscles to reconstruct the lower sternum, improving outcomes.
Area of Science:
- Pediatric Surgery
- Congenital Anomalies
- Thoracic Reconstruction
Background:
- Cantrell syndrome involves multiple congenital anomalies affecting the abdominal wall, sternum, diaphragm, pericardium, and heart.
- Inadequate initial thoracoabdominal wall reconstruction can lead to ventral herniation and cardiac exposure in survivors.
- Lower sternal reconstruction is a critical but rarely reported aspect of managing these complex defects.
Observation:
- Four patients with Cantrell syndrome requiring lower sternal reconstruction were evaluated.
- Three patients underwent thoracoabdominoplasty and umbilicoplasty utilizing autologous rib cartilage and rectus muscles.
- The surgical approach aimed to protect the heart and improve the cosmetic appearance of the thoracoabdominal region.
Findings:
- Solid sternal reconstruction was achieved in all treated patients.
- Excellent abdominal appearance was noted post-surgery.
- The described technique effectively addressed the challenges of lower sternal defects in Cantrell syndrome.
Implications:
- This surgical approach offers a viable solution for lower sternal reconstruction in Cantrell syndrome.
- Improved structural integrity protects the heart and reduces the risk of trauma.
- Enhanced cosmetic outcomes contribute to better quality of life for affected children.
Abstract:
Cantrell et al. described a syndrome with five anomalies characterised by defects of the abdominal wall, lower sternum, anterior diaphragm, diaphragmatic pericardium, and heart. Because most of the children who survived could not have the thoracoabdominal wall adequately reconstructed at the initial operation, ventral herniation is often the result and they have to live with the danger of direct trauma to the unprotected heart. It therefore becomes important protect the unguarded heart while improving the appearance of the thoracoabdominal region. The reconstruction of the lower sternum has rarely been reported. We describe four such patients, three of whom had a thoracoabdominoplasty and umbilicoplasty using autologous rib cartilage and rectus muscles. All these children had solidly reconstructed sternums, and their abdominal appearances are excellent.
