Journal of Nippon Medical School: Vol.68 No.2 page.186

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ArticleTitle A Case Report of Spondyloepiphyseal Dysplasia Congenita

AuthorList Yoshikazu Gembun, Yoshihito Nakayama, Yasumasa Shirai, Masabumi Miyamoto, Takuya Sawaizumi and Shigeyuki Kitamura

Affiliation Department of Orthopaedic Surgery, Nippon Medical School

Language EN

Volume 68

Issue 2

Year 2001

Page 186-189

Received November 14, 2000

Accepted November 30, 2000

Keywords dwarfism with a short trunk, paresis of the lower extremities, atlanto-axial instability, fusion operation

Abstract Spondyloepiphyseal dysplasia congenita (SED) is a rare form of skeletal systemic disease, characterized by congenital dwarfism with a short trunk and epiphysial dysplasia in the long bones and vertebral bodies. Patients also frequently saffer from atlanto-axial instability due to os odontoideum. Compression of the spinal cord caused by atlanto-axial instability is a common, serious complication in SED patients, and causes severe spinal cord symptoms or occasionally sudden death. We present an SED patient who underwent a posterior fusion of the occiput to the cervical spine for severe spinal cord symptoms due to atlanto-axial instability.

Correspondence to Yoshikazu Gembun M. D., Department of Orthopaedic Surgery, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan

ArticleTitle	A Case Report of Spondyloepiphyseal Dysplasia Congenita
AuthorList	Yoshikazu Gembun, Yoshihito Nakayama, Yasumasa Shirai, Masabumi Miyamoto, Takuya Sawaizumi and Shigeyuki Kitamura
Affiliation	Department of Orthopaedic Surgery, Nippon Medical School
Language	EN
Volume	68
Issue	2
Year	2001
Page	186-189
Received	November 14, 2000
Accepted	November 30, 2000
Keywords	dwarfism with a short trunk, paresis of the lower extremities, atlanto-axial instability, fusion operation
Abstract	Spondyloepiphyseal dysplasia congenita (SED) is a rare form of skeletal systemic disease, characterized by congenital dwarfism with a short trunk and epiphysial dysplasia in the long bones and vertebral bodies. Patients also frequently saffer from atlanto-axial instability due to os odontoideum. Compression of the spinal cord caused by atlanto-axial instability is a common, serious complication in SED patients, and causes severe spinal cord symptoms or occasionally sudden death. We present an SED patient who underwent a posterior fusion of the occiput to the cervical spine for severe spinal cord symptoms due to atlanto-axial instability.
Correspondence to	Yoshikazu Gembun M. D., Department of Orthopaedic Surgery, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan