STUDY DESIGN. Retrospective study of 8 children treated with vertical expandable prosthetic titanium rib (VEPTR) for correction of early onset spinal scoliotic deformities. METHODS. 8 children with progressive scoliosis due to a variety of conditions, 6 congenital (2 Goldenhar syndrome, 2 VACTERL syndrome, 2 congenital thoracic abnormalities), 1 spondyloepiphyseal dysplasia, 1 early onset of scoliosis, underwent the index procedure and subsequent lengthening procedures at 6 months intervals (1 patient had 11 lengthening procedures). Mean age was 4 years (2-6 years) and mean follow up 3.8 years (2-6 years). Mean preoperative Cobb angle was 64,8° (51-108) and mean postoperative angle 40° (31-50). RESULTS. There were no neurological complications. Three patients developed infection with wound breakdown. One patient underwent removal of one of the two VEPTR rods. In the other two patients the rods were removed followed by antibiotics and the VEPTR converted to another growing rod system. Overall, patients tolerated the multiple procedures well. Three patients experienced significant distress with multiple surgical procedures. CONCLUSION. VEPTR offers a viable treatment option for children with severe congenital and early onset scoliosis where there are no viable alternatives. It achieves and maintains spinal deformity correction, while allowing for continued spine and chest-wall growth. Complication rate is acceptable in view of the benefits. None of the complications have lead to long term complications to date, but the repeated lengthening have resulted in psychological disturbance which we are investigating further. An implantable driver would offer very significant advantages and would avoid some of the repeated operations

Aims

The aim of this study was to compare outcomes after growth-friendly treatment for early-onset scoliosis (EOS) between patients with skeletal dysplasias versus those with other syndromes.

Acute angulation at the thoracolumbar junction with segmental subluxation of the spine occurring at the level above an anteriorly hypoplastic vertebra in otherwise normal children is a rare condition described as infantile developmental thoracolumbar kyphosis. Three patient series with total of 18 children have been reported in the literature. We report five children who presented with thoracolumbar kyphosis and discuss the treatment algorithm. We reviewed the medical records and spinal imaging at initial clinical presentation and at minimum two-year follow-up. The mean age at presentation was eight months (two to 12). All five children had L2 anterior vertebral body hypoplasia. The kyphosis improved spontaneously in three children kept under monitoring. In contrast, the deformity was progressive in two patients who were treated with bracing. The kyphosis and segmental subluxation corrected at latest follow-up (mean age 52 months; 48 to 60) in all patients with near complete reconstitution of the anomalous vertebra. The deformity and radiological imaging on a young child can cause anxiety to both parents and treating physicians. Diagnostic workup and treatment algorithm in the management of infantile developmental thoracolumbar kyphosis is proposed. Observation is indicated for non-progressive kyphosis and bracing if there is evidence of kyphosis and segmental subluxation deterioration beyond walking age. Surgical stabilisation of the spine can be reserved for severe progressive deformities unresponsive to conservative treatment.

Cite this article: Bone Joint J 2015;97-B:982–7.