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

Journal of Neurosurgery
|December 25, 2007
PubMed

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.
Abstract

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