All cervical spine radiographs of 44 patients with Crouzon syndrome treated at Great Ormond Street Hospital during the past 10 years were studied.
To assess the incidence and pattern of cervical spine abnormalities of patients with a confirmed diagnosis of Crouzon syndrome, but particularly regarding progressive fusion. of Background Data.
Previous studies into the cervical spine anomalies in those with Crouzon syndrome have shown an increased incidence of congenital abnormalities compared with the normal population.
There is some suggestion from previous studies that cervical spine fusions are progressive in nature.
All radiographs were reviewed by the craniofacial team, along with a single pediatric-radiologist with experience in assessment of skeletal dysplasias.
Radiologic abnormalities included « butterfly » vertebrae and fusions of the bodies and the posterior elements.
Evidence of fusion was present in eight of 44 (18%) of patients.
C2-C3 and C5-C6 were almost equally affected.
Block fusions involving multiple vertebrae were seen.
Analysis of sequential studies in 16 patients snowed evidence of progression in five.
These results reveal an incidence of fusions that is lower than reported previously. (...)
Mots-clés Pascal : Dysostose crâniofaciale Crouzon, Rachis cervical, Symptomatologie, Radiographie, Critère, Signe radiologique, Epidémiologie, Incidence, Enfant, Homme, Face, Système ostéoarticulaire pathologie, Maladie héréditaire, Malformation, Crâne pathologie, Radiodiagnostic
Mots-clés Pascal anglais : Crouzon disease, Cervical spine, Symptomatology, Radiography, Criterion, Radiologic sign, Epidemiology, Incidence, Child, Human, Face, Diseases of the osteoarticular system, Genetic disease, Malformation, Skull disease, Radiodiagnosis
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 97-0197260
Code Inist : 002B15H. Création : 21/05/1997.