Mathews R, Jeffs R D, Maizels M, Palmer L S, Docimo S G
Division of Pediatric Urology, James Buchanan Brady Urological Institute, Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.
J Urol. 1999 Apr;161(4):1297-300.
Ureteral ectopia is frequently associated with dysplasia of the associated renal segment in girls with ureteral duplication. However, single system ureteral ectopia is an uncommon anomaly more frequently noted in boys. We report on 6 boys with single system ureteral ectopia into the prostatic urethra above the verumontanum, who presented with radiological and clinical findings of bladder outlet obstruction.
Antenatal ultrasound in 3 boys demonstrated renal abnormalities and postnatal studies suggested the diagnosis of posterior urethral valve obstruction. Older boys presented with symptoms suggestive of bladder outlet obstruction.
An ectopic ureter inserting into the prostatic urethra was noted in all 6 boys. The distal ureter was dilated elevating the bladder neck causing outlet obstruction. Surgical management consisted of nephrectomy and transurethral endoscopic incision of the distal ureter or nephroureterectomy with reconstruction of the prostatic urethra. In 2 younger boys voiding dysfunction with inability to empty developed.
Single system ectopic ureters in boys may present with symptomatic and radiological findings resembling posterior urethral valves. Surgical treatment should include nephroureterectomy with reconstruction of the hypoplastic prostate. Some patients may have later voiding dysfunction.
在重复输尿管的女孩中，输尿管异位常与相关肾段的发育异常有关。然而，单系统输尿管异位是一种罕见的异常情况，在男孩中更常见。我们报告6例单系统输尿管异位至精阜上方前列腺尿道的男孩，他们表现出膀胱出口梗阻的影像学和临床特征。
3例男孩产前超声显示肾脏异常，产后检查提示后尿道瓣膜梗阻诊断。年龄较大的男孩表现出提示膀胱出口梗阻的症状。
所有6例男孩均发现异位输尿管插入前列腺尿道。远端输尿管扩张抬高膀胱颈导致出口梗阻。手术治疗包括肾切除术、经尿道内镜下远端输尿管切开术或肾输尿管切除术并重建前列腺尿道。2例年龄较小的男孩出现排尿功能障碍且无法排空膀胱。
男孩的单系统异位输尿管可能表现出与后尿道瓣膜相似的症状和影像学特征。手术治疗应包括肾输尿管切除术并重建发育不全的前列腺。部分患者后期可能出现排尿功能障碍。
