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Introduction: Diagnosing real urinary obstruction and surgical decision making for the operative correction of urinary obstruction, are still problematic in congenital hydronephrosis (CH). Compliance of the renal pelvis is one of the important defense mechanisms of renal parenchyma against urinary obstruction. We observed early loss of function in some cases of CH with low and moderate anteroposterior diameter of the renal pelvis (APDRP)., Objective: To evaluate structural properties of the renal pelvic tissue of patients with CH and the relation of these structural properties with renal function and Anteroposterior diameter of the renal pelvis., Study Design: Ureteropelvic junction (UPJ) excised during UPJ obstruction operations from 2013 to 2019 were evaluated histopathologically. The patients were divided into the two groups according to initial renal function, i.e. group with initial preoperative differential renal function (DRF) less than 35% and group with initial DRF greater than 35%. The percentage of collagen to whole tissue area were analyzed using image processing program. The relationship between DRF and tissue collagen ratio, SFU and APDRP was evaluated., Results: There were 5 patients in the DRF <35% group and 16 patients in the DRF >35% group. However, APDRP's of the DRF <35% group were also significantly lower than the DRF >35% group. The collagen distribution in the muscle layer was more prominent in the DRF <35% group. Proportionally, percentage of collagen stained surface was significantly higher in DRF <35% group., Discussion: There are numerous histopathological studies evaluating the cause of UPJ obstruction. Besides these studies that are oriented to etiology, there are many studies comparing the histopathological changes at UPJ with surgical outcome and prognosis. In the present study, we found that renal pelvis collagen ratio was significantly increased in patients with lower APDRP and with severe renal function loss. This increase in the collagen content in the renal pelvis have been shown to affect the compliance negatively and decrease APDRP, which leads to a faster loss of renal function. Thus, pelvic structural changes accompanying UPJ obstruction may aggravate urinary obstructive process., Conclusion: Increased renal pelvis collagen ratio negatively affects the expansion of the renal pelvis, which is one of the protective mechanisms of the renal parenchyma, and may be one of the triggering mechanisms of early loss of renal function., Competing Interests: Conflict of interest None., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Open pyeloplasty (OP) has traditionally been the standard for the operative management of ureteropelvic junction obstruction in children. With advances in minimally invasive pyeloplasty (MIP) techniques, it is quickly becoming a popular alternative in both adult and pediatric population., Objective: To evaluate the differences in outcomes between MIP and OP for the surgical correction of ureteropelvic junction obstruction in children., Study Design: Data were obtained from the pediatric National Surgical Quality Improvement Program 2012-2017. We identified 1280 patients who underwent MIP and 1190 patients who underwent OP between 2012 and 2017. Propensity score matching was utilized to adjust for baseline differences. Univariate and multivariable regression were performed to assess odds of complications and procedure-related readmission., Results: Patients who underwent OP had a significantly decreased operative time (192.42 vs 142.00 min, p < 0.001) compared to MIP. There was no significant difference in the rates of overall peri-operative complications (3.7% [MIP] vs 2.4% [OP] p = 0.397). On multivariable analysis, patients undergoing OP had a lower risk of procedure-related readmission (odds ratio [OR] 0.404, 95% confidence interval [CI] 0.157-0.951, p = 0.046) than MIP. In a multivariable linear regression model, the risk of having any postoperative complication, regardless of surgical approach, decreased with increasing patient age (OR 0.945, 95% CI 0.893-0.996, p = 0.037)., Discussion: Although recent small, retrospective institutional studies have found decreased hospitalization time of MIP as compared to OP, in our large prospective database, we found no such association. While some studies suggest a higher rate of wound complications in the OP group, this was not reproduced in our study as well. MIP was, in fact, associated with higher rate of readmissions as compared to the OP group, which may act as a surrogate of long-term complications in these patients., Conclusion: MIP offers an alternative to OP in the pediatric population with similar rates of peri-operative complications. However, our study shows decreased odds of procedure-related readmission in OP, which may serve as a surrogate for less postoperative complications in these patients., (Copyright © 2019 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

