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.     

Pediatric laparo-endoscopic single site partial nephrectomy: Feasibility in infants and small children for upper urinary tract duplication anomalies

ObjectiveTo assess the feasibility and outcomes of laparo-endoscopic single site (LESS) partial nephrectomy (PN) in infants and small children for upper urinary tract duplication anomalies.Materials and methodsThe medical records of all patients undergoing LESS PN at a single pediatric institution were retrospectively reviewed for patient demographics, perioperative details, and outcomes. A cystoscopy was initially performed to place an externalized catheter into the ureter of the ipsilateral normal renal moiety. An Olympus TriPort, an Olympus Endoeye flexible tip laparoscope, standard 3- or 5-mm instrumentation, and a LigaSure Blunt were utilized.ResultsFour children (two boys, two girls) underwent LESS PN. Three patients underwent upper pole PN and one underwent lower pole PN. All procedures were performed for poorly functioning obstructed renal moieties (one ureterocele, one ureteropelvic junction obstruction and vesicoureteral reflux, and two ectopic ureters). Median age was 6.2 months (range 2.5–16.4 months). Median weight was 7.7 kg (range 6.1–12.6 kg). Median operative time was 126 min (range 97–180 min). No patient received inpatient postoperative narcotics. Median follow-up was 9.9 months (range 6.2–19.1 months). No postoperative complications were noted. Postoperative renal ultrasound demonstrated successful resection in all patients.ConclusionsLESS PN is technically feasible, safe, and effective for upper urinary tract duplication anomalies in infants and small children.     

Laparoscopic ureteral ligation (clipping): A novel,simple procedure for pediatric urinary incontinence due to ectopic ureters associated with non-functioning upper pole renal moieties

ObjectiveA simplified approach for the surgical management of symptomatic ectopic ureters, associated with a non-functioning upper moiety, with laparoscopic ureteric clipping is presented in this research paper.Materials and methodsProspectively collected data on nine consecutive girls with ectopic ureters associated with urinary incontinence who underwent laparoscopic clipping between February 2011 and December 2013. Surgical technique consisted of cystoscopy and insertion of ureteral catheter in the lower pole ureter to aid in identification and clipping of the ectopic ureter, which was achieved by standard trans-peritoneal laparoscopy.ResultsMedian age was eight years (range 4–17 years). Diagnosis was based on clinical findings, which were supported by: ultrasound (US), nuclear scans and magnetic resonance urography in Cases 9, 8 and 5, respectively. Bilateral complete duplication was present in two patients; the combination of cystoscopy and laparoscopy allowed adequate identification of the ectopic ureter causing incontinence in both. All nine patients were immediately dry after surgery and remain asymptomatic after a maximum follow up of 27 months. Eight out of nine patients had developed some degree of asymptomatic upper pole hydronephrosis on follow-up US.ConclusionLaparoscopic clipping holds promise as a simple alternative to other more-complex surgical procedures in the treatment of incontinence due to an ectopic ureter. Despite favorable and encouraging initial results, further follow up is warranted in order to determine the fate of expected associated upper-pole hydronephrosis.     

Giant neonatal hydronephrosis of the upper pole of a complete duplicated renal system

Ureteropelvic junction obstruction (UPJO) in the upper pole of a complete duplicated renal system is extremely rare. We report a case that was diagnosed prenatally and we review 10 cases published in the literature. Diagnoses of adrenal hematoma and cystic neuroblastoma were suspected on prenatal ultrasound, based on the severity of dilatation and the difficulty of identifying the exact origin of this anomaly. Neonatal magnetic resonance imagery (MRI) was subsequently able to show a normal adrenal gland. Surgical management is similar to that of a single-system pyeloureteral junction obstruction. Since this situation is very rare, we believe cystoscopy can be useful to confirm diagnosis of UPJO associated with complete duplication, especially in the absence of preoperative MRI. Among the 11 surgical cases previously published, seven patients underwent pyeloplasty and four heminephrectomy.     

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.     

Congenital hydronephrosis in the lower segment of a duplex system

Two cases of hydronephrosis of the lower moiety in duplicated collecting systems, one associated with a complete duplication and another with an incomplete duplex system, are reported. This is a rare anomaly that requires a precise preoperative anatomic diagnosis to allow the planning of the most suitable surgical procedure. Treatment of this entity should follow similar guidelines as single-system hydronephrosis. An end-to-side dismembered pyeloureterostomy is the optimal operative technique.     

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.     

