2014/07/22 by Thomas Wiesmann, Marco Castori, Fransiska Malfait +1 · 1 citation
Biochemistry, Genetics and Molecular Biology · Medicine · #Connective tissue disorders research #Dermatological and Skeletal Disorders #Dupuytren's Contracture and Treatments #Ehlers–Danlos syndrome #Joint hypermobility #Perioperative #Medicine #Connective Tissue Disorder #Intensive care medicine #Connective tissue #Surgery #Pathology #Physical therapy
paper · pdf · doi:10.1186/s13023-014-0109-5
openalex publication_date 2014/07/22 · openalex created_date 2025/10/10 · openalex updated_date 2026/07/29
Ehlers-Danlos syndrome (EDS, ORPHA98249) comprises a group of clinically and genetically heterogeneous heritable connective tissue disorders, chiefly characterized by joint hypermobility and instability, skin texture anomalies, and vascular and soft tissue fragility. As many tissues can be involved, the underlying molecular defect can manifest itself in many organs and with varying degrees of severity, with widespread implications for anesthesia and perioperative management. This review focuses on issues relevant for anesthesia for elective and emergency surgery in EDS. We searched the literature for papers related to all EDS variants; at the moment most of the published data deals with the vascular subtype and, to a lesser extent, classic and hypermobility EDS. Knowledge is fragmented and consists mostly of case reports, small case series and expert opinion. Because EDS patients commonly require surgery, we have summarized some recommendations for general, obstetrical and regional anesthesia, as well as for hemostatic therapy.