儿童重复肾上肾输尿管膨出合并下肾膀胱输尿管反流的治疗体会

目的探讨儿童重复肾上肾输尿管膨出合并下肾或对侧肾膀胱输尿管反流合理的治疗方法。方法回顾分析2007年10月至2019年10月首都医科大学附属北京儿童医院收治的重复肾上肾输尿管膨出合并下肾或对侧肾膀胱输尿管反流24例患儿的临床资料。其中,男5例,女19例,就诊年龄7个月至9岁,平均2岁。重复肾左侧13例,右侧6例,双侧5例,上肾输尿管膨出左侧15例,右侧9例,无双侧,合并下肾膀胱输尿管反流左侧13例,右侧9例,下肾及对侧2例。泌尿系统感染17例,影像学检查发现6例,间断腹痛1例。上半肾无功能者22例行重复肾上半肾切除术;上半肾积水但有功能者2例,行上下输尿管吻合术1例,行上肾输尿管膨出切除上下输尿管膀胱再植术1例。结果 22例半肾切除患儿中19例患儿获得随访,3例失随访,术后下半肾反流消失11例(含对侧反流1例),减轻3例,反流无缓解无临床表现2例(术前Ⅳ级随访4个月,术前Ⅴ级随访2年),3例术后反流未消失合并泌尿系统感染再次手术治疗后治愈。保留上肾2例中,行上下输尿管吻合者术后4个月,无临床症状,尚未复查排尿性膀胱尿道造影,行上肾输尿管膨出切除上下肾输尿管膀胱再植术后复查反流消失无临床...     

小儿重复肾畸形的早期诊断和治疗

目的 探讨小儿重复肾畸形的早期诊断和治疗方法.方法 回顾性分析本院1995年6月-2007年9月60例经手术治疗的小儿重复肾畸形病例的临床资料.60例均行B超检查,确诊55例,误诊为肾上极囊肿5例.60例患儿均行静脉肾盂造影(IVP)检查,均提示重复肾,其中2例上下肾部均有不同程度的肾盂积水和输尿管扩张,进一步行排尿性膀胱尿道造影,诊断下肾部膀胱输尿管返流、下肾部输尿管膀胱交接部梗阻各1例.结果 重复肾畸形患儿60例均行手术治疗,行上肾部切除及其输尿管切除58例,重复肾并上下肾部积水手术2例,其中1例下肾部输尿管末端梗阻致下肾部积水,上肾部输尿管囊肿,行上肾部及其输尿管切除,术后输尿管囊肿萎陷,下肾部输尿管末端梗阻解除,症状消失,术后6个月复查IVP,下肾部肾盏杯口清晰,无肾积水,肾功能良好,疗效满意;另1例为下肾部输尿管返流致下肾部积水,上肾部输尿管囊肿,行输尿管囊肿切除,双输尿管膀胱再植术,术后6个月复查排尿膀胱尿道造影,膀胱输尿管返流消失,排尿正常,无泌尿系感染.结论 术前诊断不明或误诊的原因多系患儿临床症状与其他疾病症状相类似而误诊为其他疾病,应强调对可疑征象的辅助检查.如重复肾畸形诊断明确,应早期手术治疗.     

重肾双输尿管畸形下肾积水的临床处理

目的 探讨小儿少见的重肾双输尿管畸形下肾部积水功能受损病例的临床特点及治疗方法.方法 报告2004至2007年间我院收治的4例重肾双输尿管畸形下肾部积水功能受损病例,男3例,女1例,年龄为6个月至10岁,其中下肾部并发肾盂输尿管交界部梗阻性肾积水者3例,1例完全型双输尿管者作下肾离断性肾盂成形;另1例不完全型双输尿管者作下肾部肾盂与上输尿管端侧吻合术;下肾部肾巨大积水功能丧失1例作下肾切除.不完全型双输尿管畸形下肾部并发反流积水功能受损1例,作共同通道段输尿管膀胱再植术.结果 经术后6～18个月(平均12个月)随访,经B超、利尿性肾图(DR)及排尿性膀胱尿道造影(VCUG)检查,2例下肾部并发.肾盂输尿管交界部梗阻息儿积水缩小,肾功能提高,1例下肾部反流患儿反流消失.结论 重肾双输尿管畸形下肾部积水,功能受损病例较少见,为取得良好的手术治疗效果则术前需根据不同病例进行仔细地个性化评价.     

