Operative Strategies for Thoracic Insufficiency Syndrome by Vertical Expandable Prosthetic Titanium Rib Expansion Thoracoplasty

The 3-dimensional thoracic deformity responsible for thoracic insufficiency syndrome in children can be addressed by correcting the respective volume depletion deformity through vertical expandable prosthetic titanium rib (VEPTR) expansion thoracoplasty. These deformities include absent ribs, fused ribs and scoliosis, windswept deformity of the thorax in early onset scoliosis, and hypoplastic thorax. The core principle of this surgical technique is the early restoration of maximum volume and symmetry of the thorax with indirect correction of the scoliosis without the growth inhibition effects of spine surgery. Operative strategies of VEPTR expansion thoracoplasty include stabilization of flail chest, expansion of constricted chest walls, and control of spinal deformity. This approach, especially when performed in infancy, is postulated to provide stimulus for lung growth by making available additional thoracic volume for these patients. Most of these patients also have exotic scoliosis, a rare and possibly lethal subset of spinal deformity, which requires special care and treatment. Although VEPTR expansion thoracoplasty is a straightforward surgical technique, the preoperative selection of patients for VEPTR surgery is complex, and meticulous attention to postoperative management is critical for success. The long-term goal of VEPTR treatment is to have the largest and most functional thorax possible by skeletal maturity to provide optimal respiratory function for adulthood.

Keywords:  thoracic insufficiency syndrome , expansion thoracoplasty , opening wedge thoracostomy , exotic scoliosis , VEPTR , secondary thoracic insufficiency syndrome , volume depletion deformity , lateral flexion contracture of the thorax