Difference between revisions of "Ehlers-Danlos syndromes"
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Ehlers-Danlos is a set of heritable heterogenous connective tissue disorders characterized by defects in collagen synthesis, affecting skin, ligaments, joints, blood vessels and other organ. Their are six manor subtypes ranging phenotypically from very mild to life-threatening phenotypes.<ref>{{Cite journal|last=Parapia|first=Liakat A.|last2=Jackson|first2=Carolyn|date=2008-04|title=Ehlers-Danlos syndrome--a historical review|url=https://pubmed.ncbi.nlm.nih.gov/18324963|journal=British Journal of Haematology|volume=141|issue=1|pages=32–35|doi=10.1111/j.1365-2141.2008.06994.x|issn=1365-2141|pmid=18324963}}</ref><ref>{{Cite journal|last=Wiesmann|first=Thomas|last2=Castori|first2=Marco|last3=Malfait|first3=Fransiska|last4=Wulf|first4=Hinnerk|date=2014-07-23|title=Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)|url=https://doi.org/10.1186/s13023-014-0109-5|journal=Orphanet Journal of Rare Diseases|volume=9|issue=1|pages=109|doi=10.1186/s13023-014-0109-5|issn=1750-1172|pmc=PMC4223622|pmid=25053156}}</ref> | 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.<ref>{{Cite journal|last=Parapia|first=Liakat A.|last2=Jackson|first2=Carolyn|date=2008-04|title=Ehlers-Danlos syndrome--a historical review|url=https://pubmed.ncbi.nlm.nih.gov/18324963|journal=British Journal of Haematology|volume=141|issue=1|pages=32–35|doi=10.1111/j.1365-2141.2008.06994.x|issn=1365-2141|pmid=18324963}}</ref><ref>{{Cite journal|last=Wiesmann|first=Thomas|last2=Castori|first2=Marco|last3=Malfait|first3=Fransiska|last4=Wulf|first4=Hinnerk|date=2014-07-23|title=Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)|url=https://doi.org/10.1186/s13023-014-0109-5|journal=Orphanet Journal of Rare Diseases|volume=9|issue=1|pages=109|doi=10.1186/s13023-014-0109-5|issn=1750-1172|pmc=PMC4223622|pmid=25053156}}</ref> | ||
== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. -->== | == Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. -->== | ||
Revision as of 12:33, 2 July 2021
Ehlers-Danlos syndromes
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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
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)