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。     

重肾双输尿管合并肾盂输尿管交界部梗阻性肾积水的诊治分析

目的 探讨重肾双输尿管合并肾盂输尿管交界部梗阻性肾积水的诊断与处理方法。方法 回顾分析1986～2004年间收治重肾合并肾盂输尿管交界部梗阻肾积水8例的临床资料。男2例，女6例，年龄7个月～10岁，平均4．8岁。病变位于左侧3例，右侧5例；上肾积水2例，下肾积水5例，上下肾积水1例；3例为重肾完全型双输尿管，5例重肾Y型输尿管。结果 3例重肾完全型双输尿管中，上肾积水1例因肾实质薄无功能行上肾切除术，下肾积水伴上肾输尿管膨出症1例行上肾切除下肾离断性肾盂成形术，另1例下肾积水因临床症状轻微，IVP示积水半肾的肾盏变钝不明显，未行手术门诊随诊。5例重肾Y型输尿管中，上肾积水1例行上肾盂与下输尿管吻合，下肾积水3例行上肾输尿管下肾盂吻合 下肾离断性肾盂成形术，1例上下肾均积水行上下肾盂吻合 下肾盂成形术。术后3～6个月复查IVP肾积水明显好转。结论 重肾肾盂输尿管交界部梗阻肾积水发病率很低，术前不容易明确诊断或被误诊。腹部B超、IVP或MRU是有效的辅助检查手段。治疗应根据息肾功能、形态而定，如息肾功能严重受损，行患肾切除，反之，根据积水的部位、输尿管的形态选择手术方式。     

重复肾上肾切除术后上肾输尿管末端膨出的转归分析

目的分析上肾功能差的重复肾合并输尿管膨出患儿, 行上肾输尿管切除术后, 输尿管膨出的转归情况。方法回顾性分析1998年9月至2019年6月山东第一医科大学附属省立医院收治的重复肾合并上肾输尿管末端膨出的32例患儿临床资料。所有患儿经后腹腔镜入路, 行重复肾上肾输尿管切除术, 随访其术后输尿管膨出的变化、并发症及再手术情况, 分析输尿管膨出的转归情况。结果最终仅24例患儿获得随访, 获访患儿的手术年龄为(23.3±18.9)个月, 范围在3～81个月;随访时间为(85.4±34.8)个月。其中19例术后超声检查提示输尿管膨出消失, 4例输尿管膨出较术前缩小, 1例输尿管膨出较术前无明显变化。术后共发生并发症5例:1例输尿管膨出自尿道外口再次脱出行手术治疗;2例输尿管膨出感染, 经抗生素治疗后好转;2例术后出现泌尿系感染, 排尿期膀胱尿道造影显示下肾输尿管反流。1例反流为Ⅳ级, 同时合并上肾输尿管残端的感染, 行残存输尿管膨出切除及下肾输尿管膀胱再植术;另外1例反流为Ⅱ级, 目前在观察中。结论对于上肾功能差的重复肾合并输尿管膨出患儿, 行上肾输尿管切除术后, 输尿管膨出多数会消失或缩小至无...     

