先天性单侧肾发育不良合并输尿管异位开口27例报道

目的 总结先天性单侧肾发育不良合并输尿管异位开口的诊治经验。方法 对27例先天性单侧肾发育不良合并输尿管异位开口患儿的临床表现、诊治方法进行回顾性分析。结果 27例患儿均为女性。左侧18例，右侧9例。经B超、静脉尿路造影（IVU）、阴道逆行造影、CT、MRI等检查证实，17例输尿管异位开口于阴道壁，3例开口于尿道与阴道口之间。16例发现发育不良的肾脏。27例患儿均接受了发育不良肾及输尿管切除术。结论 先天性单侧肾发育不良合并输尿管开口异位通过病史、体格检查及影象学检查，可于术前得到确诊，手术切除发育不良的肾脏及输尿管，疗效满意。     

先天性单侧肾发育不良合并输尿管异位开口的诊治体会

目的 探讨先天性单侧肾发育不良合并输尿管异位开口的临床特点和诊治方法。方法 总结分析12例肾发育不良合并输尿管异位开口的临床资料。结果 12例肾发育不良均为女性。左侧7例，右侧5例，主要临床表现为点滴性尿失禁，静脉尿路造影患肾均不显影，对侧肾显示代偿性肥大。B超探查8例未探及肾脏，仅4例在盆腔探及发育不良肾组织，其中4例接受CT扫描。2例提示盆腔有发育不良肾，12例均接受了发育不良肾及输尿管切除，术中见肾脏体积小，形态及位置异常，术后“尿失禁”症状立即消失。结论 先天性肾发育不良女孩多见，常伴有输尿管开口异位，主要表现为点滴性尿失禁，B超多不能探及患肾，静脉尿路造影患肾均不显影而对侧肾肥大，易误诊为孤立肾。手术切除患肾及输尿管效果确切。     

先天性异位肾伴输尿管开口异位的诊断和治疗(附7例报告)

目的探讨先天性异位肾伴输尿管开口异位的诊断和外科治疗。方法回顾性分析7例异位肾伴输尿管异位开口的临床症状和体征及外科治疗方法。结果7例中6例有漏尿病史，1例腹部可扪及包块，1例可见输尿管异位开口，6例美蓝试验阳性。影像学检查中B超发现4例发育不良的异位肾，IVU检查有3例异位肾显影，可见输尿管行程，CT、CT增强检查可定位7例异位肾和输尿管的位置。外科治疗中行输尿管膀胱再植手术5例，肾切除手术1例，保守治疗1例。术后随访0．5—7年肾功能均有不同程度的改善，漏尿症状消失，无肾积水。结论典型的病史、仔细的体查结合美蓝试验、IVU检查是诊断的基础，B超可作为筛选手段，CT、CT增强可作为常规的确诊检查。外科手术是治疗异位肾伴输尿管异位开口的主要方法。     

小儿单侧异位肾发育不良伴输尿管开口异位6例

目的探讨小儿先天性单侧异位肾发育不良伴输尿管开口异位的诊治经验。方法回顾性分析本院收治的6例先天性单侧异位肾发育不良伴输尿管开口异位患儿的临床资料。结果 6例均为女性,术前均经彩超检查确诊和定位,均予患侧发育不良肾及输尿管切除手术,术后滴尿症状均消失。结论先天性异位肾发育不良合并输尿管异位开口临床较少见,超声为首选检查方法。治疗上应行发育不良肾及输尿管切除术。     

先天性单侧肾发育不良的诊治体会(附23例报告)

目的总结先天性单侧肾发育不良的诊治经验。方法回顾性分析23例先天性单侧肾发育不良的临床资料。其中女20例,男3例。年龄2～14岁。合并肾位置异常2例,输尿管异位开口15例,双侧输尿管囊肿1例,输尿管积水3例,膀胱输尿管返流(VUR)1例。对侧重复肾并输尿管异位开口2例,膀胱输尿管返流1例。B超检查21例,IVP检查22例,输尿管逆行造影4例,膀胱镜检查10例,CT检查13例,VCU检查2例。均行手术治疗,其中肾切除19例;输尿管囊肿电切开窗1例;对侧重复肾切除2例;膀胱输尿管再吻合1例。结果全部病例随访6个月～3年,漏尿、发热及尿路感染等症状消失。结论临床症状结合影像学检查是诊断先天性单侧肾发育不良的有效方法,单侧肾发育不良常合并输尿管末端及对侧肾脏病变。治疗上主要是切除发育不良的肾。     

