Early-Onset Scoliosis (EOS) is a complex pediatric spinal deformity that progresses rapidly. Young children with EOS may develop impaired pulmonary function due to the high risk of aggravation of spinal curvatures and thoracic constraints during a critical period for lung development. Some of these patients are at risk of developing Thoracic Insufficiency Syndrome (TIS) due to Volume Depletion Deformities (VDD), particularly the Type II (with rib synostosis). In these cases, aggressive curve progression and thoracic constriction can compromise severe lung development at this early stage of life. Historically, early spinal fusion of the thoracic spine resulted in iatrogenic restrictive lung disease due to shortening of thorax “height” and its consequences. Although spinal hemiepiphysiodesis is a recognized growth-guided technique, appropriate for this type of condition, the outcome can be rather unreliable and its failure can lead to severe residual chest and spinal deformities that raises significant reconstructive challenges.

Case Reports: We present two cases of Congenital Scoliosis with Rib Synostosis at risk of TIS, treated with expandable thoracic distraction until final posterior instrumented fusion in adolescence. Case 1 is a two-year-old boy with a 68º thoracic scoliosis. Following a hemiepiphysiodesis, the deformity progressed to 93º by age six, requiring a salvage procedure. He was treated with an Expandable Thoracic Distraction System, later converted to VEPTR with subsequent repeated distraction program, resulting in a final Cobb angle of 55º. Case 2 is a 2 year old boy with Goldenhar Syndrome whose thoracic curve progressed to 114º despite a convex growth arrest at 2 years of age. VEPTR implantation at age five with subsequent repeated distraction program successfully controlled the deformity. In both cases patients reached skeletal maturity for definitive spinal fusion in a stable condition with preserved respiratory function.

Conclusions: For patients at risk of TIS due to congenital EOS and rib synostosis, expandable thoracic distraction device is an alternative growth sparing tool in the armamentarium to correct a severe thoracic scoliosis while preserving thoracic spine lengthening and optimizing pulmonary growth development. Failure of hemiepiphysiodesis often requires these more invasive salvage distraction techniques to manage aggressive curve progression. Clinical success should be assessed by the stabilization of respiratory condition and preservation of vertical trunk growth, rather than by Cobb angle correction only.