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Women’s Healthcare and Ehlers Danlos

Women’s Healthcare and Ehlers Danlos. Frank N. H. Harrison, Jr., MD Department OB/GYN Carolinas Medical Center Charlotte, NC. Ehlers Danlos Syndrome (EDS). A rare connective tissue disorder characterized by tissue fragility, translucent skin and joint hypermobility. EDS.

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Women’s Healthcare and Ehlers Danlos

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  1. Women’s HealthcareandEhlers Danlos Frank N. H. Harrison, Jr., MD Department OB/GYN Carolinas Medical Center Charlotte, NC

  2. Ehlers Danlos Syndrome (EDS) • A rare connective tissue disorder characterized by tissue fragility, translucent skin and joint hypermobility

  3. EDS • Yucky collagen

  4. EDS Types • Type I (gravis) severe • Type II (mitis) mild • Type III (hypermobile) joints • Type IV (vascular) acrogenic facies • Type V (x-linked) moderate

  5. EDS Types • Type VI (ocular scoliotis) scoliosis, detached retina • Type VII (arthrochalasismicronathia • Type VIII (peridontitis) tooth loss • Type IX (fibronectin) stria

  6. Collagen • A fibrous protein found in connective tissue • Includes 19 proteins encoded by 35 nonallelic genes scattered over 15 chromosomes • TNXB gene  tenasion-X  Type III • Provides structure, strength & flexibility to tissue

  7. EDS & Women’s Health • I Preadolescent (<12-15y) • II Reproductive age group (15-40y) • III Peri and post menopause (>40y)

  8. Preadolescent • Diagnosis can be difficult • Age of symptoms – 6yo • Age of diagnosis – 9yo

  9. Diagnosis Major Criteria • Translucent skin • Arterial, intestinal, uterine rupture • Bruising • Acrogenic facies

  10. DiagnosisMinor Criteria • Tendon or muscle rupture • Clubbed feet • Early varicose veins • Early pneumothorax • Gingival recession • + family history

  11. PreadolescentDiagnosis • Procollagen III deficiency • Stain biopsy – COL3AI mutation

  12. Preadolescent • Contact sports • Accident avoidance • Joint bracing • Contraception • Injury handling

  13. Reproductive Age • Contraception • 50% vertical transmission • Endometriosis • Pregnancy planning • Poor wound healing

  14. Pregnancy & EDS • Type IV advise against • Close multidisciplinary f/u • 30 weeks antenatal steroids • Early delivery ?1o c/s

  15. Perimenopause - Postmenopause • Pelvic organ prolapse • Stress urinary incontinence • Dyspareunia • Mesh vs. no mesh

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