先天性输尿管开口异位26例

目的探讨小儿先天性输尿管开口异位的诊断和治疗方法。方法回顾性分析本院1995年2月至2008年2月收治的26例先天性输尿管开口异位病例资料。其中18例为单侧重复肾、重复输尿管并输尿管开口异位，1例为双侧重复肾、重复输尿管并单侧输尿管开口异位，5例为先天性肾发育不良并输尿管开口异位，2例为单侧肾异位伴发育不全输尿管开口异位。结果均经手术治疗，其中16例行上肾部输尿管切除术；1例行患肾上肾部及输尿管切除术；2例行输尿管膀胱再植术；5例肾发育不良和2例异位肾并肾发育不全输尿管开口异位患儿行患肾及输尿管切除术。26例均痊愈出院，获6个月至1年的随访，滴尿症状均消失，2例术后半年行膀胱尿道造影未见膀胱输尿管返流，疗效满意。结论先天性输尿管开口异位易误诊或漏诊，B超、静脉肾盂造影、磁共振泌尿系统水造影是诊断的主要手段；诊断明确者应尽早手术治疗。     

小儿输尿管囊肿的诊断与治疗

目的探讨小儿输尿管囊肿诊断与治疗的方法及效果。方法输尿管囊肿39例进行总结分析。病例均行B超和静注尿路造影（IVU）检查。IVU不显影者行CT检查14例，行MRI检查8例。行膀胱镜检查10例。结果13超确诊31例，IVU确诊17例。行CT、MRI、膀胱镜检查者均得到确诊。输尿管囊肿39例中行上肾段及输尿管切除26例。行囊肿切除加输尿管膀胱移植术7例，经尿道行囊肿电切开窗术6例。并发症：膀胱输尿管返流3例，囊肿边缘出血、输尿管残端感染各1例。结论B超适于囊肿的筛选。IVU可了解肾脏功能及畸形情况。CT、MRI对IVU不显影有帮助。膀胱镜对了解囊肿大小、位置起确诊作用。囊肿侧肾发育不良或伴严重积水者行上肾段及输尿管切除；囊肿大者宜行囊肿切除加输尿管膀胱移植术，较小囊肿可经尿道电切开窗术。     

彩色多普勒显像诊断先天异位肾伴输尿管异位开口6例报告

近几年来,我院应用彩色多普勒显像技术诊断先天异位肾伴输尿管异位开口病6例,均已经手术病理证实,现报告如下。 临床资料 6例病变均为单侧。男1例,女5例;年龄2岁至6岁。初诊以自幼起除正常排尿外有滴尿、湿裤为主诉。6例均作IVP检查,1例示肾轴旋转并肾发育不全,其余均未显影,5例作CT检查,肾缺如3例;肾发育不良且移位2例,其中3例作同位素检测,异位肾功能差2例,异位肾无功能1例。6例均作普通B超检查未探及患肾。利用彩色多普勒技     

14例先天性单侧肾发育不良的临床研究

目的　发育不良肾可位于肾窝或盆腔内 ,可无症状 ,如伴有输尿管异位开口可致尿失禁。该文主要探讨先天性单侧肾发育不良的临床特点、诊疗方法以及病理分型。方法　回顾总结 14例单侧肾发育不良患儿的临床资料。其中女 10例 ,男 4例。左侧 11例 ,右侧 3例。主要临床表现为滴尿、反复尿路感染以及腹部包块等。术前均行静脉尿路造影 (IVU)、B超检查 ,7例行CT检查。术前确诊 9例。结果　 14例均采用手术治疗 ,术后所有症状消失。术中见发育不良肾脏的体积、形态和位置异常。 9例为体积较小的肾脏 ,5例为葡萄状发育不良肾。病理表现为镜下大量未成熟的扩张导管 ,异常排列的未成熟肾小球和肾小管。术后随访 1～ 2年 ,生长发育正常。结论　先天性单肾发育不良女孩多见 ,因无特异性临床表现 ,不易确诊。临床上影像学检查十分重要 ,手术治疗效果确切。     

