Cross-fused dystopia of the right kidney with cystic dysplasia of the non-functioning left kidney associated with ureterocele

G. I. Kuzovleva, Veronika V. Rogozhina, O. Staroverov, E. Ayryan, N. Кhvatynets, V. V. Rostovskaya
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Abstract

Cross-dystopia is a rare type of congenital anomaly, characterized by a displacement of the kidney to the opposite side, as a result of which both of them are located on the same side and in about 85% of such cases, a fusion of the parenchyma of two kidneys can be observed. We have not found descriptions of cases from practice when an orthotopic cystic dysplastic kidney with a lack of function associated with the pathology of the ureterovesical segment (ureterocele) was fused with the lower pole of a cross-dystopian normally formed kidney in literature.The patient, 8 d.o., a preliminary diagnosis - agenesis of the right kidney, doubling of the left kidney, cystic dysplasia, doubled left kidney whiyh ureterohydronephrosis lower half and ureterocele. In order to restore the outflow of urine and function of the lower half of the presumably doubled left kidney, a cystourethroscopy was performed. In a typical place on the right there is a correctly formed ureteral orifice, ureterocele on the left, which occupies half volume of bladder. With a holmium laser, an artificial orifice was formed in the ureterocele in order to restore the urine passage. At 9 m.o., tomography urinary tract was performed - cross-dystopia of the right kidney with fusion of the lower pole with a cystic dysplastic orthotopic left kidney.Laparoscopic nephrureterectomy of a non-functioning orthotopic kidney was performed.The control examination indicate complete clinical remission and social adaptation of the patient after discharge. Cross-dystopia of the kidney with fusion with cystic dysplastic kidney and a formed ureterocele is a rare congenital anomaly that requires timely examination in a specialized clinic and drawing up an individual treatment plan. The surgical manual should be focused on a symptomatic urological problem with an emphasis on preserving kidney function.
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右肾交叉融合异位伴左肾囊性发育不良伴输尿管膨出
交叉异位是一种罕见的先天性异常,其特征是肾脏向另一侧移位,因此两个肾脏位于同一侧,在约85%的此类病例中,可以观察到两个肾脏的实质融合。我们没有在文献中发现与输尿管膀胱段(输尿管精索细胞)病理相关的功能缺失的原位囊性发育不良肾与正常形成的跨反乌托邦肾的下极融合的病例描述。患者8岁,初步诊断为右肾发育不全,左肾加倍,囊性发育不良,左肾加倍,下半部分输尿管积水和输尿管膨出。为了恢复尿液的流出和左肾下半部分的功能,进行了膀胱输尿管镜检查。在典型的右侧,有一个正确形成的输尿管口,左侧是输尿管膨出,占膀胱体积的一半。利用钬激光,在输尿管囊肿中形成一个人工孔口,以恢复尿液通道。上午9点,行尿路断层扫描-右肾交叉异位伴下极囊性发育不良左肾融合。腹腔镜下肾输尿管切除术的功能不正常的原位肾。对照检查显示患者出院后临床完全缓解,社会适应能力较好。肾交叉异位合并囊性发育不良肾并形成输尿管囊肿是一种罕见的先天性异常,需要及时到专科诊所检查并制定个体化治疗方案。手术手册应侧重于有症状的泌尿系统问题,并强调保留肾功能。
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