膀胱尿道子宫阴道完全重复畸形一例报告及文献复习

Complete duplication of bladder,urethra,vagina and uterus in a woman:a case report and a review of the literature

目的提高对膀胱、尿道、子宫、阴道完全重复畸形的诊断和治疗水平。方法总结1例膀胱尿道子宫阴道完全重复畸形临床诊治经过并复习文献。结果麻醉下膀胱尿道镜检查和手术探查确诊为完全双膀胱、双尿道。行膀胱纵隔完全切除,保留完整的膀胱颈部,双膀胱壁吻合形成一个正常大小膀胱。病理检查示逼尿肌和双面膀胱上皮组织。双尿道、阴道及子宫未做处理。随访5年,无排尿困难,偶有双尿线,右肾7.3cm×3.9cm,右肾、输尿管无扩张,残余尿30～50ml。国内报道5例成年双膀胱,但均不是膀胱完全重复畸形。无女性生殖道的详细资料。结论膀胱、尿道、子宫、阴道完全重复畸形少见。重复膀胱若出现尿潴留、上尿路积水可试行纵隔切除并密切随访。

Objective To improve the capability in the diagnosis and treatment of very rare cases with complete duplication of bladder,urethra,vagina and uterus. Methods Our experience with a 24 - year - old unmarried female with complete duplication of bladder, urethra, vagina and uterus and right hydronephrosis was presented and the related literatures were reviewed. Results Under spinal anesthesia,two bladders of normal inside appearance were corfirmed by Cystourethro scopy and normal sphincter muscle circle were obserued in both urethras. The following operative exploration showed that the two bladders were separated by a detrusor septum and each had a normal bladder neck and received its ipsilateral ureter. The septum was thoroughly excised with attention to the outlet maintenance. The remaining parts of two bladders were anastomosed to make a bladder with two urethras. In 5 years of follow - up,she became more and more healthy without malaise and difficulty in urination. The right kidney showed slightly smaller without any dilation in the right pelvis and ureter. The residual urine was between 30ml and 50ml. Conclusions Complete duplication of the bladder may be formed by a sagittal septum. When there is an obstruction to upper urinary tract,removal of the bladder septum may increase the functional bladder volume and reduce residual urine in the dysfunctional bladder. The management of double urethra, vagina and uterus could be delayed until necessary, such as the development of increased residual urineand difficulty in intercourse and infertility.