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.     

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.     

重肾双输尿管合并下肾肾盂输尿管连接部梗阻性肾积水的诊疗分析

目的 探讨小儿重肾双输尿管合并下肾肾盂输尿管连接部梗阻性肾积水的临床特点及诊疗方法.方法 回顾性分析本院近10年来收治的190例重肾双输尿管畸形病例中,6例合并下肾肾盂输尿管连接部梗阻性肾积水患儿的临床资料,包括年龄、性别、临床症状、解剖结构及处理方法等.结果 重肾双输尿管畸形患儿190例,合并下肾肾盂输尿管连接部梗阻性肾积水6例,占3.16%.其中男5例,女1例,年龄10 d至3岁7个月,平均13.8个月.围产期行B超检查发现肾积水4例,泌尿系感染1例,腹部包块1例.病变位于左侧3例,右侧3例;3例为重肾完全型双输尿管,3例为重肾Y型输尿管,其中1例为右重肾Y型输尿管合并下肾肾盂输尿管连接部狭窄及下肾输尿管膀胱连接部狭窄.3例重肾完全型双输尿管病例中,1例行上组肾及输尿管切除＋下组肾离断性肾盂成形术,2例行下组肾离断性肾盂成形术;3例重肾Y型输尿管中,2例行上肾输尿管下肾盂端侧吻合＋下组肾离断性肾盂成形术,1例行上组肾及输尿管切除＋下组肾离断性肾盂成形术＋输尿管膀胱再植术.术后随访3~18个月,平均12个月,B超及IVP显示肾积水明显好转,无并发症.结论 重肾双输尿管合并下肾肾盂输尿管连接部梗阻性肾积水发病率低,易误诊.术前B超、IVP及MRU是有效的辅助检查手段.临床应根据患儿肾功能及解剖异常情况制定个体化的手术方案.     

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。     

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...     

小儿重复肾盂输尿管及其合并症

作者从70年6月到73年2月,行肾盂静脉造影检查了448例患儿,发现有重复肾盂输尿管畸形者19例,占4.2％。女性14例(73％),男性5例(27％)。完全重复畸形者4例,其中2例为一侧性,另2例一侧为完全性另一侧是不完全性。不完全重复畸形者14例(73％),其中13例为一则性,1例为双侧性。不明者1例。19例共38个肾,完全重复畸形者4个肾,不完全重复畸形者17个肾,不明者1个肾,正常者16个肾。 19例中有合并症者13例(68％),其中膀胱输尿管逆流者5例,肾盂积水或输尿管积水者5例,膀胱外开口2例,瘢痕性肾盂肾炎1     

完全性重肾合并膀胱下支输尿管反流2例

目的探讨重肾合并膀胱下支输尿管反流的临床表现和诊治方法。方法回顾性分析2013年1月至2013年7月我们收治的2例重肾合并膀胱下支输尿管反流患者的诊治经验。1例为女性,5个月,术前诊断为右重肾:上下肾积水,上输尿管扩张伴末端囊肿(伴反流);右下肾VURⅣ~Ⅴ级。1例为男性,4岁,术前诊断为右重肾:上肾输尿管积水,上输尿管末端开口异位(后尿道),下肾轻度积水;右下肾VURⅣ~Ⅴ级。结果 2例均行右上输尿管整形、右上下输尿管端侧吻合、右下输尿管膀胱再植术,手术顺利,术后随访均无尿路感染复发,影像学检查显示疗效良好,目前仍在随访中。结论重肾合并下支输尿管反流的手术治疗可参照单一集合系统膀胱输尿管反流的处理原则,但较单一集合系统膀胱输尿管反流更积极,如重肾功能可,则行上输尿管与下输尿管端侧吻合、下输尿管膀胱再植术,疗效确切。     

膀胱皮肤造口术在新生儿尿路畸形中早期应用探讨

目的探讨膀胱皮肤造口术在新生儿尿路畸形患儿中的早期应用。方法回顾性分析我院2003年1月至2006年6月因先天性尿路畸形行膀胱皮肤造口术的患儿资料,包括原发病、临床表现、影像检查、皮肤膀胱造口术后转归等。结果共13例新生儿,均为男婴,手术日龄16～28天,平均22天,其中后尿道瓣膜8例,前尿道瓣膜1例,双侧原发性膀胱输尿管反流4例。膀胱皮肤造口术后半年至1年复查,2例因皮肤膀胱造口狭窄改为膀胱造瘘,1例仍间断反复泌尿系感染,17侧膀胱输尿管反流中6侧反流消失,16侧肾输尿管积水中5侧消失,无肾、输尿管积水及膀胱输尿管反流加重病例。结论尿路畸形新生儿行皮肤膀胱造口术操作简单、易护理,可有效引流尿液,抑制膀胱输尿管反流,改善泌尿系感染,保护肾功能。     

