Occult spinal canal stenosis due to C-1 hypoplasia in children with Down syndrome
Shunji Matsunaga1, Takanori Imakiire, Hiroaki Koga
1Department of Orthopaedic Surgery, Imakiire General Hospital Kibougaoka Hospital, Kagoshima, Japan. shunji@m.kufm.kagoshima-u.ac.jp
Insights
Children with Down syndrome often have smaller C-1 vertebrae, increasing the risk of spinal canal stenosis and atlantoaxial subluxation, potentially leading to myelopathy.
Area of Science:
- Pediatric Orthopedics
- Genetics
- Neurology
Background:
- Down syndrome is associated with various skeletal abnormalities.
- C-1 hypoplasia and its link to spinal canal stenosis in this population remain understudied.
- Occult spinal canal stenosis poses a risk for neurological complications.
Purpose of the Study:
- To investigate subclinical spinal canal stenosis in children with Down syndrome due to C-1 hypoplasia.
- To compare C-1 dimensions and atlas canal area in children with and without Down syndrome.
- To assess the risk of atlantoaxial subluxation and myelopathy.
Main Methods:
- A matched comparison study with a cross-sectional survey.
- 102 children with Down syndrome (ages 10-15) were matched with 176 normal children.
- Measurements included anteroposterior (AP) diameter of C-1, atlas-dens interval (ADI), and cross-sectional atlas area via X-ray and CT imaging.
Main Results:
- Eight children (6.7%) with Down syndrome showed atlantoaxial subluxation with myelopathy.
- While ADI did not differ significantly, children with Down syndrome had smaller average AP atlas diameter and spinal canal area.
- These findings indicate a smaller C-1 structure in children with Down syndrome.
Conclusions:
- C-1 hypoplasia is common in children with Down syndrome, leading to occult spinal canal stenosis.
- This condition significantly elevates the risk of atlantoaxial instability and myelopathy.
- Early detection and monitoring are crucial for managing potential neurological complications.
Object:
Little has been published about subclinical spinal canal stenosis due to C-1 hypoplasia in patients with Down syndrome. In this paper the authors performed a matched comparison study with cross-sectional survey to investigate occult spinal canal stenosis due to C-1 hypoplasia in children with Down syndrome.
Methods:
A total of 102 children with Down syndrome ranging in age from 10 to 15 years were matched according to age and physique with 176 normal children. In all participants, the anteroposterior (AP) diameter of C-1 and the atlas-dens interval (ADI) were measured on plain lateral x-ray images of the cervical spine. The cross-sectional area of the atlas was also measured from a cross-sectional computed tomography image of C-1.
Results:
Eight children (6.7%) with Down syndrome developed atlantoaxial subluxation associated with myelopathy. The difference in the ADI between the patients and controls was not statistically significant. The average AP diameter of the atlas and the spinal canal area along the cross-section of the atlas were significantly smaller in children with Down syndrome than those in the control group.
Conclusions:
Atlantoaxial instability and occult spinal canal stenosis due to C-1 hypoplasia in patients with Down syndrome may significantly increase the risk of myelopathy.
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