The etiology of ureterovesical junction obstruction (UVJO) and ureteropelvic junction obstruction (UPJO) is obscure with an adynamic narrow segment causing the obstruction. In this study, the authors compared interstitial cells of Cajal (ICC) and collagen-to-muscle ratio (CM ratio) between UVJO, UPJO, and fetal ureters to investigate whether a maturational arrest of the fetal ureter could explain both clinical pathologies., Methods: Group 1 (control) involved specimens of the normal ureter (nephrectomy for trauma/tumor; n = 20), while group 2, specimens of UVJO (n = 14); group 2 was further divided into group 2a, the dilated megaureter above UVJO, and group 2b, UVJO narrow segment; group 3, UPJO narrow segment excised during pyeloplasty (n = 31); and group 4, normal fetal ureters (n = 12). The specimens were analyzed for ICC using immunohistochemistry and CM ratio on Masson's trichrome (stains collagen in blue and muscle in red)., Results: The median ICC/10 high-power field was 16.1 (8.3) in the normal and 17.3 (7.9) in the dilated segment of the megaureter, with no significant difference, but was significantly less in the narrow segment of UVJO at 4.5 (2.0), narrow segment of UPJO at 5.1 (2.3), and fetal ureter at 5.0 (2.3). The median CM ratio was 0.75 (0.29) in the normal and 0.65 (0.2) in the dilated segment of the megaureter, with no significant difference between them (figure), but was significantly higher in the narrow segment of UVJO at 3.0 (0.8), narrow segment of UPJO at 2.5 (0.71), and fetal ureter at 3.1 (0.61). Overall UVJO, UPJO, and fetal ureter segment had significantly less ICC density and more collagen compared with the normal ureter (P < 0.001 by Mann-Whitney U test)., Discussion: There are conflicting reports on the etiopathogenesis of UVJO and UPJO, with several authors showing decreased ICC and increased collagen in the narrow segment. In this study, the authors found that the pathological changes at UVJ and UPJ segments resemble fetal ureter morphology. We also found that in fetal ureters, as the gestation progressed, there was an increase in the ICC density/smooth muscle, whereas the collagen content decreased. While the entire ureter has uniform embryological origin, it essentially remains an epithelial tube until the late gestation. The maturational process involves differentiation of smooth muscles cells/ICC to establish the peristaltic machinery required to functionally connect the ureter at both ends. This process, probably, starts at the mid ureter during fetal life and extends toward the UPJ and UVJ, and its failure, probably, results in UPJO or UVJO. The study's limitations are small numbers, and further larger studies are required to validate this hypothesis., (Copyright © 2019 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: In children with upper tract dilatation, diuretic renography, which includes application of mercaptoacetyltriglicin-99 mTc (MAG3), is the standard examination. Ordinarily, furosemide is applied 20 min after tracer injection (F + 20). In children with persistent hydronephrosis and preserved split function, this protocol may lead to further examinations with repeatedly equivocal washout curves. The present study evaluated the potential of MAG3 (F-15) scans in this subgroup of children to achieve a conclusive result avoiding additional equivocal MAG3 (F + 20) scans., Objective: To evaluate whether the washout curve using the F-15 protocol is a helpful criterion with which to clarify results in patients with equivocal patterns in the F + 20 protocol., Patients: 31 children (male/female 22/9, median age at time of examination 42 months, mean anterioposterior diameter 2.1 cm, left/right 14/18) underwent MAG3 F-15 renography at the present department because of upper urinary tract dilatation and (repeatedly n ≥ 2 F + 20 = 28) equivocal results in MAG3 F + 20 examinations., Results: In 10/31 children (32.2%), MAG3 F-15 revealed an obstructive pattern, indicating a pyeloplasty in 9/10 of them (90%). In 16/31 children (51%), the F-15 protocol showed a non-obstructive curve, leading to further uneventful follow-up in 15/16 of them (93.7%). In 5/31 children (16%), MAG3 F-15 led to equivocal results, resulting in no pyeloplasty and further uneventful follow-up in all the children (mean follow-up 1.46 years)., Discussion: In children with persistent high-grade hydronephrosis on ultrasound and preserved split function, multiple scans were prompted and further management was sometimes difficult. Although there was proven evidence that the F-15 protocol reduced the frequency of equivocal pattern, there was no indication for primary F-15 investigation, due to the risk of over diagnosis of obstruction. The F-15 scan was considered to be a stress test of the upper tract leading to a diuretic challenge without any opportunity to investigate normal washout curve. In these cases, partial obstruction could lead to false positive obstructive results. A stepwise approach, in which the traditional F + 20 technique allows observation of the rate of washout of radiopharmaceuticals before forced diuresis followed by a F-15 protocol, could reduce the child's additional radiation burden and stress., Conclusion: After equivocal results in MAG3 F + 20 protocols, performing a MAG3 F-15 exam lead to a conclusive result in 80.6% (25/31 patients) of the cases. Thus, performing a MAG3 (F-15) exam can be recommended in children with persistent hydronephrosis and preserved split function