Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)

Orphanet Journal of Rare Diseases

Hinnerk Wulf³  Fransiska Malfait²  Marco Castori¹  Thomas Wiesmann³ 

[1] Division of Medical Genetics, Department of Molecular Medicine, San Camillo-Forlanini Hospital, Sapienza University, Circonvallazione Gianicolense 87, Rome, 00152, Italy;Ghent University Hospital - UZGent, De Pintelaan 185, Gent, 9000, Belgium;Department of Anesthesiology and Intensive Care Medicine, University Hospital Giessen and Marburg, Campus Marburg, Baldingerstrasse, Marburg, 35037, Germany

关键词: Connective tissue;    Spinal anesthesia;    Epidural anesthesia;    General anesthesia;    Rare disease;    Orphan disease;    Anesthesia;    EDS;    Ehlers-danlos syndrome;   

Others  :  866317
DOI  :  10.1186/s13023-014-0109-5

 received in 2014-05-12, accepted in 2014-07-02,  发布年份 2014

【 摘 要 】

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.

【 授权许可】

   
2014 Wiesmann et al. ; licensee Biomedcentral Ltd

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