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Swyer Syndrome/46 XY Gonadal Dysgenesis: Remove the Tubes or Not?

  • Mary E. Fallat
    Correspondence
    Address correspondence to: Mary E. Fallat, MD, 315 East Broadway Street, Suite 565, Louisville, KY 40202. Phone (502) 629-8638; fax (502) 583-9735.
    Affiliations
    Hiram C. Polk, Jr Department of Surgery, University of Louisville, Louisville, Kentucky

    Norton Children's Hospital and Norton HealthCare, Louisville, Kentucky
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  • Paige Hertweck
    Affiliations
    Department of Obstetrics, Gynecology, and Women's Health, University of Louisville, Louisville, Kentucky

    Norton Children's Hospital and Norton HealthCare, Louisville, Kentucky
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  • Sigal Klipstein
    Affiliations
    University of Chicago, Pritzker School of Medicine, Chicago, Illinois

    InVia Fertility Specialists, Chicago, Illinois
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      Pediatric and adolescent gynecologists and pediatric surgeons often struggle with the question of whether to perform salpingectomy at the time of gonadectomy for gonadal dysgenesis with high risk of malignancy. The insights from Anwar and colleagues
      • Anwar A
      • Akhtar M
      • Busby G
      Swyer Syndrome: A case of dysgerminoma solely within the fallopian tube.
      provide a strong argument in favor of universal salpingectomy at the time of gonadectomy.
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      Linked Article

      • Swyer Syndrome: A Case of Dysgerminoma Solely within the Fallopian Tube
        Journal of Pediatric and Adolescent GynecologyVol. 34Issue 6
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          46XY pure gonadal dysgenesis (Swyer syndrome) is a rare disorder of sexual development. Patients have a 46XY karyotype, though phenotypically they appear female with normal external genitalia and vagina. Although patients exhibit normal Müllerian structures (uterus, fallopian tubes, and vagina), they possess a pair of bilateral undifferentiated gonad streaks. Delayed puberty and primary amenorrhea are the common presentations. There is an increased risk of developing tumors in the gonads and therefore a bilateral gonadectomy is recommended.
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