影像学诊断子宫双重化合并中肾管发育异常

Imaging diagnosis of uterus duplication combined with renal dysplasia

目的观察子宫双重化合并中肾管发育异常的影像学特征。方法回顾性分析17例子宫双重化合并中肾管发育异常患者的影像学资料,其中7例接受腹盆腔CT检查,10例接受MR检查。结果 17例均以子宫双重化合并单侧肾-输尿管发育不良为基础病变,根据其是否伴有其他相关病变分为5类:(1)7例仅有基础病变,其中双子宫合并单侧肾-输尿管缺如5例,CT诊断1例、MR诊断4例;2例双角子宫和并单侧肾-输尿管发育不良,CT、MR各诊断1例;(2)2例基础病变合并输尿管异常,CT、MR各1例,均表现为双子宫畸形以及单侧发育不良的低位肾,输尿管异位开口于阴道;(3)5例阴道斜隔并单侧肾-输尿管发育异常(Herlyn-Werner-Wunderlich综合征),CT 3例,MR 2例,表现为双子宫畸形、阴道斜隔侧近端腔内积血并单侧肾-输尿管缺如;(4)2例基础病变并中肾管残留囊肿,1例CT表现为双子宫畸形、单侧肾-输尿管缺如,同侧中肾管残余囊肿形成,1例MRI表现为Herlyn-Werner-Wunderlich综合征同时出现与阴道相通的中肾管残余囊肿;(5)1例基础病变并肾源性肾瘤,MRI表现为双子宫畸形、左侧肾-输尿管缺如、膀胱左后壁肿瘤并子宫内膜异位。结论以子宫双重化合并单侧肾-输尿管发育不良为核心,根据其他相关病变的复杂程度,将子宫双重化并中肾管发育异常类疾病分为五类,有助于认识本病及分科诊疗。

Objective To observe imaging features of uterus duplication combined with mesonephric duct dysplasia related abnormalities.Methods Imaging data of 17 female with uterus duplication and renal dysplasia were retrospectively analyzed.Seven patients underwent CT and 10 patients underwent MR scanning.Results The basic abnormalities in 17 patients were uterus duplication combined with unilateral renal-ureter dysplasia with/without other associated malformation which were further classified as follows：（1） 7 patients presented as basic abnormalities without other associated malformations,include 5（CT diagnosed 1,MR diagnosed 4）with uterus duplex and unilateral renal-ureter agenesis,2（CT 1,MR 1）with uterus bifidus and unilateral renal-ureter dysplasia;（2）2 patients（CT 1,MR 1）presented as basic abnormalities accompanied with ectopic ureter orifice,uterus duplex and unilateral renal dysplasia with ectopic ureter orifice opening in vagina;（3）5 patients（CT 3,MR 2）of Herlyn-Werner-Wunderlich syndrome presented as uterus duplication,oblique vagina septum as well as unilateral renal-ureter dysplasia,which shown as uterus duplex,oblique vaginal septum with hematocolpos,also unilateral renal-ureter agenesis;（4）2 patients presented as basic abnormalities accompanied with mesonephric residual cyst,CT showed one with uterus duplex and unilateral renal-ureter agenesis with ipsilateral mesonephric residual cyst, MRI showed as the other one with Herlyn-Werner-Wunderlich syndrome combined with mesonephric residual cyst opening in vagina;（5）MRI showed 1 patient with basic abnormalities and a nephrogenic adenoma,which demonstrated as uterus duplex combined with left renal-ureter agenesis,and a tumor on the left wall of bladder accompanied with endometriosis.Conclusion Further classification into 5 types from simple to complex based on uterus duplication combined with unilateral renal-ureter dysplasia with/without other associated malformations is helpful to the diagnosis and treatment of these abnormalities.