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Published on: September 17, 2019
Characterizing Initial Cervical Spine and Neurovascular Findings in 84 Consecutive Patients with Hypermobile
Ross A Hauser1, Morgan Griffiths2, Ashley Watterson3
1Caring Medical Florida, 9738 Commerce Center Court, Fort Myers, FL 33908, USA.
Insights
Hypermobile Ehlers-Danlos syndrome (hEDS) patients often have cervical spine issues, including ligamentous instability and reduced vagus nerve size. Findings suggest potential internal jugular vein compression, contributing to multisystem symptoms in hEDS.
Area of Science:
- Orthopedics
- Neurology
- Vascular Medicine
Background:
- Hypermobile Ehlers-Danlos syndrome (hEDS) presents complex diagnostic and treatment challenges due to widespread symptoms and multisystem involvement.
- Objective characterization of cervical spine and neurovascular findings in hEDS is limited.
- Previous studies focused on upper cervical spine issues, but mechanisms for associated symptoms remain unclear.
Purpose of the Study:
- To examine objective findings in the cervical spine and neurovasculature of hEDS patients.
- To explore potential pathologies contributing to the clinical profile of hEDS.
- To understand the link between cervical spine and neurovascular findings and hEDS symptomatology.
Main Methods:
- Retrospective observational study of 71 hEDS patients (aged 20-50) at an outpatient neck center.
- Data collected via chart review, excluding prior neck surgery or trauma.
- Diagnostic tools included digital motion X-ray, cone beam CT, Doppler ultrasound, and tonometry.
Main Results:
- Over 71% of patients reported ≥29 symptoms.
- Nearly all patients showed forward head posture, decreased cervical curve depth, ligamentous instability, and reduced internal jugular vein (IJV) and vagus nerve cross-sectional area (CSA).
- Vagus nerve CSA was significantly smaller; IJV CSA was smaller at C1 than C4-C5, indicating potential C1 carotid sheath compression.
Conclusions:
- Cervical spine pathology, IJV compression, and vagus nerve degeneration are common in hEDS.
- These findings may contribute to or be an etiological basis for multisystem involvement in hEDS.
- Provides hypothesis-generating data for future mechanistic, diagnostic, and therapeutic studies in hEDS.
Abstract:
Background: Hypermobile Ehlers-Danlos syndrome (hEDS) can present as a complex interplay of widespread symptomatology and multisystem involvement, posing diagnostic and treatment challenges. Objective characterization of cervical spine and neurovascular findings in hEDS has been limited. Previous studies have emphasized upper cervical spine complications in hEDS, yet the relevance and mechanisms underlying associated symptomatology have not been elucidated. This study examined objective test findings in patients with hEDS at an outpatient neck clinic to explore cervical spine and neurovascular pathology that could contribute to further understanding the clinical profile of a subset of patients with hEDS. Methods: This single-center, retrospective observational study included patients with hEDS aged 20-50 years from 1 January 2022-31 December 2024, at an outpatient neck center. It excluded previous neck surgery, traumatic events, or related injury. Demographic, clinical, and diagnostic data were collected through a retrospective chart review, including measurements from standard clinical diagnostic protocols: digital motion X-ray (videofluoroscopy), cone beam CT, Doppler ultrasound, and tonometry. Results: More than 71% of patients reported ≥29 symptoms. Nearly all patients exhibited co-occurring forward head, decreased depth of curve, ligamentous cervical instability, and decreased internal jugular vein (IJV) and vagus nerve cross-sectional area (CSA). Vagus nerve CSA was found to be significantly smaller than the comparative healthy/normal population. IJV CSA was significantly smaller at C1 than at C4-C5, suggesting evidence of carotid sheath compression at C1. Conclusions: This study offers novel evidence that cervical spine pathology, IJV compression, and vagus nerve degeneration are uniformly prevalent in hEDS, which may contribute to, or be an etiological basis for, the multisystem involvement in a subset of patients with this disorder. These findings provide hypothesis-generating data to inform future mechanistic and therapeutic studies, including exploration of new diagnostic and treatment targets.
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