Hypermobile Ehlers-Danlos syndrome (hEDS) is a hereditary connective tissue disorder characterized by generalized joint hypermobility, soft tissue fragility, chronic pain, and autonomic dysfunction. Although major vascular complications are uncommon in hEDS, affected individuals frequently undergo orthopedic procedures, including ligament reconstructions, tendon repair, and joint stabilization, due to recurrent dislocations and musculoskeletal injury. Consequently, careful evaluation of pneumatic tourniquet use in this patient population is warranted. Pneumatic tourniquets are widely used in extremity orthopedic surgery to reduce blood loss and improve visualization. However, their effects on peripheral nerve integrity, pain outcomes, and soft tissue injury in patients with hEDS remain poorly understood. These concerns are particularly relevant in the context of regional anesthesia, where chronic pain syndromes and autonomic dysfunction may complicate perioperative analgesia and postoperative neurologic assessments. This narrative review examines the pathophysiology of hEDS and explores anesthetic and orthopedic considerations related to tourniquet use in this patient population. While tourniquets offer clear intraoperative advantages, patients with hEDS may be at increased risk for skin injury, compression neuropraxia, exaggerated ischemia-reperfusion pain, and delayed postoperative recovery. In the absence of evidence-based guidelines, tourniquet use in patients with hEDS should involve shared decision-making among anesthesiologists, surgeons, and patients. Individualized discussions addressing potential risks, alternative techniques, and modifiable tourniquet parameters, such as pressure levels, duration, and padding placement, may help mitigate perioperative complications.
Unterman et al. (Fri,) studied this question.