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Craniovertebral abnormalities in Down's syndrome.

A H Menezes1, T C Ryken

  • 1Division of Neurological Surgery, University of Iowa Hospitals and Clinics, Iowa City 52242.

Pediatric Neurosurgery
|January 1, 1992
PubMed
Summary
This summary is machine-generated.

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Occipitoatlantoaxial instability is more common in Down syndrome patients than previously thought, often leading to serious complications. Surgical stabilization offers excellent outcomes for these craniovertebral junction abnormalities.

Area of Science:

  • Neurology
  • Orthopedics
  • Genetics

Background:

  • Ligamentous laxity in Down syndrome patients is often associated with atlantoaxial subluxation.
  • Occipitoatlantoaxial instability is a poorly recognized complication in this population.

Observation:

  • A review of 18 symptomatic Down syndrome patients revealed significant craniovertebral junction abnormalities.
  • Fixed atlantoaxial luxation, occipitoatlantoaxial instability, and rotary luxation were prevalent.
  • Os odontoideum was present in 3 patients, and basilar invagination occurred in 2.

Findings:

  • Occipitoatlantoaxial instability is more common than previously assumed in Down syndrome.
  • Cervicomedullary compromise occurred in 5 patients with fixed atlantoaxial luxation.

Related Experiment Videos

  • Surgical interventions, including occipital cervical fixation and atlantoaxial dorsal fusion, yielded excellent results.

    • Implications:

    • Increased awareness of occipitoatlantoaxial instability in Down syndrome is crucial for early diagnosis and management.
    • Prompt surgical stabilization can effectively address craniovertebral junction abnormalities and prevent neurological deficits.
    • Understanding the spectrum of craniovertebral abnormalities aids in developing targeted treatment strategies.