Intern, RAK Medical and Health Sciences University, Ras Al Khaimah, UAE.
Emirates Health Service, Dubai, UAE.
J Med Case Rep. 2024 Mar 25;18(1):126. doi: 10.1186/s13256-024-04438-x.
Menstruation is a developmental milestone and usually marks healthy and normal pubertal changes in females. Menarche refers to the onset of first menstruation in a female. The causes of primary amenorrhea include outflow tract abnormalities, resistant endometrium, primary ovarian insufficiency, and disorders of the hypothalamus, pituitary, or other endocrine glands. A rare variant of mullerian agenesis, which warrants an individualized approach to management, is presented here.
We present here the case of a 25-year-old Indian female with pain in the lower abdomen and primary amenorrhea. After a thorough history, clinical examination, imaging, and diagnostic laparoscopy, two small uteri, a blind upper half vagina, bilateral polycystic ovaries, and a blind transverse connection between the two uteri-a horseshoe band cervix-were detected, which confirmed the diagnosis of mullerian agenesis. There was evidence of adenomyosis in the mullerian duct element. This is a rare form of Müllerian abnormality with an unusual presentation.
Mullerian agenesis is the most common cause of primary amenorrhea with well-developed secondary sexual characteristics. There are various forms of mullerian agenesis. Most of the cases are managed by a multidisciplinary team. Rare variants warrant an individualized approach to management.
月经是女性发育的一个里程碑，通常标志着其青春期的健康和正常变化。初潮是指女性第一次月经来潮。原发性闭经的原因包括流出道异常、子宫内膜抵抗、原发性卵巢功能不全以及下丘脑、垂体或其他内分泌腺的疾病。这里介绍一种罕见的中肾管发育不全的变异，需要个体化的管理方法。
我们在此报告一例 25 岁印度女性，有下腹痛和原发性闭经。经过详细的病史询问、临床检查、影像学检查和诊断性腹腔镜检查，发现两个小子宫、一个盲端上半阴道、双侧多囊卵巢和两个子宫之间的横向盲连接——马蹄形宫颈，确诊为中肾管发育不全。中肾管成分有腺肌病的证据。这是一种罕见的中肾管异常形式，表现不典型。
中肾管发育不全是最常见的有良好第二性征的原发性闭经原因。有各种形式的中肾管发育不全。大多数病例由多学科团队管理。罕见的变异需要个体化的管理方法。