输尿管端侧吻合术耦合输尿管膀胱再植术治疗儿童完全性重复肾畸形

目的评价采用输尿管端侧吻合术耦合输尿管膀胱再植术治疗儿童完全性重复肾畸形的疗效。方法回顾性分析2010年1月至2018年12月采用输尿管端侧吻合术耦合输尿管膀胱再植术治疗的37例同侧双输尿管病变的完全性重复肾畸形患儿的临床资料。其中,男4例,女33例;患侧为左侧25例,右侧12例。临床症状中,尿路感染34例,滴尿2例,排尿困难1例。病理类型中(同一患儿不限于一种病理类型),上肾部输尿管异位开口11例,上肾部输尿管异位开口伴反流3例,上肾部输尿管囊肿26例,上肾部输尿管囊肿伴反流6例,下肾部输尿管反流26例,下肾部输尿管异位开口伴狭窄11例。分析手术时间、肾盂分离前后径(anterior and posterior diameter,APD)、输尿管直径、患肾分肾功能(differential renal function,DRF)、并发症等,评价这种术式的优缺点。结果所有患儿均顺利完成同侧输尿管端侧吻合术耦合输尿管膀胱再植术,平均手术年龄为8.3个月,范围为4～110个月,手术时间为(128.84±35.06)min。术后上肾部APD为(9.41±6.40)mm,术后上肾部输尿管直径为...     

不完全性重复肾双输尿管畸形的诊治及文献复习

目的探讨不完全性重复肾双输尿管畸形的诊断与处理方法。方法回顾性分析2004年10月至2008年8月夺院收治的3例Y形输尿管病例的临床资料。男2例,女1例。病变位于左侧2例,右侧1例。结果3例不完全性重复肾双输尿管畸形中,1例为左不完全性重复肾双输尿管畸形,下肾部肾盂和输尿管连接部狭窄,Y形输尿管汇合于近下肾部肾盂和输尿管连接处。行下肾部肾盂与上输尿管端侧吻合术。1例为有不完全性输尿管重复畸形,有膀胱输尿管返流Ⅲ～Ⅳ级,Y形输尿管汇合于输尿管中下段,行有输尿管膀胱再植术。1例为左不完全性重复肾双输尿管畸形,下肾部肾盂和输尿管连接部狭窄,Y形输尿管汇合于输尿管中部,行下肾部离断式肾盂成形术。术后随访6,15个月,症状均明显好转。结论不完全性重复肾双输尿管畸形发病率低,术前不容易明确诊断或被误诊。腹部B超、IVP、DR或CTU是有效的辅助检查手段。治疗应根据患肾功能、形态等多种因素选择手术方式。     

单纯上或下患肾部输尿管膀胱外再植治疗完全性重复肾畸形

目的 评价采用单纯上或下患肾部单根输尿管膀胱外再植治疗完全性重复肾畸形的疗效.方法 从2009年12月1日起至2014年12月31日间,我科采用单纯上或下患肾部单根输尿管膀胱外再植治疗完全性重复肾畸形患儿27例,均为单侧病变,女19例,男8例,年龄2个月至8岁.所有患儿经影像学检查明确诊断为完全性重复肾畸形且均为单根输尿管病变.其中上输尿管异位开口者9例;重复上肾上输尿扩张积水伴上输尿管末端囊肿者10例;重复下肾部输尿管单纯反流者8例.临床症状主要表现为正常排尿间歇性滴尿或发热性尿路感染.所有患儿术后随访观察11个月至6年,随访项目包括泌尿系统超声、同位素和膀胱造影等.结果 所有患儿手术过程顺利,平均手术时间63 min,术后平均住院时间为5d,术后所有患儿临床症状均消失.术后有1例患儿因血尿检查发现再植输尿管开口处结石形成,予以行膀胱镜下钬激光碎石术.术后6个月复查超声示患肾部积水均有好转,同位素示患肾部功能改善或稳定,正常肾部功能未见异常改变.7例患儿术后复查排泄性膀胱尿道造影检查未见膀胱输尿管反流等发生.结论 经腹股沟皮纹小切口单纯上或下患肾部单根输尿管膀胱外再植术治疗完全性重复肾畸形是一种安全、有效的方法,值得临床推广.     

