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AtlantoAxial Rotatory Subluxation in Down’s Syndrome

- See: Down's Syndrome:

- Discussion:
     - atlantoaxial instability is common in pts with Down's syndrome.
     - significant instability requires stabilization when the spinal cord is at risk for injury.
     - children interested in Special Olympics require screening;

- Radiographs:
     - children should be screened w/ dynamic lateral flexion/ extension;
     - normal atlanto-dens intervals in Down's syndrome may be up to 5 mm;

- Non Operative Rx:
     - ADI > 5 mm:
           - in asymptomatic pts avoid activities involving high-impact flexion loading on the cervical spine;
           - avoidance of contact sports, high jump, and diving;
     - ADI of 7-9 mm;
           - implies moderate instability;
           - managed by observation, cervical orthosis, & avoidance of contact sports;

- Indications for Surgical Treatment:
     - significant instability;
     - neurologic signs or symptoms;
     - ADI > 10 mm;

- Complications:
     - attempts at surgical stabilization of upper C-spine in children w/ Down  syndrome are fraught w/ significant complications;
     - fixed dislocation at junction of C-1 and C-2 are not treated by open reduction because of the high incidence of mortality.
     - treatment of choice is w/ fixed dislocation is  laminectomy of C-1 w/ posterior arthrodesis from occiput to C-2

Surgical treatment of symptomatic atlantoaxial subluxation in Down's syndrome.

Chronic atlanto-axial instability in Down syndrome.

Cervical-spine instability in children with Down syndrome (trisomy 21).

Complications of Posterior Arthrodesis of the Cervical Spine in  Patients Who Have Down Syndrome.

Atlanto-axial dislocation in Down's syndrome: Report of 2 cases requiring surgical correction

Atlantoaxial instability in Down syndrome: subject review. American Academy of Pediatrics Committee on Sports Medicine and Fitness

Instability of the upper cervical spine in Down syndrome.

Reproducibility in the measurement of atlanto-occipital instability in children with Down syndrome