螺旋CT及三维重建尿路成像在肾发育不良诊断中的应用

目的 寻觅诊断小儿肾发育不良最好的检查方法。方法 对7例正常排尿的间歇期有会阴部滴尿的患儿，均经超声、单光子发射计算机断层肾动态显像（SPECT）、静脉尿路造影、（IVU）和CT尿路成像（CTU）检查。并对照术后诊断，比较CTU、IVU、超声、SPECT对肾发育不良的诊断符合率。结果 7例患儿的发育不良肾CTU均清楚显示，左侧3例，右侧4例。增强后实质期图像示病变侧腰大肌前方明显强化的肾样小结节，分泌期图像可显示集合系统及输尿管腔，MPR、3D、4D像直观、立体，显示了发育不良肾的大小、位置、轮廓及其输尿管走行。CTU诊断符合率100％。ECT检查仅1例显示发育不良肾，诊断符合率14.3%(1/7)。IVU、B超均未能显示发育的肾和输尿管。CTU的诊断符合率明显高于SPECT、IVU及B超。结论 如疑有肾发育不良或经超声初步检查仅发现一个肾，应行CTU检查，以明确是否为单侧肾发育不良。CTU是目前诊断肾发育不良最准确的非创伤性影像检查方法。     

Single-system ectopic ureters associated with renal dysplasia

The purpose of this study was to inquire into the clinical features and methods for the diagnosis and therapy of single-system ectopic ureters associated with renal dysplasia. Intravenous urography (IVP), diuretic B-mode ultrasound (B-US), and, in four cases, computerized tomography (CT) were performed in twelve female patients with renal dysplasia-seven on the left and five on the right-and the clinical records were retrospectively summarized and analyzed. The main presentation was droplet urinary incontinence. IVP and B-US showed that in all in whom the affected kidneys did not function, the contralateral kidneys had undergone compensatory hypertrophy. Only in four cases were the dysplastic renal tissues displayed in the pelvic cavity on B-US. In eight cases, the affected kidneys were not found. Of the four patients who had CT scanning, the dysplastic kidneys were explored in the pelvis in two cases. All patients underwent ureteronephrectomy, and the urinary incontinence disappeared after operation. Single-system ectopic ureters associated with renal dysplasia mainly affect female children, and most present with droplet urinary incontinence; the affected kidney may not be displayed on ultrasonography and IVP, but the contralateral kidney may display compensatory hypertrophy on IVP and can easily be misdiagnosed as a solitary kidney. The curative effect of ureteronephrectomy is definite.     

99mTe-DMSA用于诊断肾发育不良伴输尿管开口异位

目的 通过运用影像学手段诊断肾发育不良伴输尿管异位开口的经验，探讨这一疾病的最佳诊断方法。方法 回顾性分析14例肾发育不良伴输尿管开口异位的病史资料，比较各类影像学检查对异位肾诊断之优劣。结果 14例患儿异位开口位于阴道12例，膀胱颈2例。常用的影像学诊断方法包括：B超发现异位肾的阳性率为35．7％(5／14)、IVP18．2％(2／11)、CT0％(0／2)、MR100％(2／2)、而^99mTe—DMSA为100％(9／9)。结论 肾组织对放射性核素^99mTe-DMSA极为敏感，通过该检查能发现绝大部分发育不良的肾组织。对本病的诊断有重要意义。     

小儿先天性发育不良肾的临床特点和治疗

目的 探讨小儿先天性发育不良肾的临床特点、诊断和治疗.方法 总结分析1988年至2009年我科收治的83例单侧先天发育不良肾的临床资料.所有患儿术前均行多普勒超声、静脉肾盂照影和99mTc-DTPA肾脏扫描.35例患儿行增强CT检查,39例行IVP后延迟CT检查三维重建.所有病例均行发育不良肾切除,肾切除的指征包括无功能发育不良肾脏合并临床症状55例,无临床症状的无功能多囊发育不良肾,随访不消退或增大27例,无功能发育不良肾合并肾输尿管积水1例.结果 各项术前检查中IVP后延迟CT三维重建技术阳性诊断率最高,达100%.术后病理均提示为发育不良肾脏.4例因高血压行发育不良肾切除的患儿,术后2例仍然有高血压,其他患儿术后临床症状消失,没有恶变者.结论 儿童先天发育不良肾脏发育不良程度不一,合并畸形不同,临床表现各异.IVP后延迟CT检查三维重建技术诊断发育不良肾脏阳性率高.合并临床症状的无功能发育不良肾、多囊发育不良肾长期随访不消退者可手术治疗,随访过程中有明显消退的多囊发育不良可定期观察,有功能的非多囊发育不良肾可以保留.近19%发育不良肾脏可合并对侧肾脏畸形,应密切监测对侧肾脏功能及血压.     