after equivocal results on MAG3 (F + 20) scans., (Copyright © 2016 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare syndrome characterized by mullerian and renal anomalies. Renal agenesis is classically considered to be part of the definition of OHVIRA syndrome. There are increasing reports that the lack of an ipsilateral kidney on imaging studies is not from true renal agenesis but renal dysplasia and atrophy. We have observed cases where this ipsilateral dysplastic and atrophic kidney leads to clinical symptoms. The objective of this study is to report cases of OHVIRA syndrome where an ipsilateral dysplastic and atrophic kidney led to clinical symptoms, and to raise awareness in the pediatric urology community of this variant of OHVIRA syndrome., Material and Methods: All cases of OHVIRA syndrome with an ipsilateral dysplastic and atrophic kidney were identified from January 2010 to June 2013. The patient's presentation, clinical course, surgical management, radiologic findings, and pathologic finding were reviewed., Results: There were three cases of OHVIRA syndrome with a symptomatic ipsilateral dysplastic and atrophic kidney identified. All three of these dysplastic and atrophic kidneys had an ectopic ureter to the obstructed hemivagina and led to persistent vaginal drainage after resection of the vaginal septum. These dysplastic and atrophic kidneys were not visualized on any imaging studies. Laparoscopic removal of the dysplastic and atrophic kidney led to cessation of vaginal drainage in all cases., Conclusion: This study reports additional cases to the literature that do not fit the classic definition of OHVIRA syndrome. In the cases in this study, there was no ipsilateral renal agenesis, but an ipsilateral dysplastic and atrophic kidney with an ectopic ureter to the obstructed hemivagina. In addition, these cases demonstrate that the dysplastic and atrophic kidney may cause symptoms such as persistent vaginal drainage after resection of vaginal septum. The dysplastic and atrophic kidneys may not be detected by any imaging modality including MRI and DMSA scan and may be ectopic in location. All three of our cases had persistent vaginal drainage after resection of the vaginal septum which was cured by removal of the dysplastic and atrophic kidney ipsilateral to the obstructed hemivagina. The limitations of this study include the small number of patients and retrospective nature. While the classic definition of OHVIRA syndrome includes ipsilateral renal agenesis, there is increasing evidence that the absence of a kidney on imaging is due to renal dysplasia and atrophy and not true renal agenesis. In addition, we have observed that this dysplastic and atrophic kidney may cause clinically significant issues such as persistent vaginal drainage through an ectopic ureter to the ipsilateral hemivagina after resection of vaginal septum. This small dysplastic and atrophic kidney may not be visualized on imaging studies and laparoscopy can be diagnostic and therapeutic if indicated. Ipsilateral renal anomalies, not ipsilateral renal agenesis, should be considered part of the definition of OHVIRA syndrome. Pediatric urologists need to be aware of the spectrum of renal anomalies in OHVIRA syndrome., (Copyright © 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Unlabelled: Although initial conservative management is popular in the management of antenatally detected pelvi ureteric junction (PUJ) obstruction [1-3], several authors [4,5] have questioned this approach and expressed concern about failure to recover the function lost during expectant management following surgery. In this single center prospective study, we have compared the functional outcomes following early versus delayed pyeloplasty in SFU grade 3-4 PUJ obstruction., Methods: Among those children, who presented between 2004 and 2013, with prenatal diagnoses of unilateral PUJ obstruction (n = 886), those with SFU grade 1 or 2 hydronephrosis on USG (n = 533) were excluded. In the remaining 353 children with SFU grade 3 and 4 hydronephrosis, 243 had obstructive pattern on radionuclide scan. After excluding those with severely impaired or supranormal split renal function (SRF), palpable mass, single kidney status, bilateral disease and associated other urological anomalies a total of 126 children were included in the study group. Parents who were unwilling for a frequent follow-up underwent early pyeloplasty, (Group I: n = 62) while the remaining underwent initial conservative management, with 3 monthly USG and nuclear scans (Group II; n = 64). In this group pyeloplasty was performed whenever there was deterioration in SRF >10%, or urine infection or pain during the follow-up. A standard open dismembered pyeloplasty was performed by the same surgeon in all patients. Radionuclide scan was performed at 1 year, at the same center using the same protocol, to assess final SRF and drainage. The functional outcomes were compared using student's t test and chi square test., Results: Group I comprised of 62 patients while Group II 64 patients. The mean age at pyeloplasty was 2.8 months in group I while 12.5 months in group II. There was no significant difference in the initial antero posterior diameter (APD) between