Symptomatic atlantoaxial instability in Down syndrome.
J Neurosurg
; 103(3 Suppl): 231-7, 2005 Sep.
Article
en En
| MEDLINE
| ID: mdl-16238076
ABSTRACT
OBJECT The aim of this study was to audit the treatment of symptomatic atlantoaxial instability in Down syndrome and to assess factors associated with fusion failure in this group of patients. METHODS:
The authors reviewed the cases of 12 children with Down syndrome presenting with symptomatic atlantoaxial instability who underwent surgery for internal fixation and fusion. A review of clinical histories, radiological investigations, and details of operative interventions was also performed. The mode of presentation was acute spinal cord injury (five cases), progressive myelopathy (four cases), and neck pain or stiffness (three cases). The atlantodental interval ranged from 5 to 13 mm. The posterior atlantodental interval at the C-1 level was 9.5 mm (range 6-11 mm). In 10 patients an os odontoideum was present. Translocation of the odontoid process occurred in one patient, and occipitoatlantal instability was also identified in two cases. Twenty-four operations were performed in the 12 patients. A transoral odontoidectomy was required in four children. Successful fusion was demonstrated in seven patients at the first operation. Three of the five patients with acute cord injury made significant functional recovery and were left with no or mild disability.CONCLUSIONS:
Additional bone abnormalities at the CVJ are common in the Down syndrome population. Young age at the time of fusion and multiple osseous anomalies pose a higher risk factor in fusion failure. The authors recommend an aggressive surgical approach for management of symptomatic cases of CVJ instability.
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Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Articulación Atlantoaxoidea
/
Fusión Vertebral
/
Síndrome de Down
/
Procedimientos Ortopédicos
/
Inestabilidad de la Articulación
Tipo de estudio:
Diagnostic_studies
/
Etiology_studies
/
Observational_studies
/
Prognostic_studies
Límite:
Adolescent
/
Child
/
Child, preschool
/
Female
/
Humans
/
Male
Idioma:
En
Revista:
J Neurosurg
Año:
2005
Tipo del documento:
Article
País de afiliación:
Reino Unido