Abstract：
Persistent Müllerian Duct syndrome is a rare male disorder of sexual development. The phenotypically and genotypically male patient presents with female internal organs (i.e., uterus, cervix, fallopian tubes and upper part of vagina) due to deficiency of anti-mullerian hormone or insensitivity of tissues to Anti Mullerian Hormone. We present a 19 year old male who came with complaint of right iliac fossa pain. He was investigated for acute appendicitis and on imaging, he was diagnosed to have bilateral cryptorchidism with rudimentary uterus. Computed tomography followed by pelvic ultrasonography was done which indicated two testes in abdomen and a soft tissue density structure, identified as a rudimentary uterus located posterior to the urinary bladder. CT scan findings were further confirmed by magnetic resonance imaging pelvis. A trial of stepwise orchidopexy followed by orchidectomy with removal of rudimentary uterus was performed laparoscopically. Additionally, he was counselled for long term sex hormone replacement and reproductive failure in future.
摘要：
持续性苗勒管综合征是一种罕见的男性性发育障碍。表型和基因型男性患者表现为女性内部器官(即，子宫,子宫颈,输卵管和阴道上部）由于抗苗勒管激素缺乏或组织对抗苗勒管激素不敏感。我们介绍了一名19岁的男性，他主诉右髂窝疼痛。他接受了急性阑尾炎和影像学检查，他被诊断为双侧隐睾，子宫不完整。进行了计算机断层扫描，然后进行了盆腔超声检查，发现腹部有两个睾丸，软组织密度结构，被确定为位于膀胱后方的原始子宫。磁共振成像骨盆进一步证实了CT扫描的结果。腹腔镜下进行了逐步进行睾丸固定术，然后进行睾丸切除术并切除原始子宫的试验。此外,他被建议长期性激素替代和将来的生殖失败。
