# Characterizing Initial Cervical Spine and Neurovascular Findings in 84 Consecutive Patients with Hypermobile Ehlers–Danlos Syndrome: A Retrospective Study

## TL;DR

This study finds that cervical spine issues and neurovascular changes are common in patients with hypermobile Ehlers–Danlos syndrome, potentially explaining their multisystem symptoms.

## Contribution

The study provides novel evidence linking cervical spine pathology and neurovascular compression to multisystem symptoms in hEDS patients.

## Key findings

- Over 71% of patients reported 29 or more symptoms, indicating significant multisystem involvement.
- Nearly all patients showed ligamentous cervical instability and reduced cross-sectional area of the internal jugular vein and vagus nerve.
- Vagus nerve cross-sectional area was significantly smaller than in healthy populations, suggesting degeneration.

## 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.