Ehlers Danlos
Background
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A type of connective tissue disorder
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Characterized by skin hyperextensibility, joint hypermobility, & tissue fragility
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Classified into 13 subtypes: classical, classical-like, cardiac-valvular, vascular, hypermobile, arthrochalasia, dermatosparaxis, kyphoscoliotic, Brittle Cornea syndrome, spondylodysplastic, musculocontractural, myopathic, periodontal
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Vascular type associated with ↑ risk of death
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Considerations
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Multisystem disease:
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High risk of bleeding & friable tissue → affects ETT placement, regional techniques, invasive lines
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Desmopressin useful if bleeding
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Cardiovascular: mitral regurgitation, proximal aortic dilatation, conduction abnormalities
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Spontaneous ruptures: bowel, uterus, or major arteries
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Skin laxity/fragility & joint hypermotility
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↑ risk pneumothorax: keep low airway pressures
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Regional anesthesia is relatively contraindicated due to hematoma risk
Pregnancy Considerations
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Very difficult management, requires multidisciplinary effort
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High risk of preterm labor, uterine rupture, & hemorrhage
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Neuraxial relatively contraindicated, may need GA
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If vascular type, recommendations are either termination of pregnancy or cesarean section before 32 weeks
References
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Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J, Bloom L, Bowen JM, Brady AF, Burrows NP, Castori M, Cohen H, Colombi M, Demirdas S, De Backer J, De Paepe A, Fournel-Gigleux S, Frank M, Ghali N, Giunta C, Grahame R, Hakim A, Jeunemaitre X, Johnson D, Juul-Kristensen B, Kapferer-Seebacher I, Kazkaz H, Kosho T, Lavallee ME, Levy H, Mendoza-Londono R, Pepin M, Pope FM, Reinstein E, Robert L, Rohrbach M, Sanders L, Sobey GJ, Van Damme T, Vandersteen A, van Mourik C, Voermans N, Wheeldon N, Zschocke J, Tinkle B. The 2017 international classification of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017 Mar;175(1):8-26. doi: 10.1002/ajmg.c.31552. PMID: 28306229.