Assessment of craniocervical motion in Down syndrome: a pilot study of two measurement techniques

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OBJECTIVE

Hypermobility of the craniocervical junction (CCJ) in patients with Down syndrome (DS) is common. Whereas atlantoaxial (C1–2) hypermobility is well characterized, occipitoatlantal (Oc–C1) laxity is recognized but poorly defined. A clear understanding of the risks associated with DS-related hypermobility is lacking. Research efforts to address the topic of axial cervical spine instability in the patient with DS require a reliable and reproducible means of assessing CCJ mobility. The authors conducted a pilot study comparing two methods of quantifying motion of the CCJ on dynamic (flexion/extension) plain radiographs: the delta–condyle-axial interval (ΔCAI) and the delta–basion-axial interval (ΔBAI) methods.

METHODS

Dynamic radiographs from a cohort of 10 patients with DS were evaluated according to prescribed standards. Independent movement of Oc–C1, C1–2, and Oc–C2 was calculated. Interrater and intrarater reliability for CCJ mobility was then calculated for both techniques.

RESULTS

Measurement using the ΔCAI technique had excellent fidelity with intraclass correlation coefficients (ICCs) of 0.77, 0.71, and 0.80 for Oc–C1, C1–2, and Oc–C2, respectively. The ΔBAI technique had lower fidelity, yielding respective ICCs of 0.61, 0.65, and 0.50.

CONCLUSIONS

This pilot study suggests that ΔCAI is a superior measurement technique compared to ΔBAI and may provide reliable assessment of the mobility of the CCJ on dynamic radiographs in the pediatric patient with DS. The use of reliable and reproducible measurement techniques strengthens the validity of research derived from pooled database efforts.

ABBREVIATIONS AAI = atlantoaxial interval; AAL = anterior axial line; CCJ = craniocervical junction; ΔAAI = delta–atlantoaxial interval; ΔBAI = delta–basion-axial interval; ΔCAI = delta–condyle-axial interval; ΔNCW = delta–neural canal width; DS = Down syndrome; ICC = intraclass correlation coefficient; Oc = occipital level; PAL = posterior axial line.
Article Information

Contributor Notes

Correspondence Jonathan E. Martin: University of Connecticut School of Medicine, Connecticut Children’s Medical Center, Hartford, CT. jmartin03@ccmckids.org.INCLUDE WHEN CITING Published online October 4, 2019; DOI: 10.3171/2019.7.PEDS191.Disclosures Dr. Pahys is a consultant for DePuy Synthes, NuVasive, and Zimmer Biomet.
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