A Case Report of Spondyloepiphyseal Dysplasia Congenita

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. Com...

Full description

Saved in:
Bibliographic Details
Published inJournal of Nippon Medical School Vol. 68; no. 2; pp. 186 - 189
Main Authors Gembun, Yoshikazu, Nakayama, Yoshihito, Shirai, Yasumasa, Miyamoto, Masabumi, Sawaizumi, Takuya, Kitamura, Shigeyuki
Format Journal Article
LanguageEnglish
Japanese
Published Japan The Medical Association of Nippon Medical School 2001
Subjects
atlanto-axial instability
Atlanto-Axial Joint - surgery
dwarfism with a short trunk
fusion operation
Humans
Joint Instability - etiology
Joint Instability - surgery
Male
Middle Aged
Osteochondrodysplasias - complications
Osteochondrodysplasias - congenital
paresis of the lower extremities
Spinal Fusion
Online AccessGet full text

Cover

More Information
Summary: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.
Bibliography:ObjectType-Case Study-3
SourceType-Scholarly Journals-1
content type line 23
ObjectType-Review-1
ObjectType-Feature-5
ObjectType-Report-2
ObjectType-Article-4
ISSN:1345-4676
1347-3409
DOI:10.1272/jnms.68.186