Title |
Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)
|
---|---|
Published in |
Orphanet Journal of Rare Diseases, July 2014
|
DOI | 10.1186/s13023-014-0109-5 |
Pubmed ID | |
Authors |
Thomas Wiesmann, Marco Castori, Fransiska Malfait, Hinnerk Wulf |
Abstract |
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. |
X Demographics
Geographical breakdown
Country | Count | As % |
---|---|---|
United States | 15 | 27% |
United Kingdom | 9 | 16% |
Malaysia | 1 | 2% |
Spain | 1 | 2% |
Belgium | 1 | 2% |
Sri Lanka | 1 | 2% |
Unknown | 28 | 50% |
Demographic breakdown
Type | Count | As % |
---|---|---|
Members of the public | 46 | 82% |
Practitioners (doctors, other healthcare professionals) | 4 | 7% |
Scientists | 4 | 7% |
Science communicators (journalists, bloggers, editors) | 2 | 4% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
United Kingdom | 1 | <1% |
United States | 1 | <1% |
South Africa | 1 | <1% |
Unknown | 131 | 98% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Other | 24 | 18% |
Researcher | 16 | 12% |
Student > Bachelor | 16 | 12% |
Student > Master | 12 | 9% |
Student > Doctoral Student | 9 | 7% |
Other | 26 | 19% |
Unknown | 31 | 23% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 74 | 55% |
Nursing and Health Professions | 6 | 4% |
Agricultural and Biological Sciences | 5 | 4% |
Neuroscience | 3 | 2% |
Biochemistry, Genetics and Molecular Biology | 3 | 2% |
Other | 10 | 7% |
Unknown | 33 | 25% |