56例小儿重复肾临床诊疗分析

目的探讨小儿重复肾的诊断与治疗方法,以进一步规范其诊疗流程。方法对遵义医学院附属医院近10年收治的小儿重复肾畸形56例的临床资料进行回顾性分析。对其临床表现、分型、诊断及治疗方法进行总结分析。结果 56例中重复肾重复输尿管无积水且IVU显影正常者11例,保守随访观察。重复肾发育不良、重复肾积水伴重复输尿管积水并输尿管异位开口、IVU不显影和SPECT检测GFR(肾小球滤过率)无功能35例,予以重复肾重复输尿管探查切除。重复肾功能尚存、重复输尿管扩张(直径大于4. 0 cm)、积水3例,予以重复肾与下位肾肾盂吻合成形术治疗;重复肾功能尚存、重复输尿管扩张(直径小于4. 0 cm)、积水伴输尿管末端囊肿5例(不伴膀胱输尿管反流),予以输尿管末端囊肿戳穿术治疗;不伴输尿管末端囊肿2例,予以重复输尿管膀胱再植术治疗。术后随访6个月至7年,除1例因合并骶脊膜膨出术致神经源性膀胱表现为尿失禁外,其余术前存在泌尿系感染的病例感染症状均消失。术前不存在泌尿系感染的病例术后亦未出现泌尿系新发感染,行输尿管囊肿戳破术及膀胱输尿管再植的病例术后未出现膀胱输尿管反流。结论小儿重复肾畸形临床表现多样,B超、IVU、SPECT、VCUG及泌尿系MRU检查是诊断的主要方法,膀胱镜输尿管插管逆行造影有一定的补充性诊断价值。治疗则应依据重复肾功能、重复肾重复输尿管有无积水以及是否合并重复输尿管异位开口,采用个体化治疗方案。     

The natural history of reflux in the lower pole of duplicated collecting systems: a controlled study

Thirty two children with reflux into the lower pole of duplicated collecting systems, followed non-operativelh for one to five years, were compared to a carefully selected control group of similar children who had reflux into a single collecting system. There were no significant differences between the two groups, either in the outcome of reflux or in the incidence of new renal scars. We conclude that reflux into the lower pole of a duplex kidney does not in itself constitute an indication for early surgical treatment.     

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.     

Ureterocele associated to duplex system: an individualized approach to endoscopic incision

BACKGROUND: Management of Ureterocele (UC) associated to duplex kidney depends from the type of UC (ectopic or intravesical), from the presence of symptoms and from associated vesicoureteral reflux (VUR) in the lower pole of the duplex system. Individualized approach to UC must consider endoscopic puncture as a possible option to reduce hydronephrosis and preserve function. METHODS: The records of 68 patients with duplex system have been reviewed. UC was detected in 27 renal units: it was intravesical in 11 and ectopic in 16. VUR was present in the lower pole in 16 cases. Patients were observed between 1991 and 2001 and mean follow up lasted 20 months (range 2-70). Endoscopic incision was performed in 21 patients. Upper pole nephrectomy, intravesical reconstruction or simple observation were decided for six patients. RESULTS: Endoscopic incision was effective in the treatment of intravesical UC and in some cases, without VUR in the lower pole, it was the unique procedure. Among ectopic UC, a secondary VUR developed following endoscopic incision in 43% of cases and required further treatment. Renogram did not show any significant functional improvement in the affected renal pole, secondary to endoscopic treatment. An open antireflux procedure was necessary in cases with associated VUR. CONCLUSIONS: Total or partial nephrectomy were performed among the first cases of the present series and in most of them it required a double step operation (abdominal and intravesical). In few cases of uncomplicated UC with a non functioning upper pole, a conservative approach was followed by spontaneous reduction of dilatation.     

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.     

