Abstract
Background: Congenital scoliosis is nowadays addressed by many surgical solutions. There are rare cases presenting with mixed vertebral defects associated with Thoracic Insufficiency Syndrome (TIS). The presence of mixed vertebral defects may present with a very high progressive potential, inducing a severe scoliotic curve which impairs the development of the thoracic cage. Additionally, these cases may associate rib fusions which will further impair the development of the lung in these patients. The unique surgical solution in these cases is represented by VEPTR (Vertical Expandable Prosthesis Titanium Rib) devices able to expand the thorax and to control the scoliotic curve. Material and methods: The surgical technique of the VEPTR device in a rib-to-spine construct consists in a distraction based device able to expand the thorax on the concave side of the scoliosis by consecutive distractions and, secondary, to control de scoliotic curve. The upper module of the device is placed on the first, second and/or third rib and the lower module is fixed to the upper lumbar spine. The construct is completed with the expansion module which allows distractions (1/2 or 1 cm for each distraction). The patients undergo every 4-6 months consecutive distractions until skeletal maturity when definitive spinal fusion is performed. Results: The VEPTR device in standard constructs with placement as recommended by the developer proved to be efficient in controlling congenital scoliotic curves and thoracic expansion. Unusual placements of the device complicated the evolution of patients with congenital scoliosis and required additional surgical procedures to correct the deformities and to try to ensure remnant spinal growth. Conclusions: The VEPTR device has strict indications, congenital scoliosis with TIS being one of them. Proper indication and surgical procedure will aid the patients all along spinal and thoracic growth, in order to obtain a lung and spinal development as close to normal as possible in order to avoid premature death due to TIS.
Published Version
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