the groups; 30.2 (±3.2) mm in group I and 29.6 (±3.7) mm in group II. At 1-year follow up after surgery, there was improvement in the APD, 16.8 (±4.2) mm in group I and 18.2 (±4.5) mm in group II, with no significant difference between them. In group I, the initial mean SRF was 34.1% (±6.4) and there was significant improvement (p = 0.01) in mean SRF to 37.2 (±7.1) at 1-year follow up after surgery. In group II, the mean SRF was 35.9 (5.7) initially and there was a deterioration to 32.6 (±5.5) before surgery (Figure). At 1-year follow up after surgery, there was a marginal improvement to 33.5 (5.6), however it was significantly lower compared to the initial SRF (p = 0.01). Compared to initial function, at 1-year follow up after pyeloplasty, SRF improved in significantly higher number of patients; 17/62 (27.4%) in group I while only 7/64 (10.9%) in group II (p = 0.03) (Table). There was significantly fewer patients with deterioration in final SRF at 8/62 (12.9%) in group I compared to 22/64 (34.4%) in group II (p = 0.03)., Discussion: Although several publications [1-3] have reported functional recovery during initial conservative treatment of PUJ obstruction, in our study a large proportion of patients (80%) in Group II had loss of function during follow-up. This is probably because the study population included only SFU grade 3-4 with obstructive renogram. Several authors have expressed concern about irreversible loss of renal function during expectant management [4,5]. Findings of our study reveal that irrespective of initial SRF, early pyeloplasty in prenatally diagnosed SFU grade 3-4 PUJ obstruction leads to significant improvement of SRF, while delayed pyeloplasty leads to a marginal but, significant loss. This fact should be highlighted to parents so that informed decisions can be made regarding early versus delayed surgery., (Copyright © 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: In this study we aim to demonstrate that robot-assisted laparoscopic (RAL) reoperative repair is safe and effective and even less technically demanding than open repair for recurrent ureteropelvic-junction obstruction (UPJO)., Study Design: A retrospective study was conducted of all cases of failed open pyeloplasties who underwent RAL reoperative repair at our institution between January 2010 and December 2013. The general surgical procedure was the same we previously described for robot-assisted laparoscopic pyeloplasty. Success was defined as: improvement in the degree of hydronephrosis at ultrasound, improvement of diuretic washout time at postoperative diuretic renogram (<15 min), improvement or at least stable differential renal function and absence of symptoms. These radiographic and symptomatic criteria of success were considered the primary outcomes. Secondary outcomes included complications and length of hospital stay., Results: Between 2000 and 2013 a total of 153 patients underwent open Anderson-Hynes dismembered pyeloplasty. Of these 9 (6%) had recurrent UPJO. Four patients underwent open redo pyeloplasty. As a result, our study population comprised 5 children who underwent reoperative RALP repair. Patient characteristics and outcomes are summarized in the table below. Our success rate was 100%., Discussion: Due to the low failure rate of open dismembered pyeloplasty there is no consensus on the best surgical approach for recurrent obstruction. While endoscopic approaches have been favored in adults, children have shown better success rates with repeat pyeloplasty. Laparoscopic salvage pyeloplasty for failed open procedures has become more popular and has been shown to result in excellent outcomes while providing the advantages of minimally invasive surgery. To date, the literature regarding the use of RALP for failed open procedures in the pediatric population is scarce. Only 2 pediatric series of robotic reoperative pyeloplasty have been reported by Helmal et al. (9 patients) and Lindgren et al. (16 patients) with a success rate of 100 and 88%, respectively. Although this is one of the first published studies about robot-assisted laparoscopic reoperative repair for failed open pyeloplasty in pediatric patients, we acknowledge the limitations of our study due to the small number of patients, its retrospective nature and limited follow-up time., Conclusions: The incidence of failed open pyeloplasty is as low as 5% and management remains controversial. As reported by other authors, we believe that crossing vessels play a particularly important role in secondary obstruction and adversely impact the outcome. Redo pyeloplasty, open or minimally invasive, is associated with high success rates (80-100%) and therefore considered the treatment of choice by the majority of authors nowadays. Additionally, RALP for secondary procedures has demonstrated to be safe and even less technically demanding when compared to the open approach, providing the advantages of minimally invasive surgery., (Copyright © 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Pediatric ureteropelvic junction obstruction (UPJO) due to an extrinsic crossing vessel (CV) is rare and often remains undiagnosed preoperatively. Vascular hitch procedures are often performed as associated intrinsic obstruction