gms | German Medical Science

56. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e. V. (DGNC)
3èmes journées françaises de Neurochirurgie (SFNC)

Deutsche Gesellschaft für Neurochirurgie e. V.
Société Française de Neurochirurgie

07. bis 11.05.2005, Strasbourg

Occipito-cervical junction in spondyloepiphyseal and spondylometaphyseal dysplasia : neurosurgical aspects: a review of two paediatric observations

Meeting Abstract

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  • corresponding author O. Klein - Department of Neurosurgery, Unit of Paediatric Neurosurgery, CHU Nancy
  • J. C. Marchal - Department of Neurosurgery, Unit of Paediatric Neurosurgery, CHU Nancy
  • P. Lascombes - Department of paediatric orthopaedic surgery, CHU Nancy

Deutsche Gesellschaft für Neurochirurgie. Société Française de Neurochirurgie. 56. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e.V. (DGNC), 3èmes journées françaises de Neurochirurgie (SFNC). Strasbourg, 07.-11.05.2005. Düsseldorf, Köln: German Medical Science; 2005. Doc09.05.-01.03

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Veröffentlicht: 4. Mai 2005

© 2005 Klein et al.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen ( Er darf vervielfältigt, verbreitet und öffentlich zugänglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.




Spondyloepiphyseal dysplasia (SED) and spondylometaphyseal dysplasia (SMD) belong to the large group of osteochondrodysplasia: anomalies of bony growth, erroneous development of cartilaginous tissue and abnormalities of density and structure of the bone. In some cases, these anomalies may be present at the site of the occipito-cervical junction (OCJ) and could be revealed by compression or instability problems.


We report two observations of OCJ abnormalities in the context of SED and SMD. The first patient (suffering from SED) was admitted in our institution when he was 11 year old with tetra upper motor syndrome. MRI showed a posterior compressing medulla at the level of the OCJ. A trepanolaminectomy was performed with a good result. Three years later he was admitted again with neck pain and diffuses paresthesias. CT-scan demonstrated a C1-C2 subluxation, a fragmentation of the odontoid process and an agenesis of the C1 anterior arch. An occipito-cervical fixation via a posterior approach was done with a patient who completely recovered. The second patient (suffering from SMD) was admitted with upper motor syndrome when he was 17. Neuroradiological data showed a C1-C2 disclocation, basilar impression and cervical kyphosis. A posterior occipito-cervical arthrodesis was performed with an immediate post-operative worsening of symptoms before a complete recovery within a few months.


The two patients needed a neurosurgical intervention (C0-C3 arthrodesis with bony grafts by posterior approach) with a good success, but with a transitory worsening in one patient.


Osteochondrodysplasias may be at risk for the OCJ. The neurosurgeon must bear in mind that a decompression and/or a stabilization of the OCJ may be necessary in the evolution of the disease. The mechanisms of these lesions and the surgical possibilities are discussed.