Craniocervical instability after inadvertent neck hyperextension in Ehlers-Danlos syndrome: a retrospective case series and literature review
Abstract
BACKGROUND: Patients with hypermobile Ehlers-Danlos Syndrome (hEDS) are at risk of developing craniocervical instability (CCI). Iatrogenic causes of CCI in hEDS are underreported. We describe a case series of hEDS patients who developed CCI from routine neck hyperextension during common procedures (e.g., intubation for anesthesia, dental work). METHODS: We conducted a retrospective case series of 8 adults (≥ 18 years old) with hEDS (2017 diagnostic criteria) who had no preoperative CCI symptoms but developed new CCI symptoms after a surgical procedure that persisted for at least 5 years. Cases were identified from patients seen at a single tertiary center between January 1, 2024, and July 31, 2025. Data collected included symptom onset, symptom types, procedure details (including neck positioning), imaging findings, and clinical outcomes. A structured literature review was performed using PubMed, Scopus, and Web of Science (through November 2025) for reports of CCI in EDS, including terms “Ehlers-Danlos syndrome,” “craniocervical instability,” “atlantoaxial instability,” “occipitocervical fusion,” “upper cervical instability,” “neck hyperextension,” and “ligamentous laxity.” RESULTS: All 8 patients were female (mean age 26.3 ± 6.0 years). Three patients underwent dental extractions, two had laparoscopic appendectomy, one had laparoscopic cholecystectomy, one had turbinate reduction, and one had upper endoscopy. Four cases involved prolonged neck hyperextension without…
How strong is the evidence?
Evidence Hub reads this as a case report, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health. Reported size: 8 participants or records.
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