小儿输尿管异位开口的诊治

目的 探讨小儿输尿管异位开口的诊断及治疗。方法 回顾总结16例小儿输尿管异位开口的诊治。结果 一侧重复肾上肾段输尿管开口9例，其中7例行重复肾上肾段及输尿管切除，2例行异位输尿管与膀胱抗反流移植术；单侧单一输尿管异位开口7例，均行患肾及输尿管切除术；16例术后滴尿症状消失。结论 本病一经确诊，应及时手术治疗。IVP与B超检查相互补充，有助于异位输尿管开口的定侧及手术进路，手术方法依双肾功能而定。     

Bladder surgery as first-line treatment of complete duplex system complicated with ureterocele

ObjectivesWe retrospectively analyzed our experience to determine the optimum management of complete duplex system complicated with ureterocele.Materials and methodsBetween 1994 and 2003, we reviewed 15 patients treated with bladder surgery for complete duplex system complicated with ureterocele. The associated anomalies were seven vesicoureteral reflux (VUR) of the lower pole ureter and one of both poles. All patients had ureterocele (11 intravesical, 4 extravesical) and a functioning upper moiety. Initial treatment was transurethral incision of ureterocele (TUI) (14) or common sheath reimplantation (1). The median follow-up was 30 (13–48) months.ResultsThere were no requirements for secondary procedures, with a significant improvement or conservation of ipsilateral renal function and no reflux, in 10 patients (67%). Five patients (33%) with ureterocele (1 intravesical, 4 extravesical) who initially underwent TUI required additional operative management due to moderate to severe VUR, recurrent urinary tract infection or decreased function of upper moiety. The secondary operation performed was lower pole nephrectomy with ureteral reimplantation (1), ureteroureterostomy with ureteral reimplantation (2) or common sheath reimplantation (2). One patient who underwent common sheath reimplantation as secondary operation needed a nephrectomy due to breakthrough febrile urinary tract infection and decreased renal function.ConclusionTUI is recommended as the first-line treatment of complete duplex system with intravesical ureterocele and well-conserved renal function.     

Incidence of urinary tract infection in neonates with antenatally diagnosed ureteroceles.

PURPOSE: To evaluate the incidence of neonatal urinary tract infection (UTI) in the presence of a ureterocele. PATIENTS AND METHODS: Fifteen successive patients with antenatally detected ureteroceles were reviewed between 1991 and 1996. Sixteen ureteroceles were treated (in one patient they were bilateral). Seven ureteroceles were intravesical, and nine were ectopic. Fourteen patients had a duplex upper tract associated with a ureterocele. One patient had one duplex upper tract and one simple upper tract, both associated with a ureterocele. RESULTS: Of the fifteen neonates with a ureterocele, eight already had a UTI on admission. Of these, three had had prophylactic antibiotics. Of the eight infected patients, seven were symptomatic (three with septicaemia). CONCLUSION: If diagnosed antenatally, obstruction has to be relieved as early as possible during the first week of life as antibiotic prophylaxis alone is not sufficient to avoid UTI in these patients. The authors point out the high frequency of UTI in patients with ureterocele.     

Management of duplex ureteroceles detected by prenatal ultrasound

During the last 13 years 110 children with duplex ureteroceles were treated. There was a 3 to 1 female-to-male predominance. The authors report their experience with a group of 25 patients with duplex ureteroceles diagnosed in utero. Endoscopic incision of the ureterocele was performed as the initial treatment in 18 (72%) of these patients; decompression of the upper pole of the ureterocele was achieved in 100% of cases. Preservation of the upper pole was possible in 8 patients (44%); vesicoureteral reflux was created at the level of the upper pole in 8 (44%). Reconstruction of the lower urinary tract was accomplished in 15 (83.3%) patients who had had endoscopic treatment. Our experience has led us to believe that endoscopic incision of the ureterocele deserves reconsideration and re-evaluation because, as our series has shown, this intervention offers the great advantage of allowing good functional recuperation of the obstructed moiety with a high rate of success in a totally asymptomatic group of patients diagnosed in utero.     