Endoscopic injection of dextranomer hyaluronic acid copolymer for the treatment of vesicoureteral reflux in duplex ureters

PurposeEndoscopic injection of dextranomer hyaluronic acid copolymer (DxHA) has been increasingly utilized for the treatment of complex cases of vesicoureteral reflux (VUR). We present our 6-year experience with the use of DxHA for the treatment of VUR in duplex ureters.Materials and methodsBetween July 2001 and April 2007, 52 children were identified retrospectively who had been treated by endoscopic injection of DxHA for VUR into duplex ureters. Mean age was 3 years (range 9 months–10 years) with a mean maximum grade of reflux of III (range 2–5). The refluxing lower pole ureter was injected using a subureteric injection technique (STING) or intraureteric injection technique (utilizing the hydrodistention implantation technique). The endpoint in all cases was the loss of hydrodistention of the ureteral orifice. Voiding cystourethrograms was obtained at 6 weeks–3 months to evaluate for the presence of VUR. Cure was defined as complete resolution of reflux in the treated moiety.ResultsAfter initial treatment, 38/52 (73%) patients were cured. Of the 14 failures, nine children underwent repeat endoscopic treatment with a 67% (6/9) resolution rate for repeat injection. Seven of the initial failures failed to grade I VUR. Overall, 85% (44/52) were cured after one or two treatments, 98% (51/52) were improved and only one (1.9%) with grade V VUR required open surgery. The treatment was well tolerated and there were no associated complications.ConclusionEndoscopic injection of DxHA copolymer corrected VUR in 85% of children with VUR into duplex ureters. This minimally invasive approach should be considered as a viable alternative to open surgery or antibiotic prophylaxis for the treatment of VUR associated with duplex ureters.     

Ureteroureterostomy irrespective of ureteral size or upper pole function: A single center experience

ObjectiveAlthough ureteroureterostomy (UU) is an established procedure for the treatment of duplex anomalies, there may be a reluctance to apply this approach to patients with poor upper pole function and/or marked degrees of ureteral dilation.MethodsAn institutional review board (IRB)-approved retrospective analysis of all patients undergoing UU between 2006 and present was performed. All patients underwent an end-to-side anastomosis with a double-J stent left in the lower pole ureter. Laparoscopic repairs were done ‘high’ and open repairs were done ‘low’. If the upper pole ureter remained massively dilated after transection, the ureter was partially closed to reduce the length of the anastomosis. Data collected included demographics, diagnosis, surgical interventions, imaging studies and outcomes.ResultsA total of 41 patients (43 renal units) were identified. There were 35 females and six males with an average age at surgery of 2.3 years (range 55 days to 15.9 years) and an average follow up of 2.8 years. Diagnosis included ureterocele (17), ectopic duplex ureter (25) and ureteral triplication (1). Thirty-six patients underwent UU only and five underwent UU with simultaneous lower pole reimplantation. Twelve of the 41 patients (29%) underwent laparoscopic repair. Twelve of the 43 renal units (28%) required ureteral tapering, of which three were performed laparoscopically. Preoperative median upper pole function was 17% (0–35%).Six patients had no measurable function and ten had < 15%. No patient developed lower pole hydronephrosis in the follow-up period. There were two complications: one patient was found to have a post-operative ureterovesical junction (UVJ) stricture and the second had an anastomotic stricture.ConclusionUreteroureterostomy is a safe and effective technique for the reconstruction of duplex anomalies, even with a massively dilated and poorly functioning upper pole moiety. With no identifiable negative effect on the lower pole system, the concept of automatically removing ‘dysplastic’ upper pole segments can be challenged.     

Endoscopic correction of vesicoureteric reflux in infants under 2 years of age

Forty-seven infants under the age of 2 years with vesicoureteric reflux were treated by subureteric injection of Polytef paste; 4 have completed sufficient follow-up to be analysed. The 41 patients had reflux into 60 single-system ureters and 12 duplex systems. Fifty-five single-system ureters have ceased refluxing and 4 of the remaining 5 have improved, whereas 7 of the 12 kidneys with reflux into one or both segments of a duplex system no longer reflux. Thirty-one patients had only single-system disease, and 26 (84%) are totally corrected. Of the 10 patients with 12 duplex systems that refluxed, 5 were completely corrected. Overall, 31 (76%) of the patients are free of reflux. Correspondence to: P. A. Dewan     

Complications of pyelo-ureteral duplications in children and their surgical treatment. Apropos of 69 cases (78 pathogenic duplications)

The authors report their experience on the surgical treatment of 69 infants and children with pyelo-ureteral duplication. Urinary infection was the main revealing symptom (52 cases). Vesico-ureteral reflux was the major associated anomaly (39 cases) involving most often the lower pole ureter; the surgical correction of the reflux was performed according to the technique of Cohen with a favorable outcome in all cases. Because of poorly functioning and dysplastic upper renal segment, partial upper pole nephrectomy was performed in 8 among 12 cases of associated ectopic ureteral orifice and in 9 among 15 cases of associated ureterocele. A reimplantation of the ureter was made possible in 3 cases of ectopic ureteral orifice and in 5 cases of ureterocele with little corresponding renal lesions. With the experience of one neonate who died from septic shock following partial nephrectomy there were no other post-operative complications in this series.