双子宫-半阴道-肾发育不全综合征

定义 英文原文（暂无中文）

A rare congenital urogenital anomaly characterized by the presence of double uterus (didelphys, bicornuate or septum-complete or partial), unilateral cervico-vaginal obstruction (obstructed hemivagina-communicant, not communicant or septate and unilateral cervical atresia) and ipsilateral renal anomalies (renal agenesis and/or other urinary tract anomalies). Patients are usually diagnosed at puberty after menarche due to recurrent severe dysmenorrhea, chronic pelvic pain, excessive foul smelling mucopurulent discharge, spotting and intermenstrual bleeding (depending on the existence of uterine or vaginal communications). Fever, dyspareunia, and a palpable abdominal, pelvic or vaginal mass (mucocolpos or pyocolpos) may also be present.

别名

梗阻性半阴道-同侧肾发育不全综合征

基本事实

遗传方式

未知

发病年龄

产前、婴儿期、新生儿期

患病率

<1 / 1 000 000

临床表型 12

外部标识与链接