Posterior Spinal Fusion for Scoliosis in Ehlers-Danlos Syndrome, Kyphoscoliosis Type

Liu, Yang MD
Gao, Rui MD
Zhou, Xuhui MD
Yuan, Wen MD, PhD

Drs Liu, Gao, Zhou, and Yuan are from the Department of Orthopedics, Changzheng Hospital, Second Military Medical University, Shanghai, China.

Drs Liu, Gao, Zhou, and Yuan have no relevant financial relationships to disclose.

Drs Liu and Gao contributed equally to this article.

Correspondence should be addressed to: Wen Yuan, MD, PhD, Department of Orthopedics, Changzheng Hospital, Second Military Medical University, 415 Fengyang Rd, Shanghai, 200003, China ( [email protected]).

Orthopedics 34(6):p e228-e232, June 14, 2011. | 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.