Review Perioperative opioid-minimization approach as a useful protocol in the management of patients with Ehlers–Danlos syndrome-... 2023 Ramírez-Paesano

Full title: Perioperative opioid-minimization approach as a useful protocol in the management of patients with Ehlers–Danlos syndrome-hypermobility type, craniocervical instability and severe chronic pain who are to undergo occipito-cervical fixation

Patients suffering from connective tissue disorders like Ehlers–Danlos syndrome hypermobility type/joint hypermobility syndrome (EDS-HT/JHS) may be affected by craniocervical instability (CCI). These patients experience myalgic encephalomyelitis, chronic fatigue, depression, extreme occipital-cervical pain, and severe widespread pain that is difficult to relieve with opioids. This complex and painful condition can be explained by the development of chronic neuroinflammation, opioid-induced hyperalgesia, and central sensitization.

Given the challenges in treating such severe physical pain, we evaluated all the analgesic methods previously used in the perioperative setting, and updated information was presented. It covers important physiopathological aspects for the perioperative care of patients with EDS-HT/JHS and CCI undergoing occipital-cervical/thoracic fixation/fusion. Moreover, a change of paradigm from the current opioid-based management of anesthesia/analgesia in these patients to the perioperative opioid minimization strategies used by the authors was analyzed and proposed as follow-up considerations from our previous case series. These strategies are based on total-intravenous opioid-free anesthesia, multimodal analgesia, and a postoperative combination of anti-hyperalgesic coadjuvants (lidocaine, ketamine, and dexmedetomidine) with an opioid-sparing effect.

Open access, https://ojrd.biomedcentral.com/articles/10.1186/s13023-023-02829-9

 

"Some patients with Ehlers–Danlos syndrome-hypermobility type/joint hypermobility syndrome (EDS-HT/JHS) may suffer from Craniocervical Instability (CCI). CCI can result in continuous microtraumas and inflammation on craniocervical joint surfaces. It causes repetitive peripheral sensitization, which eventually leads to the phenomenon of central sensitization (CS) and hyperalgesia [1, 2]. These patients have complex clinical features that often consist of depression, chronic fatigue syndrome (CFS), myalgic encephalomyelitis (ME) [3]"

Reference [3] is to Jason LA, Zinn ML, Zinn MA. Myalgic encephalomyelitis: symptoms and biomarkers. Curr Neuropharmacol. 2015;13(5):701–34. https://doi.org/10.2174/1570159x13666150928105725. It is paywalled, I would be interested if anybody could advise if it talks about CCI at all.