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Bilateral congenital midureteric stricture

机译:双侧先天性输尿管中段狭窄

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Midureteric stricture is a rare cause of congenital hydronephrosis. Its etiology and pathophysiology is uncertain. We report a case of an 8 year old male child who presented with recurrent urinary tract infections and moderate renal impairment. Sonar revealed bilateral hydronephrosis. This was confirmed to be due to intrinsic midureteric narrowing on MRU. Bilateral ureteric exploration confirmed these findings and uretero-ureterostomy was performed. Postoperative imaging showed improvement in hydronephrosis. We review the published literature. Case report An 8 year old boy was admitted to our institution with a documented UTI. He had no significant past medical history. Physical examination was non-contributory. He was in moderate renal impairment with a serum Creatinine of 130mmol/l and an estimated Creatinine Clearance, using the Schwartz formula, of 50ml/min/m?. Urine cultures for Tuberculosis were negative.Renal sonar revealed gross bilateral hydroureteronephrosis with thin renal cortices. The bladder appeared normal. Of note no ureteric dilatation was seen at the level of the bladder. An MCUG revealed a normal bladder and posterior urethra with no reflux. Radioisotopic mercaptoacetyltriglycine (MAG3) renography showed poor uptake bilaterally with apparent holdup at the midureteric level.In view of the unusual imaging and to exclude extrinsic retroperitoneal pathology a Gadolinium enhanced magnetic resonance urogram was requested. This showed no retroperitoneal pathology, but bilateral midureteric obstruction. (See Figure 1).
机译:输尿管中段狭窄是先天性肾积水的罕见原因。其病因和病理生理尚不确定。我们报告了一例8岁男孩,该男孩表现出反复尿路感染和中度肾功能不全。声纳显示双侧肾积水。证实这是由于MRU固有的输尿管中部狭窄。双侧输尿管探查证实了这些发现,并进行了输尿管-输尿管造口术。术后影像显示肾盂积水改善。我们回顾了已发表的文献。病例报告一名8岁的男孩被录入了我们的机构,并记录了UTI。他没有重要的既往病史。身体检查是无贡献的。他处于中度肾功能不全,血清肌酐为130mmol / l,使用Schwartz公式估算的肌酐清除率为50ml / min / m?。结核病的尿培养阴性,肾脏声纳显示双侧严重输尿管肾盂肾病,肾皮质薄。膀胱看起来正常。值得注意的是,在膀胱水平未见输尿管扩张。 MCUG显示膀胱和尿道后部正常,无反流。放射性同位素巯基乙酰基三甘氨酸(MAG3)肾电图显示双侧摄取不良,在输尿管中段明显滞留。鉴于影像学异常,并排除外在性腹膜后病理,要求使用enhanced增强磁共振尿路造影。这表明没有腹膜后病理,但双侧输尿管中段梗阻。 (请参见图1)。

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