完全型アンドロゲン不応症に対する性腺摘出術

抄録

  Complete androgen insensitivity syndrome (CAIS) is caused by genetic defects associated with vaginal and uterine agenesis in women with a 46,XY karyotype. These defects cause the body to be unable to respond to androgen. CAIS is associated with abnormal testicular development, and gonadectomy is generally recommended in early adulthood to avoid the risk of germ cell malignancy. The procedure is managed laparoscopically if the gonads are intra-abdominal. However, there has been no report of a detailed method of laparoscopic gonadectomy in a CAIS patient with inguinal canalicular gonads. Herein, we report a case of CAIS in siblings. In case of the elder sibling, the palpable inguinal canalicular gonads were removed via inguinal incision. In the case of the younger sibling, the right palpable inguinal canalicular gonad and the left unpalpable inguinal canalicular gonad were removed through laparoscopic gonadectomy. The surgical procedures for laparoscopic gonadectomy are described.