In female patients with pure gonadal dysgenesis (PGD, Swyer syndrome, 46 XY karyotype), prophylactic gonadectomy is recommended because of the malignant potential of streak glands. We herein present a case of PGD whose uterus was safely preserved during prophylactic laparoscopic gonadectomy with use of hormone replacement therapy (HRT).
A 15-year-old female visited our hospital complaining of an absence of menarche. She had female-type external genitalia, however, the corpus uteri and bilateral ovaries were not identified on MRI. Hormonal tests showed hypogonadism and chromosome analysis revealed a 46 XY karyotype. Accordingly, she was diagnosed with PGD. After the diagnosis, HRT was started at the age of 16. One month later, the uterus was markedly enlarged, and the uterine body and endometrium became visible on MRI. Prophylactic laparoscopic gonadectomy was performed at the age of 17. Intraoperatively, the fallopian tubes and streak gonads were located at normal sites of the fallopian tubes and ovaries. Although the uterine size was small, it was easily identified. The fallopian tubes and gonads were safely removed without any uterine damage by power sources, because appropriate distances from the uterine body were secured.
Successful pregnancies were reported in patients with PGD using donated eggs. HRT, even for a short period of time, may be advantageous to preserve fertility because the enlarged uterine body enables safe gonadectomy.
