Expansion Thoracoplasty as a Life-Saving Procedure in an Adolescent With Severe Spinal Deformity and Sacral Agenesis

Case report.

To describe use of expansion thoracoplasty (ET) for severe thoracic insufficiency syndrome (TIS) in an adolescent with severe spinal deformity.

ET is typically performed in young patients with TIS to increase chest cavity volume, improve alveolar expansion, and potentially improve alveolar proliferation. ET has not been well-described in adolescent patients with TIS.

A mature adolescent with previously treated myelokyphosis and sacral agenesis developed severe TIS with dependence on supplemental oxygen and noninvasive ventilation. She was treated with two-stage bilateral ET and vertical expandable prosthetic titanium rib (VEPTR) placement. Yearly pulmonary function testing (PFT) was performed over 7 years of follow-up.

Significant clinical pulmonary improvement was achieved and maintained at final follow-up, as the patient no longer required supplemental oxygen. Percentage predicted forced vital capacity (FVC) improved from 29% to 36%; percentage predicted forced expiratory volume-1 second (FEV1) improved from 30% to 36%.

This case demonstrates that improvement and stabilization of respiratory function can be achieved with instrumented ET in a skeletally mature adolescent with severe TIS and spinal deformity.