Prone retroperitoneoscopic approach for 30 Heminephroureterectomies; The Specific Advantages

PurposeThe lateral approach is more widely used in retroperitoneoscopic renal surgery due to familiar orientation and ease of conversion. We present a series of 30 heminephroureterectomies (HNU) where prone approach was utilised extremely effectively with no conversions and low complications.Material and MethodsFrom November 2003 to October 2007, 31 HNU were performed. Thirty of these, done in prone position, were included in the study. Females constituted 57%. Mean age was 3.1 years (range 0.9-13.3, 60% under 2 years). The upper moieties were excised in 17 patients (9 ureterocels, 8 ectopic ureters) and lower moieties in 13 (10 reflux, 2 pelviureteric junction obstruction, 1 lower moiety stones). The video demonstrates the important steps and the specific advantages of this approach. Prospectively recorded data and notes were reviewed. Mean follow up was 19 months.ResultsMean operative duration was 141 min (range 100- 280 min). Drains were used in 50% for a mean of 2 days. Average post-operative stay was 1.7 days (range 0-6 days). One child, undergoing lower HNU, sustained ‘nipping’ of the upper moiety ureter with an endoloop causing incomplete obstruction. This was the 8th patient of the series. Residual ureteric stump was seen in 5; however, only one was symptomatic requiring excision of the stump and the ureterocele (this was the third patient of the series). There were no transfusions or conversions. Routine ultra sound showed healthy residual moieties in all.ConclusionsWith prone approach, the anatomy could be demonstrated quickly and clearly. The dissection could be done with ‘two-hands’ as the kidney is hanging down with no need for retraction. The whole length of the renal vessels could be seen, enabling accurate and meticulous control. The extent of ureteric dissection wasn't impaired and stump complications were minimal. Prone retroperitoneoscopic approach could be highly recommended for HNU.     

Bypass pyeloplasty: Description of a procedure and initial results

Introduction and objectiveDismembered pyeloplasty is the surgical technique of choice for open, laparoscopic and/or robot-assisted repair of ureteropelvic junction obstruction (UPJO). We describe a new technique, bypass pyeloplasty, ideally suited for the high inserting ureter, and present initial results.Patients and methodsA wide 1–2-cm side-to-side anastomosis is created between the dilated and elastic portion of the ureter just distal to the UPJO and the lower and dependent portion of the hydronephrotic renal pelvis. The UPJ is not disturbed and the renal pelvis is not surgically reduced. Since 2004, of 27 patients requiring surgery for UPJO, 7 underwent bypass pyeloplasty. The indications for surgery included increasing hydronephrosis or decreasing individual renal function in four, pain in two and pyelonephritis in one. The remaining 20 underwent a classic dismembered pyeloplasty.ResultsDuring a mean follow-up of 26 months, the anteroposterior diameter of the repaired kidney decreased by a mean of 55%. The individual renal function in the repaired kidney improved in two and remained stable in the remainder.ConclusionThese favorable initial results justify further exploration of this simplified technique and its adaptation for laparoscopic and robot-assisted approaches. Bypass pyeloplasty may be a more physiologic procedure in patients with mid to high insertion of the ureter.     

Laparoscopic management of ureteropelvic junction obstruction in pediatric patients: A new approach to crossing vessels,crossing vein division,and upward transposition of the crossing artery

ObjectiveTo demonstrate the role of the laparoscopic approach for management of primary ureteropelvic junction obstruction (UPJO) using two different techniques in the pediatric age group.Material and methodsFrom April 2005 to October 2008, 63 pediatric patients underwent treatment of primary UPJO via a laparoscopic approach. Dismembered pyeloplasty was elected in 56 renal units while nine patients were managed by upward transposition of accessory renal artery after division of accessory renal vein. No JJ stent was required in these nine patients.ResultsMean age of patients was 61 (2–180) months. Mean operative time was significantly lower in those managed by transposition of aberrant vessels compared with dismembered pyeloplasty. The mean hospital stay was 6.4 (2–14) days for the dismembered technique and 2.1 (1–4) days for the vascular transposition approach. Significant improvement of obstruction was achieved in all of the patients who underwent the modified Hellstrom technique and in 92.81% of the renal units undergoing dismembered pyeloplasty.ConclusionThe technique of laparoscopic transposition of crossing renal artery without violating collecting system may have a role in minimally invasive management of UPJO in selected pediatric patients. Further research with larger samples and a longer follow-up period is required.     