小儿先天性发育不良肾的临床特点和治疗

目的 探讨小儿先天性发育不良肾的临床特点、诊断和治疗.方法 总结分析1988年至2009年我科收治的83例单侧先天发育不良肾的临床资料.所有患儿术前均行多普勒超声、静脉肾盂照影和99mTc-DTPA肾脏扫描.35例患儿行增强CT检查,39例行IVP后延迟CT检查三维重建.所有病例均行发育不良肾切除,肾切除的指征包括无功能发育不良肾脏合并临床症状55例,无临床症状的无功能多囊发育不良肾,随访不消退或增大27例,无功能发育不良肾合并肾输尿管积水1例.结果 各项术前检查中IVP后延迟CT三维重建技术阳性诊断率最高,达100%.术后病理均提示为发育不良肾脏.4例因高血压行发育不良肾切除的患儿,术后2例仍然有高血压,其他患儿术后临床症状消失,没有恶变者.结论 儿童先天发育不良肾脏发育不良程度不一,合并畸形不同,临床表现各异.IVP后延迟CT检查三维重建技术诊断发育不良肾脏阳性率高.合并临床症状的无功能发育不良肾、多囊发育不良肾长期随访不消退者可手术治疗,随访过程中有明显消退的多囊发育不良可定期观察,有功能的非多囊发育不良肾可以保留.近19%发育不良肾脏可合并对侧肾脏畸形,应密切监测对侧肾脏功能及血压.     

Spectrum of ectopic ureters in children

We reviewed the clinicopathological features, diagnosis, and surgical treatment of ectopic ureters (EU) in children in order to evaluate the anatomy, renal function, outcome, and the existence of any racial or regional difference in this abnormality. The records of 27 consecutive patients with 33 EU managed over a period of 7 years were analyzed. There were 25 female and two male patients. Their age ranged from 1 to 13 years, with an average of 4.5 years. The presenting features included history of continuous dribbling of urine with otherwise normal voiding in toilet-trained female patients, incontinence of urine, urinary tract infection, abdominal pain, and renal failure. The diagnostic work-up included ultrasonography (US) in all 27 patients, intravenous pyelography (IVP) in 23, micturating cystourethrogram (MCU) in 19, nuclear imaging (NI) in 16, and computed tomogram (CT) in five patients. All patients underwent examination under anesthesia (EUA) and cystourethrovaginoscopy immediately before the operation. Single-system ectopic ureter (SSEU) was present in 19 patients with 25 ureters (six bilateral), and duplex with ectopic ureter was present in eight cases. The left side was involved in 14 cases, the right side in seven, and the anomaly was bilateral in six cases. Abnormalities noted in the imaging studies were USG 23/27, IVP 23/23, MCU 11/19, NI 13/16, and CT 5/5 cases. EUA and cystourethrovaginocsopy helped in localization of the ectopic site and evaluation of the bladder. The surgical treatment included nephroureterectomy (NUT) for non-functioning kidney in 7 patients, heminephroureterectomy (HNUT) for non functioning upper pole of duplex kidney with EU in 6 patients, ureteric reimplantation (UTR) in 13 patients (19 ureters, 6 bilateral), and ureteropyelostomy (UTP) in one patient. In the follow-up period ranging from 1 to 5 years, 20 patients achieved continence; however, six cases with bilateral SSEU and one case of unilateral SSEU, who also had a patulous bladder neck, continued to dribble urine. SSEU was more common than duplex with ectopic ureter. A large number of functioning renal units associated with SSEU deserved preservation. The success of surgical treatment in terms of achievement of continence was high and depended on the integrity of the bladder neck.     

Single trocar retroperitoneoscopic nephrectomy for dysplastic poorly functioning kidney with ectopic ureter in children

This is the first report of a single trocar nephrectomy for poorly functioning dysplastic kidney and ectopic ureter, performed in 5 patients from 2010 to 2011. The mean operative time was 63 min and mean postoperative hospital stay was 31 h. There were no perioperative complications. The single trocar nephrectomy is a feasible and safe procedure for patients with dysplastic kidney and ectopic ureter.