Wünsch L, Hübner U, Halsband H
Department of Pediatric Surgery, Medizinische Universität zu Lübeck, Germany.
Pediatr Surg Int. 2000;16(7):493-7. doi: 10.1007/s003830000414.
Single-system ureteral ectopia (UE) encompasses a spectrum of malformations involving the bladder trigone, ureter, and kidney. The clinical presentation is variable, and both diagnostic and therapeutic problems are common. Reduced renal function in these patients may result from primary dysplasia, obstruction, vesicoureteral reflux, or recurrent infection. Based on our experience of seven patients, suggestions for diagnostic procedures and criteria for renal saving versus nephrectomy are offered. The relationship between ostium localization, renal function, and long-term results was investigated. From 1972 to 1990, five female and two male patients were studied. During the same period, 31 patients with UE and duplex kidneys were seen. Ages ranged from 1 day to 7 years. A ureteric opening into the bladder neck was associated with dilatation of the ureter and renal pelvis. Two patients had vaginal ectopia and severe renal dysplasia. In one, a cyst of the vaginal wall (Gardner's cyst) was detected at birth. A male newborn had multicystic renal dysplasia on the left and ureteric ectopia to the ductus deferens on the right side. To our knowledge, he is the first patient reported with renal function totally dependent on a kidney with severe UE. Follow-up ranged from 4 to 9 years. One patient died in the postoperative period because of renal failure and sepsis. All the others are well and have normal creatinine values. Improvement of renal function was noted after ureteral reimplantation (URI) in patients with bladder-neck ectopia. The numbers of infections were also drastically reduced. Our observations suggest that the combination of ultrasound, cyst urethrography, and cystoscopy will be diagnostic in most patients. A suspicion of UE should be raised in symptomatic patients with apparently solitary kidneys, enuresis ureterica, or atypical obstructive uropathy. Reduced renal function in some patients with ectopia to the bladder neck will improve after URI. This may be of importance in patients with bilateral anomalies and marginal renal function.
单系统输尿管异位（UE）包括一系列涉及膀胱三角区、输尿管和肾脏的畸形。临床表现多样，诊断和治疗问题都很常见。这些患者肾功能减退可能源于原发性发育异常、梗阻、膀胱输尿管反流或反复感染。基于我们对7例患者的经验，提出了诊断程序建议以及保留肾脏与肾切除术的标准。研究了输尿管口位置、肾功能和长期结果之间的关系。1972年至1990年，对5例女性和2例男性患者进行了研究。同一时期，还诊治了31例患有UE和重复肾的患者。年龄范围从1天至7岁。输尿管开口于膀胱颈与输尿管及肾盂扩张有关。2例患者有阴道异位和严重肾发育异常。其中1例在出生时检测到阴道壁囊肿（加德纳囊肿）。1例男性新生儿左侧为多囊性肾发育异常，右侧输尿管异位至输精管。据我们所知，他是首例报道的肾功能完全依赖于患有严重UE的肾脏的患者。随访时间为4至9年。1例患者术后因肾衰竭和败血症死亡。其他所有患者情况良好，肌酐值正常。膀胱颈异位患者输尿管再植术（URI）后肾功能有所改善。感染次数也大幅减少。我们的观察结果表明，超声、膀胱尿道造影和膀胱镜检查相结合对大多数患者具有诊断价值。对于有明显孤立肾、输尿管性遗尿或非典型梗阻性尿路病症状的患者，应怀疑UE。一些膀胱颈异位患者的肾功能减退在URI后会改善。这对于双侧异常和边缘肾功能患者可能很重要。
