Case Report: Surgical management of thoracic chondrodysplasia in children: complete mobile thoracic replacement

Several syndromes, including Jeune's syndrome and Sensenbrenner's syndrome, can cause thoracic chondrodysplasia in children, leading to various degrees of thoracic insufficiency syndrome. Surgical management if often necessary and numerous surgical techniques have been described in the past, but all of these offer rigid solutions, which are incompatible with growth, and full recovery of ventilatory dynamics. We describe a new surgical technique for these patients, which offers a complete mobile thoracic replacement.

We present the case of two patients with Jeune and Sensenbrenner syndromes in whom we performed a complete thoracic replacement. We used the Multidirectional Thoracic Wall Stabilization (MTS) system, (StraTos™ MedXpert's), in order to recreate a mobile thorax, allow for recovery of ventilatory dynamics and enable thoracic growth.

Both surgeries were successful. Long-term follow-up (>4 years), shows both patients regained respiratory autonomy for acts of daily routine, increased their walking perimeter and increased their lung volumes.

Total thoracic replacement is safe and feasible for thoracic dysplasia in children. Further experience is needed to formally compare outcomes with the traditional techniques.