Demetracopoulos Constantine A, Sponseller Paul D
Department of Orthopaedic Surgery, Johns Hopkins Medical Institutions, The Johns Hopkins Hospital, 601 North Caroline Street, Room 5253, Baltimore, MD 21287, USA.
Orthop Clin North Am. 2007 Oct;38(4):563-72, vii. doi: 10.1016/j.ocl.2007.04.003.
Marfan syndrome is a connective tissue disease that affects the skeletal system among other organ systems. Kyphoscoliosis, spondylolisthesis, and atlantoaxial subluxation are common spinal deformities in Marfan syndrome, and distinctive vertebral morphology within such patients presents significant treatment challenges. Although most scoliosis curves in patients who have Marfan syndrome are minor, those that require treatment progress rapidly; brace treatment has proven ineffective for most patients. Surgical correction is associated with complications, such as failure of fixation and additional deformity; however good results are possible when consideration is given to the unique challenges presented by patients who have Marfan syndrome.
马凡综合征是一种影响骨骼系统及其他器官系统的结缔组织疾病。脊柱后凸侧弯、椎体滑脱和寰枢椎半脱位是马凡综合征常见的脊柱畸形，此类患者独特的椎体形态给治疗带来了重大挑战。虽然大多数马凡综合征患者的脊柱侧弯弧度较小，但那些需要治疗的侧弯进展迅速；事实证明，支具治疗对大多数患者无效。手术矫正会引发并发症，如固定失败和额外畸形；然而，若考虑到马凡综合征患者所呈现的独特挑战，仍有可能取得良好效果。
