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

Consensus was achieved for all seven preliminary anesthesia guidelines for EDS by the third round of a modified Delphi survey.

• A three-round modified Delphi survey methodology was used.
• 15 experts in anesthesia and EDS participated.
• Seven preliminary guidelines were developed from a comprehensive literature review prior to the Delphi rounds.
• Guidelines were evaluated and refined across all three rounds before consensus was reached.

Comprehensive airway assessment was recommended for all EDS subtypes as a key anesthesia guideline.

• The recommendation applies across all subtypes of EDS, not limited to specific variants.
• EDS is characterized by joint hyperextensibility, skin hyperelasticity, and increased tissue friability, all of which may affect airway management.
• This guideline was among the seven that achieved expert consensus by the third Delphi round.

Bleeding risk evaluation and consideration of desmopressin was recommended for classical, classical-like, kyphoscoliotic, and vascular EDS subtypes.

• The recommendation for desmopressin consideration was specific to four named EDS subtypes: classical, classical-like, kyphoscoliotic, and vascular EDS.
• This guideline reflects the increased tissue friability and bleeding risk associated with these subtypes.
• Consensus on this guideline was achieved by the third Delphi round.

Multimodal analgesia and regional anesthesia strategies were recommended specifically for hypermobile EDS.

• The guideline targets hypermobile EDS (hEDS), which is described as the most common subtype.
• Multimodal analgesia approaches were recommended, reflecting the complex pain profiles associated with hEDS.
• Regional anesthesia strategies were included as part of the pain management recommendations for this subtype.

Perioperative planning that incorporates common EDS comorbidities was established as a consensus guideline.

• The guideline recognizes that EDS patients frequently present with significant comorbidities relevant to perioperative care.
• No specific comorbidities were enumerated in the abstract, but the guideline requires their systematic incorporation into planning.
• Consensus was achieved for this guideline by the third Delphi round.

Cautious use of regional or neuraxial anesthesia in vascular EDS was recommended, requiring individual risk-benefit analysis due to hematoma risk.

• Vascular EDS was specifically identified as requiring heightened caution with regional and neuraxial anesthesia techniques.
• The primary concern cited is hematoma risk associated with these anesthesia approaches in vascular EDS patients.
• The guideline calls for individualized risk-benefit analysis rather than a blanket contraindication.
• Consensus was achieved for this guideline by the third Delphi round.

The study acknowledges limitations of small sample size and gaps in existing evidence base underlying the guidelines.

• The expert panel consisted of 15 participants, which the authors identify as a sample size limitation.
• The authors note 'gaps in existing evidence' as a constraint on the strength of the recommendations.
• Despite these limitations, the authors characterize the guidelines as providing 'a framework to enhance patient safety by guiding clinical decision-making and encouraging future research in the perioperative management of EDS.'