The role of minimal surgery with renal preservation in abnormal complete duplex systems

Among 36 children, mostly less than 1 year of age, referred for a complete duplex-system anomaly, 18 were diagnosed before birth by obstetrical ultrasonography where a diagnosis of dilated upper urinary tract was the most frequent report; in the remaining cases urinary tract infection was the main presenting symptom. Five children had bilateral anomalies. Principal diagnoses associated with the complete duplex system were: 18 ureteroceles (UC) (11 extravesical [EUC] with bladder-neck or urethral extension, 7 intravesical [IUC]), 5 ectopic ureters without UC and 28 lower-polar vesicoureteral reflux (VUR) associated in 6 with upper polar VUR. The initial assessment was based on a voiding cystogram and radionuclide scan. Renal-polar function was severely impaired when major ureteral ectopia or severe primary reflux was present. Primary surgery was performed in 8 patients, demolitive in 4 (3 upper-polar nephrectomies, 1 nephrectomy) and reconstructive in 4 (duplex en-bloc reimplantation); staged management with minimal endoscopic incision was undertaken in 15 UCs (9 EUCs and 6 IUCs). Expectant management was elected in all cases of mild primary, single, or double VUR not associated with UC and was followed by spontaneous reduction in one-half of the cases. Secondary VUR complicated endoscopic incision in 2 of 6 IUCs and 5 of 9 EUCs; a certain degree of functional improvement after decompression could be observed in all IUCs versus only 2 EUCs. Twenty-one patients requested secondary surgery; 17 needed an open intravesical procedure for ureteral reimplantation, combined in 8 with UC excision and bladder-floor reconstruction and in 5 with upper-pole nephrectomy. A nephrectomy was required in 4 cases. All primary or secondary demolitive procedures involved 9 of 11 EUCs extravesical and 2 of 5 ectopic ureters. EUCs and ureteral ectopia were associated with severe renal-polar damage, and function was rarely affected by primary decompressive procedures even in prenatally detected, uninfected cases. Endoscopic incision of EUCs was frequently followed by secondary VUR, which made secondary intravesical operations more complex. For these reasons, primary elective resection of a dysplastic upper pole is preferable in most cases to temporary decompressive measures. Conservative surgery is always indicated in IUC, which may benefit from endoscopic decompression. Isolated VUR involving the lower pole of a completely duplicated system may respond to expectant management in a significant number of cases. Accepted: 5 January 1999     

Experience with a novel approach to upper-pole nephrectomy and partial ureterectomy

The upper-pole moiety of a renal duplication anomaly associated with an ectopic ureter or ureterocele is often hydronephrotic and non-functioning. Thus, the treatment of choice is frequently upper-pole nephrectomy and partial ureterectomy. Previous surgical techniques have emphasized the initial removal of the upper pole followed by partial ureterectomy. The approach described here begins by identifying and dissecting the obstructed upper-pole ureter, dividing it distally, and performing the partial nephrectomy last. We have performed this procedure on 12 consecutive nonfunctioning and obstructed upper-pole moieties of duplicated kidneys. In 10 patients the obstructed upper pole was associated with an ectopic ureter or an ectopic ureterocele (bilateral in 1) and in 1 with uretero-pelvic junction obstruction of the upper-pole moiety. This anatomic approach has resulted in preservation of function in all 12 remaining lower renal and ureteral units as assessed by postoperative intravenous urography. Only 1 patient required an intraoperative blood transfusion. We believe that this safe and easy-to-teach approach should be included in the urologic armamentarium.     

Familial ureteroceles: an evidence for genetic background?

In the pediatric population, ureteroceles may present with different clinical pictures, and the severity of the renal damage is greater than in adults. Ureterocele, an anomaly of ureteric budding, is likely a component of a spectrum of anomalies including vesicoureteral reflux and ureteral duplications. Both have been confirmed to have a genetic and familial basis. We document the largest series of familial cases of ureteroceles, giving evidence for genetic background. We retrospectively reviewed the charts of patients with familial ureteroceles seen between 1992 and 2002. Coexisting ureteral anomalies and features of the cases were documented and compared to sporadic cases and all familial cases within the literature. This is the largest series of familial ureterocele patients in the literature. The review of the literature revealed seven publications with seven ureterocele families (15 affected patients) between 1936 and 2002. Comparing sex, ureterocele location, and single versus duplex systems, familial series are similar to other sporadic cases. Three of the families have twin siblings with ureteroceles. Familial cases, despite their rarity, raise the issue of the genetic origin of uretereoceles. Family members of ureterocele cases should be informed and followed carefully, especially twins. Increased reporting and genetic analysis of familial ureteroceles may prove to link the genetic mouse models of abnormal ureteric budding to the human conditions.     

