Gakiya M, Nakai H, Higuchi A, Shishido S, Kawamura T
Department of Urology, Tokyo Metropolitan Children Hospital.
Hinyokika Kiyo. 1995 Jan;41(1):69-72.
Duplicated uterus associated with unilateral imperforate vagina and ipsilateral renal agenesis is a very rare anomaly. We report a case of a 10-month-old female infant presenting with pus discharge from vagina, and discussed the embryologic and clinical features relevant to this interesting disease complex with a review of the past literature. Echographic and CT examinations demonstrated a right-sided cystic pelvic mass. IVP revealed a left slight hydronephrosis and non-visualizing right kidney. No right ureteral orifice was found at cystoscopy. Under anesthesia the mass and pus was aspirated. Under the diagnosis of Gartner's cystic duct and a right renal agenesis or dysplasia with or without ectopic ureter, we subsequently performed laparotomy. Surgical exploration revealed a duplicated uterus with a normal ovary, and the mass was an imperforate right-sided vagina which communicated through the uterus. The diagnosis was changed to a double uterus with right-sided imperforate vagina, then the vaginal septum was excised. The post-operative course was uneventful, and pus discharge and the left hydronephrosis have disappeared.
双子宫合并单侧阴道闭锁及同侧肾缺如是一种非常罕见的异常情况。我们报告一例10个月大的女婴，其出现阴道脓性分泌物，并结合既往文献复习，讨论了与这一有趣的疾病复合体相关的胚胎学和临床特征。超声和CT检查显示右侧盆腔囊性肿块。静脉肾盂造影显示左侧轻度肾积水，右侧肾脏不显影。膀胱镜检查未发现右侧输尿管口。在麻醉下抽吸肿块及脓液。在诊断为加特纳囊肿管及右侧肾缺如或发育异常伴或不伴异位输尿管的情况下，我们随后进行了剖腹手术。手术探查发现双子宫及正常卵巢，肿块为右侧闭锁阴道，通过子宫相通。诊断改为双子宫伴右侧阴道闭锁，然后切除阴道隔膜。术后过程顺利，脓性分泌物及左侧肾积水消失。
