Artikel
Occipito-cervical junction in spondyloepiphyseal and spondylometaphyseal dysplasia : neurosurgical aspects: a review of two paediatric observations
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Autoren
Veröffentlicht: | 4. Mai 2005 |
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Gliederung
Text
Objective
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.
Methods
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.
Results
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.
Conclusions
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.