Refluxing ureteral reimplantation: A logical method for managing neonatal UVJ obstruction

ObjectiveAn obstructed megaureter can be managed using a number of techniques, with the primary goal being to minimize the potential for further injury to the affected kidney. Classically, these obstructed ureters have been treated using cutaneous ureterostomy. However, this technique has certain limitations including the potential for stenosis. We describe our experience with the refluxing ureteral reimplantation as a novel, yet technically simple, method for temporary internal diversion of the obstructed megaureter.MethodsTreatment consists of transecting the ureter proximal to the obstruction and performing an end-to-side anastomosis with the bladder in a freely refluxing fashion. Patients are placed on antibiotic suppression following surgery. Subsequent open definitive surgery is performed through the same incision site once the child is older than 1 year of age.ResultsSixteen patients identified with severe hydroureteronephrosis were found to have an obstructed megaureter(s) involving 19 ureteral moieties. Patients underwent internal diversion at an average age of 5 months. All patients demonstrated improved drainage of the affected kidney(s) following surgery. Three patients developed a febrile urinary tract infection. Definitive surgical treatment was undertaken in 18 of 19 ureters, and consisted of ureteral reimplantation with tapering or plication (13), ureteral reimplantation without tapering (3), and nephrectomy (2). One patient with multiple other congenital anomalies is not a candidate for further genitourinary reconstruction.ConclusionsRefluxing ureteral reimplantation is a safe and easy method of temporary internal urinary diversion. Simple in principle, the concept of creating a refluxing ureteral reimplantation is no different from that of incising an obstructing ureterocele. This technique allows time for the child to mature, while preserving renal function and awaiting definitive repair.     

输尿管镜钬激光治疗儿童输尿管末端囊肿的疗效分析

目的：探讨输尿管镜钬激光治疗儿童输尿管末端囊肿的疗效。方法回顾性分析2010年3月至2014年3月本院22例儿童输尿管末端囊肿临床资料,均经B 超、泌尿系CT、静脉尿路造影、膀胱逆行造影检查确诊,并接受输尿管镜钬激光囊肿切开术。22例患儿年龄3个月至15岁,平均3.6岁,其中女性15例（68.2％）,男性7例（31.8％）;左侧9例（40.9％）,右侧11例（50.0％）,双侧2例（9.1％）;囊肿开口于膀胱内20例（90.9％）,开口于膀胱颈尿道内口2例（9.1％）;16例（72.7％）合并患侧肾积水或者输尿管扩张,7例（31.8％）合并重复肾畸形,且均为上下位肾输尿管Y形融合并以末端囊肿开口于膀胱内,4例（18.2％）伴有膀胱输尿管反流,1例（4.5％）合并输尿管末端结石,13例（59.1％）合并尿路感染;均行输尿管镜钬激光囊肿切开术,手术时间为14～46 min,平均（28±10）min,术中出血量2～10 mL,住院时间2～5 d,平均（3.1±1.0）d。结果术后21例随访,1例失访,随访时间为3～31个月,20例未见输尿管囊肿复发,10例尿路感染症状完全消失,12例肾积水或者输尿管扩张得到明显改善,1例合并结石的未见复发,1例出现膀胱输尿管反流加重而行输尿管膀胱再植术。结论输尿管镜钬激光治疗儿童输尿管末端囊肿操作简便,创伤小,手术时间短,恢复快,并发症少,可作为首选治疗方式;对于严重膀胱输尿管反流,异位输尿管开口的重复肾患儿,术前需要做充分评估,以减少再次手术的风险。