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Repeat Pediatric Trisomy 21 Radiographic Exam: Does Atlantoaxial Instability Develop Over Time?
Jennifer M Bauer1, Virkamal K Dhaliwal2, Samuel R Browd3
1Seattle Children's Hospital Department of Orthopaedic Surgery, University of Washington Department of Orthopaedics and Sports Medicine.
Journal of Pediatric Orthopedics
|June 25, 2021
Summary
Atlantoaxial instability (AAI) affects 4.4% of children with Trisomy 21, with a 1.6% progression rate over 4 years. Os odontoideum increases risk, warranting continued surveillance in affected children.
Area of Science:
- Orthopedics
- Genetics
- Radiology
Background:
- Atlantoaxial instability (AAI) is a significant concern in pediatric patients with Trisomy 21, posing a risk of spinal cord injury.
- Current screening recommendations for cervical spine abnormalities in these children lack consistent age and timing guidelines.
- Understanding the temporal development of AAI is crucial for effective patient management.
Purpose of the Study:
- To investigate the incidence and progression of atlantoaxial instability (AAI) in pediatric patients with Trisomy 21 over time.
- To identify risk factors and patterns of instability development in this population.
Main Methods:
- Retrospective review of cervical spine radiographs from pediatric Trisomy 21 patients with at least two imaging series (2008-2020).
- Measurement of Atlanto-dens interval (ADI) and Space Available for the Cord (SAC); assessment for bony abnormalities like os odontoideum.
- Comparison of patients who developed AAI with those who remained stable, analyzing age and time intervals between radiographs.
Main Results:
- Seven of 160 (4.4%) pediatric Trisomy 21 patients exhibited AAI, with 3 (1.6%) developing instability on subsequent imaging over an average of 4.3 years.
- Os odontoideum was significantly associated with instability (71% in unstable vs. 2% in stable spines, P<0.0001).
- No specific age or surveillance duration reliably excluded the risk of developing AAI.
Conclusions:
- Pediatric Trisomy 21 patients have a notable risk of developing AAI, with a subset showing progression over time.
- Initial cervical spine screening is recommended for children with Trisomy 21 over 3 years of age.
- Continued surveillance is advised for those with os odontoideum due to their elevated risk of instability.
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