is not expected. We compared data and intravenous urography (IVU) findings of patients with aberrant CV versus those with intrinsic UPJO, all undergoing open dismembered pyeloplasty., Primary Objective: Is accurate pre-operative diagnosis of aberrant CV causing extrinsic UPJO possible?, Secondary Objective: To assess differences in the demographic, clinical, radiological, intra-operative features and postoperative improvement after pyeloplasty between patients with a CV and those with only intrinsic UPJO., Patients and Methods: Prospective study of all children below 12 years with UPJO presenting to a tertiary referral centre and who underwent open Anderson - Hynes dismembered pyeloplasty between 2003 and 2013 was conducted. Pre-operative investigations included serial ultrasonography, renal dynamic [ethylene di-cysteine (EC)] scan and IVU. These were repeated 3 months after pyeloplasty. Pre-operative IVUs of children with CV were compared with the IVUs of an equal number of similar aged children, randomly selected from the intrinsic obstruction group., Results: Pyeloplasty was performed in 643 children during the study period. Data of 33 children with aberrant CVs (mean age 6.99 years) were compared with the remaining 610 children (mean age 3.27 years) with only intrinsic obstruction. Highly significant associations of those with CV included age above 2 years, female gender, associated anomalies, abdominal pain in those above 2 years and poor preoperative function on IVU. Specific IVU features which were statistically highly significant in favor of presence of CV were small, intrarenal and globular flat bottomed pelvis. (Figure) Calyceal dilatation was also more prominent in the CV group. A funnel shaped, extrarenal pelvis was highly significant in favor of intrinsic obstruction. There was associated intrinsic obstruction in addition to CV obstruction in 8 children. All children symptomatically improved after pyeloplasty and did well on long term follow up. The majority showed improvement or stabilization of function on EC scan., Discussion: With the advent of antenatal ultrasonography, most children with UPJO are detected early. Children with CV tend to present later. This is often detected during surgery. Color Doppler is useful but is operator dependant and not performed routinely. In this study, IVU showed the presence of obstruction and loss of function unlike color Doppler, but also revealed specific diagnostic features not previously reported in literature. This can help in accurate preoperative prediction and avoid endopyelotomy, or a dorsal lumbotomy/retroperitoneal approach. Renal function in CVs is expected to be good as the obstruction is thought to be intermittent. However, we noted delayed contrast uptake on IVU in 60.6% and differential renal function on EC scan below 40% in 17 patients (56.6%). These indicate the effect of the obstruction on the renal parenchyma and the importance of early detection. Higher association with other anomalies and higher incidence in females has also not been emphasized in the literature so far. We noted associated intrinsic obstruction in 24.24% patients which is highly significant. This category of patients is likely to be missed and inappropriately treated if a "vascular hitch procedure" is performed. None of our patients had postoperative complications., Conclusions: Characteristic features were seen on IVU helping in preoperative diagnosis which can be extrapolated to magnetic resonance urography. There is a higher association of CV in age above 2 years, females, associated congenital anomalies, delayed uptake on IVU and differential renal function below 40% compared to intrinsic obstruction. Associated intrinsic obstruction in 24% with no postoperative complications indicates the superiority of dismembered pyeloplasty over vasculopexy procedures., (Copyright © 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: A marked decrease in the density of interstitial cells of Cajal (ICC) has been shown in patients with ureteropelvic junction obstruction. ICC may also play a role in primary obstructive megaureter (POM). An immunohistochemical study was conducted to investigate the density of ICC in ureterovesical junction (UVJ) segments resected from patients with POM and from control autopsy specimens., Materials and Methods: Resected UVJ segments from 11 patients operated for POM comprised the study group. Control UVJs were obtained from autopsy specimens of 7 children who died from causes other than urogenital pathology. The UVJs including the narrowest parts were studied using immunohistochemical staining for C-kit positive cells., Results: The number of ICC was significantly lower in the UVJs of the POM compared to the control group. The UVJs of the POM group had 1.75 ± 1.14 ICC (mean ± 1SD), whereas the control group had 5.76 ± 2.99 ICC (mean ± 1SD)., Conclusion: The number of ICC was decreased in the UVJs of the patients with POM compared to the normal control group. As dilation of the ureter during fetal life is a common condition and ureteral dilation is often self limiting, we hypothesize that ureteral peristalsis is a maturational event including the maturational development of ICC., (Published by Elsevier Ltd.)

