title: Posterior spinal fusion for scoliosis in Ehlers-Danlos syndrome, kyphoscoliosis type.
abstract: 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.
authors.affiliation: Department of Orthopedics, Changzheng Hospital, Second Military Medical University, Shanghai, China.
authors.forename: Yang
authors.initials: Y
authors.lastname: Liu
authors.forename: Rui
authors.initials: R
authors.lastname: Gao
authors.forename: Xuhui
authors.initials: X
authors.lastname: Zhou
authors.forename: Wen
authors.initials: W
authors.lastname: Yuan
mesh_terms: D000293:Adolescent; D004535:Ehlers-Danlos Syndrome / Q000601:surgery*; D005260:Female; D006801:Humans; D008297:Male; D011859:Radiography; D012600:Scoliosis / Q000000981:diagnostic imaging / Q000601:surgery*; D013123:Spinal Fusion / Q000295:instrumentation* / Q000379:methods*; D016896:Treatment Outcome