To describe a new surgical technique for thoracic chondrodysplasia in children with Jeune and Sensenbrenner syndromes that allows for mobile thoracic replacement, recovery of ventilatory dynamics, and thoracic growth.
Approach:
Surgical Technique: The Multidirectional Thoracic Wall Stabilization (MTS) system was used to achieve complete thoracic replacement in two patients with Jeune and Sensenbrenner syndromes, facilitating growth and recovery of ventilatory dynamics.
Key Findings:
Both surgeries were successful.
Long-term follow-up (>4 years) showed regained respiratory autonomy, increased walking perimeter, and increased lung volumes in both patients.
Interpretation:
Total thoracic replacement is safe and feasible for thoracic dysplasia in children.
Limitations:
Further experience is needed to formally compare outcomes with traditional surgical techniques for thoracic chondrodysplasia.
Conclusion:
Total thoracic replacement offers a dynamic solution for thoracic chondrodysplasia in children.