The extrarenal calyx (ERC) is a rare congenital anomaly, associated with various other abnormalities of the urogenital system. We report a unique case of ERC in a solitary functioning kidney with a massively dilated pelvis that developed spontaneous rupture. A dismembered pyeloplasty was performed at the time of rupture. The patient did well post-operatively with a stable creatinine and stable SFU grade 2 hydronephrosis., (Copyright © 2013 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: Studies of surgical outcomes after reconstructive surgery for giant hydronephrosis (GH) secondary to ureteropelvic junction (UPJ) obstruction are limited. Over the past two decades, laparoscopic pyeloplasty has gradually replaced open repair in children in several centres. The objective of this study was to assess surgical outcomes of laparoscopic pyeloplasty in children with GH., Materials and Methods: Children with unilateral primary UPJ obstruction and GH were prospectively included and underwent laparoscopic pyeloplasty. Postoperative ultrasonography was repeated at 3 and 12 months to assess renal parenchymal thickness, and similarly a renogram was repeated to assess improvement in differential renal function., Results: During the study period 2005-2009, 53 children underwent laparoscopic dismembered pyeloplasty for UPJ obstruction. Of these, 8 children had GH caused by UPJ obstruction. The postoperative differential renal function improved in all of them. The postoperative improvement in renal parenchymal thickness at the end of 12 months was comparable to that of the non-GH group., Conclusions: At 12 months, surgical outcomes after laparoscopic pyeloplasty for GH were satisfactory. Relief of obstruction allows adequate and comparable nephron sparing., (Copyright © 2011 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: The mechanism underlying the evolution of congenital obstructive hydronephrosis is still unclear. In a previous study, we have shown that it is possible to create renal lesions in newborn mice specific to partial ureteral obstruction. We aimed to study the long-term results of such partial obstruction., Methods: Mice were operated on the third day of life. We created 2 groups: partial unilateral obstruction and control. We studied antero-posterior pelvis diameter, kidney length and volume on magnetic resonance imaging at day 10 and 3 months. We assessed ureteric patency by injecting Patent Blue dye. Kidney weight and fibrosis were histologically assessed. Fibrosis was assessed using Sirius Red staining and morphometry., Results: Imaging showed parenchymal atrophy in the partially obstructed kidney and compensatory hypertrophy of the contralateral kidney. Pelvis dilatation was detected at day 10 but remained stable without significant increase at 3 months. The patency test confirmed the absence of total obstruction in the long term. There was no correlation between the degree of dilatation and parenchymal atrophy or the contralateral hypertrophy. Pathological studies at 3 months revealed fibrosis in the parenchyma without significant correlation with pelvis dilatation., Conclusions: Long-term results confirmed that partial ureteral obstruction in newborn mice produces fibrotic lesions of the renal parenchyma, which are not correlated with dilatation of the upper tract. These results could contribute to the clinical management of obstructive uropathy in children, emphasizing that follow up with simple evaluation of upper tract dilatation is insufficient to predict renal deterioration., (Copyright © 2011 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: Open dismembered pyeloplasty is usually performed through flank, anterior subcostal or posterior lumbotomy incisions. These incisions are cosmetically less acceptable and may produce significant postoperative pain. We present the smallest incision for open pyeloplasty, called a 'miniature pyeloplasty'. The aim of this study was to reduce hospital stay and postoperative pain, along with enhanced cosmetic results., Patients and Method: 373 infants (mean age 4 months) with hugely dilated pelvises underwent the miniature pyeloplasty. The exact site of incision was determined by intraoperative renal ultrasonography and palpation. A muscle-splitting incision was made in the most dependent part of the lower quadrant. After meticulous dissection of the ureteropelvic junction component, the affected section was pulled out and underwent classic dismembered pyeloplasty without renal pelvis reduction. All children had long-duration stented anastomoses. Surgical incision size, operative time, hospital stay, postoperative analgesic use and complication rate were recorded., Results: The operation was successful in all patients. The mean operative time was 53 min (range 43-75) and patients were discharged after 18 ± 3 (mean ± SD) h. Incision size ranged from 11 to 15 mm (mean 13). No narcotic analgesic was required postoperatively and there were no major complications during follow up., Conclusions: Miniature pyeloplasty is a safe and successful