Syncope due to Cervical Spine Hyperextension in Hypermobile Ehlers-Danlos Syndrome: Successful Treatment with Cranio-Cervical Stabilization: Case Report

Abstract

Background:The hypermobile form of Ehlers-Danlos syndrome (h-EDS) may be associated with cranio-vertebral instability causing neurological symptoms. The underlying mechanism of these symptoms refers to brainstem compression in flexion. We report a unique case of h-EDS with cranio-cervical instability causing syncopal events in cervical extension successfully treated with surgical stabilization.Case Presentation: A 25-year-old woman with h-EDS complained of syncopal events occurring systematically when she made cervical extension. Radiological evaluation confirmed cranio-cervical instability due to ligamentous hyper laxity. Radio metrics performed on dynamic cervical MRI in flexion and extension revealed pathological values in the range of motion of the cervical spine. Surgical treatment with occipito-axial stabilization was performed. No postoperative complication occurred. The patient did not experience any syncopal event after the surgery. The reduction in the range of motion was not felt to be disabling by the patient. Postoperative dynamic radiographs confirmed the stabilization of the occipitocervical junction.Conclusions: Syncope occurring systematically due to cranio-cervical instability in extension may occur in patients with h-EDS. Successful treatment with surgical posterior stabilization of C0-C2 allows to eradicate the disabling symptoms of syncope.