Liu Yang, Gao Rui, Zhou Xuhui, Yuan Wen
Department of Orthopedics, Changzheng Hospital, Second Military Medical University, Shanghai, China.
Orthopedics. 2011 Jun 14;34(6):228. doi: 10.3928/01477447-20110427-28.
The Ehlers-Danlos syndromes comprise a clinically and genetically heterogeneous group of heritable connective tissue disorders characterized by articular hypermobility, skin extensibility, and tissue fragility. Surgical treatment of scoliosis associated with Ehlers-Danlos syndrome poses a challenge to spine surgeons because of the high risk of major complications. There is a paucity of evidence in the literature on surgical treatment for scoliosis in the Ehlers-Danlos syndrome patient.This article describes 3 adolescent patients diagnosed with Ehlers-Danlos syndrome, kyphoscoliosis type, which was treated by posterior spinal fusion only. After unsuccessful conservative treatment for at least 1 year, the patients underwent posterior spinal surgery for the correction of spinal deformity. A satisfactory correction in the spinal curve was achieved, with no obvious loss of correction during follow-up. No intra- or postoperative major complications were observed.Our experience supports that a satisfactory correction of scoliosis can be achieved by posterior spinal fusion only in patients with Ehlers-Danlos syndrome, kyphoscoliosis type.
埃勒斯-当洛综合征是一组临床和遗传异质性的遗传性结缔组织疾病，其特征为关节活动过度、皮肤伸展性和组织脆弱性。与埃勒斯-当洛综合征相关的脊柱侧弯的手术治疗对脊柱外科医生构成了挑战，因为主要并发症的风险很高。关于埃勒斯-当洛综合征患者脊柱侧弯手术治疗的文献证据很少。本文描述了3例诊断为埃勒斯-当洛综合征、后凸脊柱侧弯型的青少年患者，仅通过后路脊柱融合术进行治疗。在至少1年的保守治疗失败后，患者接受了后路脊柱手术以矫正脊柱畸形。脊柱侧凸得到了满意的矫正，随访期间矫正无明显丢失。未观察到术中或术后的主要并发症。我们的经验支持，对于埃勒斯-当洛综合征、后凸脊柱侧弯型的患者，仅通过后路脊柱融合术就可以实现脊柱侧弯的满意矫正。
