腹腔鏡下性腺摘除術を施行した完全型アンドロゲン不応症の1例

抄録

  We present a case of complete androgen insensitivity syndrome (CAIS) treated by laparoscopic gonadectomy. Case: A 19-year-old phenotypical female, who complained of primary amenorrhea, was referred to our hospital. Physical examination revealed the absence of the proximal vagina and female external genitalia. Pelvic magnetic resonance imaging revealed bilateral pelvic masses, and the absence of both the uterus and the vagina. Hormonal examination revealed elevated serum testosterone level at 8.3 ng/mL (normal values in female adults, 0.06-0.86 ng/mL). Her karyotype was 46,XY. Therefore, we diagnosed complete CAIS. She underwent laparoscopic gonadectomy. Histopathological examination showed immature seminiferous tubules and Leydig cells in both gonads. Her postoperative course was uneventful and she was discharged on postoperative day 5. She is being treated with and will continue to receive hormone replacement. Care for patients with CAIS needs to be individualized, flexible, and holistic because they are often raised socially as women. Laparoscopic gonadectomy in patients with CAIS is useful, because it is less invasive and has improved cosmetic results compared with open surgery.