Askary Elham, Fakharmoghadam Fatemeh, Dehghanian Amirreza, Shamohammad Iman, Alborzi Saeed, Poordast Tahereh, Mahmoodi Samaneh, Alamdarloo Shaghayegh Moradi
Department of Obstetrics and Gynecology, School of Medicine, Maternal-Fetal Medicine Research Center, Shiraz University of Medical Sciences, Obstetrics and Gynecology Office, Shahid Faghihi Hospital, Zand Avenue, Shiraz, 7134844119, Iran.
Department of Molecular Pathology & Cytogenetics, Department of Pathology, School of Medicine, Trauma Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Hormones (Athens). 2025 Mar;24(1):207-215. doi: 10.1007/s42000-024-00610-6. Epub 2024 Oct 31.
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*44 cm 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 199.0 µg/mL (139.7-484.4 µg/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.
在本病例报告中，我们介绍了一名43岁男性（XY），患有无精子症，具有典型男性外观，处于性发育的坦纳5期，出现严重的绞痛性腹痛并伴有恶心。盆腔CT扫描显示盆腔内有一个梨形结构，完全位于膀胱后方，大小为106×44厘米，内部有积液，并延伸至右侧腹股沟管。还有证据表明形成了阴道上三分之二和中三分之一部分，止于前列腺。患者接受了腹腔镜手术以切除子宫和左侧性腺。患者有一个积血的子宫，子宫末端有一个4厘米长的盲袋阴道，向后延伸至膀胱后方至前列腺尖部，内有陈旧血液。激素分析显示血清雌二醇<5.0 pmol/L（11 - 44 pg/mL），游离睾酮为1.57 ng/mL（男性参考范围：2.5 - 20 ng/mL），睾酮为0.56 ng/mL（2.27 - 10.30），促卵泡生成素为44.8 mIU/L（0.95 - 11.95 mIU/L），促黄体生成素为20.4 mIU/L（0.57 - 12.07），硫酸脱氢表雄酮为199.0 μg/mL（139.7 - 484.4 μg/mL）。目前，该患者在泌尿科医生的照料下，每周接受人绒毛膜促性腺激素药物治疗。他报告性功能正常，包括性交、性高潮、勃起和射精。
