Cranio-cervical instability has been well identified in diseases with connective tissue like Ehlers-Danlos Syndrome- Hypermobility Type/Joint Hypermobility Syndrome. It is frequently associated with functional gastrointestinal disturbances, mast cell activation syndrome, and autonomic symptoms as postural orthostatic tachycardia syndrome. These patients suffer from chronic fatigue, and severe widespread pain of exceedingly difficult management and poor control with opioids. Chronic neuroinflammation, opioid-induced hyperalgesia, and Central Sensitization phenomena may explain this complex and painful condition.

The aim of this narrative review is to present updated information on the peri-operative considerations of patients with Ehlers-Danlos Syndrome-Hypermobility Type/Joint Hypermobility Syndrome and cranio-cervical instability who are undergoing Occipital-Cervical Fixation/Fusion. Our protocol of opioid-free total intravenous anesthesia will also be described. Acute post-operative pain management includes the use of a combination of anti-hyperalgesic coadjuvants (lidocaine, ketamine and dexmedetomidine) with an opioid-sparing effect.