Abstract

In this case report, we present a 43-year-old man (XY) with azoospermia and typical male appearance, at Tanner stage 5 of sexual development, who presented with severe colicky abdominal pain accompanied by nausea. A pelvic CT scan revealed a pear-shaped structure in the pelvic cavity, located entirely behind the bladder, measuring 106*44cm with fluid accumulation inside it, extending into the right inguinal canal. There was also evidence suggesting the formation of the upper and mid-third part of a vagina, terminating in the prostatic gland. The patient underwent laparoscopic surgery for the removal of the uterus and the left gonad. The patient had a uterus with hematometra and a blind vaginal pouch measuring 4 centimeters at the end of the uterus, extending posteriorly behind the bladder to the apex of the prostate, containing old blood. Hormonal analysis showed serum estradiol < 5.0 pmol/L (11-44pg/mL), free testosterone at 1.57 ng/ mL(male reference range: 2.5-20 ng/mL), testosterone at 0.56 ng/mL (2.27-10.30),FSH at 44.8 mIU/L (0.95-11.95 mIU/L), LH at 20.4 mIU/L(0.57-12.07), and DHEA-SO4 at 199.0µg/mL (139.7-484.4µg/mL). Currently, the patient is under the care of a urologist and is receiving weekly treatment with hCG medication. He reports normal sexual function, including intercourse, orgasm, erection, and ejaculation.

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