Comparison of surgical outcomes and cosmetic results between standard and mini laparoscopic pyeloplasty in children younger than 1 year of age

ObjectiveTo evaluate safety, efficacy, and cosmetic results after mini laparoscopic (mL) pyeloplasty and standard (sL) pyeloplasty in children younger than 1 year of age with ureteropelvic junction obstruction (UPJO).Materials and methodsFrom August 2009 to March 2011, 20 sL pyeloplasties were performed in pediatric patients younger than 1 year of age; afterwards, 20 patients younger than 1 year of age underwent mini laparoscopic (mL) pyeloplasty from June 2011 to August 2012. The patients were followed by urine culture and ultrasonography at 3 and 6 months after surgery. Cosmetic appearance was assessed in all patients in both groups group 3 months after surgery using the Patient Scar Assessment Questionnaire.ResultsPeri and postoperative results revealed that operative time (total and anastomosis of ureteropelvic junction), hospital stay, and overall complication rate were significantly lower in mL than in sL. Persistent hydronephrosis in follow-up imaging and recurrence of obstruction was not observed in any cases. Mean appearance score and consciousness score showed significantly better results in the mL group.ConclusionWe believe that mL pyeloplasty in infant cases with UPJO is more cosmetically pleasing and less invasive than sL pyeloplasty and has similar functional outcomes.     

A minimal invasive surgical approach for children of all ages with ureteropelvic junction obstruction

ObjectiveOpen dismembered pyeloplasty is considered the gold standard to treat ureteropelvic junction obstruction (UPJO) in children. Laparoscopic pyeloplasty (LP) and robot-assisted pyeloplasty (RAP) are increasingly popular. Our present protocol consists of using minimally invasive techniques for all children with UPJO. Here, we report our first 40 cases operated under this protocol.Patients and methodsRetrospective chart review of patients who underwent LP and RAP for UPJO between 2006 and 2010 was performed. Children younger than 4 years of age underwent LP and children aged 4 years and older with robot assistance. Results were assessed comparing pre- and postoperative imaging studies, operating time, hospital course and complications.ResultsThirty-nine patients underwent 41 dismembered pyeloplasties (20 patients LP, 19 patients RAP). No conversions to open surgery were performed. The difference in operative time was statistically significant. The average hospital stay was 7 days (LP) and 6 days (RAP). All patients showed significant decrease of hydronephrosis and the overall success rate was 100%. The complication rate was 25% in the LP and 28% in the RAP group.ConclusionOur data show that RAP and LP are effective to correct UPJO with similar outcomes and complication rates. None of the patients in this series required re-intervention to correct obstruction and the results are comparable with open surgery.     

Transumbilical laparoendoscopic single-site pyeloplasty in infants and children: initial experience and short-term outcome

Background  

Scar-free abdominal wall surgery is a research hotspot in recent years. This study presented surgical skills of transumbilical laparoendoscopic single-site pyeloplasty (LESS-P) for pediatric patients with ureteropelvic junction obstruction (UPJO) and its clinical application.     

The roles of extracellular matrix proteins,apoptosis and c-kit positive cells in the pathogenesis of ureteropelvic junction obstruction

AimTo investigate histopathological changes in ureteropelvic junction obstruction (UPJO) from an etiological perspective.Patients and methodsMedical records of patients with UPJO were reviewed and pathological specimens collected. Nephrectomy materials from forensic autopsies were taken as controls. Specimens were assessed with light microscopy. Fibronectin, type 4 collagen, laminin, Bax and Bcl-2 expression for apoptosis, together with interstitial cells of Cajal determination with c-kit were determined immunohistochemically. Staining scores were evaluated semiquantitatively. Results were evaluated using Mann–Whitney U-test.ResultsControl group comprised 14 children (median age, 3.5 years; 6 months–17 years). Study group comprised 22 children with UPJO (median age, 9 months; 1 month–10 years). Light microscopy revealed non-specific inflammation, epithelial proliferation and atrophy with fibrosis in the smooth muscle of the UPJ in all patients. Fibronectin, type 4 collagen and laminin were found to be significantly increased in UPJO at the intrafascicular space of smooth muscle and the matrix of stroma. Bcl-2 expression was increased in UPJO. c-Kit was unable to stain interstitial cells of Cajal, but staining for mast cells was significant.ConclusionsHigh expression of fibronectin, laminin and type 4 collagen may indicate a relation to the pathogenesis of UPJO. Defective kidney morphogenesis, during branching and tubulogenesis of ureteric bud, may be responsible for this congenital pathology.     