technique for ureteropelvic junction obstruction that avoids long operative time with negligible postoperative pain compared to the classic open pyeloplasty in infants. The exact incision site must be reconfirmed intraoperatively by physical examination or renal ultrasonography., (Copyright © 2011 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objectives: To document the change in anterio-posterior pelvic diameter (APPD), cortical thickness (CT) and pelvis/cortex ratio (P/C ratio) following pyeloplasty, and determine the usefulness of each of these parameters in assessing postoperative outcome., Patients and Methods: Twenty-four children with unilateral pelvi-ureteric junction obstruction who underwent standard dismembered pyeloplasty with stent were prospectively studied (median age 13 months; range 2 months-5 years). Maximum APPD, CT and P/C ratio values were compared pre- and postoperatively., Results: Mean APPD was 40.70 mm preoperatively, and 30.04 mm at 3 months and 12.75 mm at 1 year postoperatively. The reduction in size at 3 months was not significant (P = 0.34), but was significant at 1 year (P < 0.01). Mean CT was 4.41 mm preoperatively, 6.29 mm at 3 months and 9.45 mm at 1 year. The increase in CT was not significant at 3 months (P = 0.44), but was significant at 1 year (P < 0.01). The P/C ratio was 13.48 mm preoperatively, 5.73 mm at 3 months and 1.45 mm at 1 year. The reduction in P/C ratio was significant at 3 months (P < 0.01) as well as at 1 year (P < 0.01). Sonographic resolution rate did not correlate with outcomes on radionuclide scans., Conclusion: P/C ratio could be used as an early marker of success following pyeloplasty. Improvement in other sonographic criteria or on radionuclide scans may be useful only in the long term., (Copyright © 2009 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Laparoscopic dismembered pyeloplasty for ureteropelvic junction (UPJ) obstruction is considered to be a routine procedure in many pediatric surgical centers. UPJ obstruction is known to be associated with horseshoe kidney and several reports on successful laparoscopic repair in such cases exist. The case of a 9-month-old girl with Turner syndrome is reported. A horseshoe kidney with grade 4 hydronephrosis on the left side was diagnosed by ultrasound during the neonatal period. MAG3 diuretic renography and dynamic magnetic resonance imaging nephrography revealed a differential renal function of 31% and 69% on the left and right side, respectively. No drainage from the left renal pelvis could be demonstrated. Laparoscopy showed a combined UPJ obstruction and a calyceal diverticulum with a narrow infundibulum of the upper pole calices on the left side of the horseshoe kidney. Laparoscopic dismembered pyeloplasty and an additional infundibulopelvic anastomosis was performed. No intraoperative complications occurred. The immediate postoperative course was uneventful. Unobstructed drainage and stable differential renal function on the left side could be demonstrated on MAG3 diuretic renography 6 weeks postoperatively. In conclusion, laparoscopic repair of complex malformations of the upper urinary tract is feasible and leads to good functional outcome in selected cases., (Copyright © 2010 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Aim: To investigate histopathological changes in ureteropelvic junction obstruction (UPJO) from an etiological perspective., Patients and Methods: Medical 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., Results: Control 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., Conclusions: High 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., (Copyright © 2009 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Ureteropelvic junction obstruction with concomitant ureterovesical junction obstruction is a rare condition whose treatment remains challenging [1-3]. Renal ultrasonography and diuretic renogram are universally utilized to assess a patient prior to pyeloplasty; however, the role of retrograde pyelography at the time of pyeloplasty is still debatable [5-7]. Herein, we describe two cases where the use of retrograde pyelography preoperatively helped to avoid pyeloplasty failure by allowing the surgeon to visualize a concurrent ureterovesical junction obstruction, which would not have been visible in the surgical field had the procedure been carried out without intraoperative imaging., (Copyright © 2009 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Maternal ultrasound is a routine examination in prenatal evaluation. The number of fetal abnormalities detected has been increasing, and includes fetal hydronephrosis which is secondary to ureteropelvic junction obstruction in 80% of cases. We report a case of a 1-year-old female infant with hydronephrosis prenatally detected by ultrasound. After close postnatal follow-up and diagnosis, she eventually underwent a definitive reconstructive procedure. This is the first reported case of a bilateral ureteropelvic junction obstruction of the upper moieties of a duplex collecting system.

