Difference between revisions of "Ehlers-Danlos syndromes"
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Preoperative evaluation should focus on bleeding history, cervical/TMJ hypermobility, skin fragility, scoliosis, signs of mitral/aortic insufficiency | Preoperative evaluation should focus on bleeding history, cervical/TMJ hypermobility, skin fragility, scoliosis, signs of mitral/aortic insufficiency | ||
===Intraoperative management< | ===Intraoperative management=== | ||
===== <u>Monitoring</u>: ===== | |||
===== <u>Positioning:</u> ===== | |||
* Eyes should be protected to present retinal detachment and globe rupture | |||
* Careful padding of extremities | |||
===== <u>Skin Fragility</u> ===== | |||
* Minor forces can result in skin injuries such as tape, stickers, and other monitors that attach to the patient | |||
* Bruising and hematoma formation occurs with non-invasive BP monitoring | |||
===Postoperative management<!-- Describe how this comorbidity may influence postoperative management. -->=== | ===Postoperative management<!-- Describe how this comorbidity may influence postoperative management. -->=== | ||
Revision as of 13:39, 2 July 2021
Ehlers-Danlos syndromes
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| Anesthetic management |
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Ehlers-Danlos (EDS) is a set of heritable heterogenous connective tissue disorders characterized by defects in collagen synthesis, affecting skin, ligaments, joints, blood vessels and other organ. EDS patients often have vascular fragility syndromes with arterial aneurysms and dissections. Patients also have kyphoscoliosis and due to the connective tissue abnormalities, these patients often present for orthopedic surgery. Their are six manor subtypes ranging phenotypically from very mild to life-threatening phenotypes.[1][2]
Anesthetic implications
Preoperative optimization
Preoperative evaluation should focus on bleeding history, cervical/TMJ hypermobility, skin fragility, scoliosis, signs of mitral/aortic insufficiency
Intraoperative management
Monitoring:
Positioning:
- Eyes should be protected to present retinal detachment and globe rupture
- Careful padding of extremities
Skin Fragility
- Minor forces can result in skin injuries such as tape, stickers, and other monitors that attach to the patient
- Bruising and hematoma formation occurs with non-invasive BP monitoring
Postoperative management
Related surgical procedures
Pathophysiology
Signs and symptoms
Diagnosis
Treatment
Medication
Surgery
Prognosis
Epidemiology
References
- ↑ Parapia, Liakat A.; Jackson, Carolyn (2008-04). "Ehlers-Danlos syndrome--a historical review". British Journal of Haematology. 141 (1): 32–35. doi:10.1111/j.1365-2141.2008.06994.x. ISSN 1365-2141. PMID 18324963. Check date values in:
|date=(help) - ↑ Wiesmann, Thomas; Castori, Marco; Malfait, Fransiska; Wulf, Hinnerk (2014-07-23). "Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)". Orphanet Journal of Rare Diseases. 9 (1): 109. doi:10.1186/s13023-014-0109-5. ISSN 1750-1172. PMC 4223622. PMID 25053156.CS1 maint: PMC format (link)