Laparoscopic management of obstructed partial duplex with Y ureteric configuration

An 11-year-old girl underwent a laparoscopic upper-to-lower moiety ureteropyelostomy for lower moiety pelviureteric junction obstruction associated with Y ureteric duplication. She presented with left flank pain and was noted to be hypertensive with microscopic haematuria on admission. An abdominal ultrasound scan revealed a duplex left kidney with gross lower moiety pelvicalyceal dilatation. A subsequent MAG-3 scan demonstrated reduced differential function in the left lower moiety. A laparoscopic transmesocolic approach confirmed a dilated lower moiety pelvis and revealed the Y ureteric duplication. An upper to lower moiety ureteropyelostomy and insertion of a JJ stent is described in the accompanying video. The child was discharged home on the second day post operation. Postoperative imaging showed good decompression and improved differential function. The child has been completely well and normotensive at 2 years' follow-up.     

Laparoscopic pyeloplasty in children: Is the outcome different in children under 2 years of age?

PurposeTo report the outcomes of a series of children younger than 2 years with ureteropelvic junction obstruction (UPJO) who underwent laparoscopic pyeloplasty (LP), compared to children older than 2 years.Patients and methodsTwenty-three children (18 male) with UPJO underwent 24 Anderson–Hynes transperitoneal LPs between March 2003 and July 2007. The diagnosis of UPJO was confirmed on renal sonography and diuretic renogram. Nine children were younger than 2 years (Group 1) and 14 were older (Group 2). One child had bilateral UPJO and underwent two non-simultaneous procedures. All children were investigated with postoperative diuretic renogram and renal sonography, and the results were statistically analysed.ResultsLP was feasible without conversions or intraoperative complications in all 24 cases. Median age in Groups 1 and 2 was 11 months (4–24) and 74 months (27–204), respectively, (P < 0.00001). No differences were found between the groups regarding median operative time, complications, technical difficulties, discharge home and follow-up. All patients in both groups experienced resolution of symptoms or obstruction.ConclusionsThe LP in children younger than 2 years is highly successful with a low-rate of complications, and has the same outcomes as in older children.     

Laparoscopic pyeloplasty is feasible for lower pole pelvi-ureteric obstruction in duplex systems

Exclusive lower pole pelvi-ureteric junction obstruction (PUJO) in double collecting systems in children is a rare condition requiring reconstructive surgery. We report on the feasibility of laparoscopic transabdominal dismembered pyeloplasty in two cases. Two children with duplicated collecting systems presented with hydronephrosis of lower pole moiety due to exclusive PUJO. Isotope renography revealed impaired drainage of affected lower kidney pole. A four-trocar transabdominal technique was used. No stent was used in one patient with bilateral duplication (male, 6 years) while a 4 Fr. double-J stent was placed laparoscopically in another with unilateral duplication (male, 9 months). Both patients had uneventful laparoscopic transabdominal dismembered pyeloplasty. The operation time was 115 and 155 min, respectively, and was comparable to our previously reported patient series undergoing laparoscopic pyeloplasty for singular collecting systems. After a mean follow-up of 21 months (range 12-42), both patients were asymptomatic and showed improved pelvi-ureteric drainage on isotope renography and improved hydronephrosis on ultrasound scan. Laparoscopic transabdominal dismembered pyeloplasty is safe and effective in children with rare lower pole PUJO in double collecting systems.     

Misleading appearances in pediatric uroradiology

Certain misleading appearances are peculiar to pediatric uroradiology. The most frequently encountered pitfalls are related to the bladder, to vesicoureteral reflux, and to the duplicated collecting system. The bi-chambered nature of the child's bladder, and the rapid settling of contrast material to the most dependent portion causes many pitfalls in diagnosis. When the child is prone, normal ureters may seem to be ectopic, and ureteroceles may become invisible. When the child is supine, the volume of urine in the bladder may be grossly under-estimated. Reflux can mimic function at urography. The dynamic nature of reflux leads to under-estimation of its presence and degree on the IVP and static cystogram. Reflux into an already dilated system can lead to over-estimation of its degree. Aberrant micturition with rapid refilling of the bladder can simulate incomplete emptying. The diagnosis of ectopic ureterocele is based on indirect evidence. Any condition that affects the urinary apparatus in the same way will have a similar appearance. A huge ureterocele may have a small ureter, and massive reflux into a lower pole ureter may make the diagnosis of duplication difficult. Ureterocele lookalikes, and effacement or intussusception of the ureterocele are cystographic pitfalls. Lower pole ureteropelvic junction obstruction and Wilms tumor in the lower portion of a kidney can have surprisingly similar appearances.     

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.