Objective: Despite worldwide availability of prenatal ultrasound, many patients are diagnosed in adult life with congenital anomalies such as ureteropelvic junction obstruction (UPJO), undescended testicle (UDT), ureterocele, hypospadias, vesicoureteral reflux (VUR) and primary obstructing megaureter (POM). The aim of this review was to describe these clinical conditions and their suggested management based on the available medical literature., Review: Adult UPJO is not a rare condition; symptomatic patients should be treated rather than observed. Treatment options are nephrectomy for non-functioning kidneys and reconstructive surgery for functioning renal units. The adult UDT has low fertility potential and increased cancer risk; hence most of the data in the literature indicate performing an orchiectomy. Adult ureteroceles are usually related to single systems and they are intravesical and less obstructive. For symptomatic patients endoscopic incision showed high efficacy for symptom elimination with minimal side effects. Primary hypospadias correction in the adult patient is feasible, but success rates are low compared to the pediatric age group. Secondary correction, whether primary correction was performed in childhood or adulthood, is a challenging task with a high complication rate. Treatment decisions regarding adult patients with VUR are difficult to make as the available data are inconsistent; there is no strict evidence that reflux in an adult is directly related to renal growth impairment, ascending pyelonephritis, and/or embryo loss in a pregnant woman. In contrast to the pediatric age group, adult POM is usually a symptomatic condition and related to a high complication rate including infections, stone formation and renal failure. Spontaneous resolution is rare and hence active surgical management is advocated., Conclusion: Congenital urological anomalies identified in adulthood are not rare and pose a management challenge to the urologist. For most of the reviewed diseases, evidence-based management direction is difficult due to a lack of randomized trials and long-term follow up.

Background: Ureteric replacement in part or in total is rarely needed in children. We present our experience in using the appendix to replace the ureter., Methods: A retrospective case note review was carried out at Sheffield Children's Hospital (UK), Ekta Institute of Child Health (Raipur, Chhattisgarh, India) and Christian Medical College Hospital (Vellore, India) of all cases of ureteric substitution using the appendix., Results: Ten patients were identified, operated in 2002-2007: seven males and three females with a median age of 2.5 years (range 2.5 months to 12 years). The reasons for ureteric replacement were traumatic ureteric avulsion (n=1), congenital ureteric stenosis (n=5), non-drainage following previous pyeloplasty for pelvi-ureteric junction obstruction (n=3) and ureteric stricture following reimplantation for vesico-ureteric reflux (n=1). The appendix was used in an anti-peristaltic manner in all cases, and in one case a transureteroureterostomy was performed. At a median follow up of 16months (1-72 months), all the patients were well except one whose kidney function had deteriorated., Conclusions: Total or partial replacement of the ureter using the appendix, even in the first year of life, preserved renal function in nine cases. Ureteric continuity can be successfully restored in children using the appendix.

Objective: The ability to predict surgically relevant fetal renal pyelectasis is limited. We sought to determine whether the intrauterine timing of prenatal pyelectasis can predict the need for postnatal surgery., Methods: We retrospectively reviewed all patients with ultrasound measurements of the fetal renal pelvis during the 2nd and 3rd trimesters and postnatally. Pyelectasis was defined as >7mm for renal pelvis antero-posterior diameter in the 2nd trimester and >10mm in the 3rd trimester., Results: Fifty-nine patients out of 2397 candidates met the criteria for inclusion. A total of 116 kidneys were analyzed independently. Second trimester pyelectasis was associated with grade of postnatal hydronephrosis but not the need for surgery. Third trimester pyelectasis was associated with both postnatal hydronephrosis and surgery. The positive and negative predictive values for postnatal surgery were respectively 18% and 95% in the second trimester, and 27% and 100% in the third trimester., Conclusions: Third trimester ultrasound for fetal pyelectasis was better able to predict surgically relevant hydronephrosis than second trimester ultrasound.