Spinal deformities in patients with connective tissue disorder are more likely to develop early, progress more rapidly, and be less responsive to bracing than patients with idiopathic deformities, often resulting in surgical fixation. Surgeries can be challenging as pedicle, lamina, and vertebral shapes are affected by the syndrome and dural ectasia is almost universal, especially in Marfan syndrome. Evidence for a non-fusion surgery is limited. Level selection principles are different than in idiopathic deformity. Geneticists can be helpful in diagnosing these patients because of the complexity of this group of disorders.

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Nuances of Syndromic Scoliosis: Connective Tissue Marfan, Ehlers-Danlos, and Loeys-Dietz

  • Myung-Jin Cha,
  • Paul Sponseller

摘要

Spinal deformities in patients with connective tissue disorder are more likely to develop early, progress more rapidly, and be less responsive to bracing than patients with idiopathic deformities, often resulting in surgical fixation. Surgeries can be challenging as pedicle, lamina, and vertebral shapes are affected by the syndrome and dural ectasia is almost universal, especially in Marfan syndrome. Evidence for a non-fusion surgery is limited. Level selection principles are different than in idiopathic deformity. Geneticists can be helpful in diagnosing these patients because of the complexity of this group of disorders.