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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 Yucky collagen EDS Types Type I (gravis) severe Type II (mitis) mild Type III (hypermobile) joints Type IV (vascular) acrogenic facies Type V (x-linked) moderate EDS Types Type VI (ocular scoliotis) Type VII (arthrochalasis Type VIII (peridontitis) Type IX (fibronectin) scoliosis, detached retina micronathia tooth loss stria 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 EDS & Women’s Health I Preadolescent (<12-15y) II Reproductive age group (15-40y) III Peri and post menopause(>40y) Preadolescent Diagnosis can be difficult Age of symptoms – 6yo Age of diagnosis – 9yo Diagnosis Major Criteria Translucent skin Arterial, intestinal, uterine rupture Bruising Acrogenic facies Diagnosis Minor Criteria Tendon or muscle rupture Clubbed feet Early varicose veins Early pneumothorax Gingival recession + family history Preadolescent Diagnosis Procollagen III deficiency Stain biopsy – COL3AI mutation Preadolescent Contact sports Accident avoidance Joint bracing Contraception Injury handling Reproductive Age Contraception 50% vertical transmission Endometriosis Pregnancy planning Poor wound healing Pregnancy & EDS Type IV advise against Close multidisciplinary f/u 30 weeks antenatal steroids Early delivery ?1o c/s Perimenopause Postmenopause Pelvic organ prolapse Stress urinary incontinence Dyspareunia Mesh vs. no mesh