Anesthesia Clinical Practice Guidelines for Ehlers-Danlos Syndrome: A Modified Delphi Survey

Ehlers-Danlos syndrome (EDS) is a family of inherited connective tissue disorders distinguished by joint hyperextensibility, skin hyperelasticity, and increased tissue friability. No standardized anesthesia guidelines currently exist for this population, despite facing increased perioperative risks. To address this gap in literature, a three-round modified Delphi survey was conducted with 15 experts in anesthesia and EDS. Seven preliminary guidelines, developed from a comprehensive literature review, were evaluated and refined across three rounds. Consensus was achieved for all seven guidelines by the third round. Key recommendations include comprehensive airway assessment for all EDS subtypes; bleeding risk evaluation and consideration of desmopressin in classical, classical-like, kyphoscoliotic, and vascular EDS; multimodal analgesia and regional anesthesia strategies for hypermobile EDS; perioperative planning that incorporates common comorbidities; and cautious use of regional or neuraxial anesthesia in vascular EDS, requiring individual risk-benefit analysis due to hematoma risk. This study establishes the first consensus-based clinical practice guidelines for the anesthetic care of patients with EDS. While limited by sample size and gaps in existing evidence, these recommendations provide a framework to enhance patient safety by guiding clinical decision-making and encouraging future research in the perioperative management of EDS.