Table 4.

Overview of articles reporting surgical treatment for CCI in EDS patients. The clinical assessment measures, criteria for surgery, and imaging methods described in these articles are listed.

Author	Year	Clinical Assessment and Surgical Criteria	Imaging	Imaging Criteria
Henderson F et al	2016	Moderate to severe headache or suboccipital pain	Dynamic MRI, CT	CXA <135°
		Bulbar symptoms constituting the cervical medullary syndrome
		Neurological findings of myelopathy
Henderson F et al	2018	Formal genetic evaluation and diagnosis with a hereditary connective tissue disorder	Dynamic MRI or CT	CXA <135°
		Severe headache and/or neck pain ≥ to7/10 VAS >6 months		Harris/Bai measurement in flexion minus extension >4 mm
		Symptoms of cervical medullary syndrome
		Demonstrable neurological deficits
		Failed conservative treatment
Henderson F et al	2020	Formal genetic evaluation and diagnosis with a hereditary connective tissue disorder	Dynamic CT, x-ray	Angle subtended by C1-C2 > than 41°
		Severe headache and/or neck pain for greater than 6 months		C1-C2 facet overlap <10%
		Symptoms compatible with atlanto-axial instability		Translation on lateral tilt >3.5 mm on open mouth views
		Congruent neurological deficits
		Failed conservative treatment
Henderson F et al	2021	Same as above	Same as above	Same as above
Spiessberger A et al	2020	Interdisciplinary evaluation confirming CCI-related symptoms	MRI, CT, x-ray (flexion/extension)	Confirmed CCI, not further specified
		Assessment by EDS specialist
		Symptoms perceived as unbearable
		Improvement of symptoms after 4–6-week trial of hard collar immobilization