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Objective: To explore the therapeutic value of laparoscopic ureteroureterostomy compared to single ureteral bladder reimplantation in the treatment of pediatric complete renal duplication., Methods: This retrospective study included 80 pediatric patients with complete renal duplication who underwent surgical treatment at the First Affiliated Hospital of Zhengzhou University from January 2015 to December 2022. Patients were divided into two groups based on the surgical approach: the laparoscopic ureteroureterostomy group (LUU group, n = 45) and the single ureteral bladder reimplantation group (UR group, n = 35). The two groups were compared in terms of operative time, intraoperative blood loss, number of stent placements, postoperative length of hospital stay, changes in ante-posterior diameter (APD) of the affected upper kidney pelvis before and after surgery, changes in upper ureteral diameter (UD), changes in upper renal cortex thickness (RCT) and variations in renal function., Results: The LUU group demonstrated significantly shorter operative time (t = 3.480, P = 0.004), less intraoperative blood loss (t = -2.465, P = 0.0196), and reduced postoperative length of stay (t = 2.308, P = 0.027) compared to the UR group. There was no significant difference between the two groups regarding the number of stent placements (x 2  = 0.762, P = 0.383). The UR group had four cases of long-term complications (two cases of anastomotic stricture, one case of vesicoureteral reflux, and one case of recurrent urinary tract infection), while the LUU group experienced one case of long-term complication (one case of anastomotic stricture), with no significant difference between groups (x 2  = 1.493, P = 0.222). Both groups showed significant improvement in preoperative and postoperative APD, UD, RCT and affected side differential renal function (DRF). However, the differences in improvement values for upper kidney pelvis APD (ΔAPD; t = -0.032, P = 0.962), upper RCT (ΔRCT; t = -0.042, P = 0.957), ureteral diameter (ΔUD; t = 1.832, P = 0.079), and differential renal function (ΔDRF; Z = 1.895, P = 0.073) were not statistically significant., Conclusion: Both LUU and UR procedures are safe and effective in treating pediatric complete renal duplication. Compared to UR, LUU results in shorter operative time, less intraoperative blood loss, and reduced postoperative length of stay, while also causing less damage to the bladder., Competing Interests: Declarations. Conflict of interest: The authors declare no competing interests., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Müllerian (paramesonephric) duct anomalies (MDA) are a rare condition, occurring in 5.5% of female newborns. One of the most complex malformations is represented by Obstructed Hemivagina and Ipsilateral Renal Anomalies (OHVIRA) syndrome, also known as Herlyn -Werner-Wunderlich (HWW) syndrome. Case presentation: We present the case of a 7-year-old asymptomatic premenarchal female patient diagnosed with OHVIRA syndrome with ipsilateral renal hypoplasia and ectopic ureteral implantation at the level of the uterus. As the patient developed urinary incontinence after the incision of the vaginal septum, right-sided 3D laparoscopic total nephrectomy was performed. Literature review: OHVIRA syndrome associated with ureteral ectopy is a rare occurrence, being encountered in 0.0064% of cases. The premenarchal diagnosis represents a challenge, due to the underdeveloped status of the genital tract. However, it should be ruled out in female newborns with unilateral renal agenesia or multicystic dysplastic kidney. Most reported cases describe the obstructed hemivagina as the site of ureteral ectopy. To our knowledge, this is the first reported case of OHVIRA syndrome with ectopic ureter draining at the level of the ipsilateral hemiuterus, diagnosed before the pubertal age. Conclusions: OHVIRA syndrome is one of the rarest Müllerian duct abnormalities. The management of these patients should be conducted in multidisciplinary teams, with long-term urological and gynecological follow-ups.

Background: Bilateral duplicated kidney and ureter is a rare condition in urology, and it is even rarer for patients to have multiple stones simultaneously. We delineate the diagnostic and therapeutic trajectory of a patient presenting with bilateral duplex kidneys and ureters, characterized by the presence of multiple stones. Notably, the left kidney is a complete duplication, whereas the right kidney exhibits an incomplete duplication., Case Presentation: A 47-year-old male patient was diagnosed with bilateral duplex kidney and ureter combined with multiple stones. Ureteral flexible lithotripsy and percutaneous nephrolithotomy were performed successively in our hospital. On the postoperative five day, he was discharged from the hospital without apparent discomfort.The double J tube was pulled out one month later, and no stone recurrence was found after 3 months of follow-up., Conclusions: Bilateral duplicated renal ureteral malformations combined with multiple stones are very rare. Stones can be removed by ureteroscopic lithotripsy or percutaneous nephrolithotripsy and sometimes multiple procedures are required, which should be chosen according to the patient's relevant condition., (© 2024. The Author(s).)

Background: Anatomical and developmental variations of ureters and renal pelvis have been observed frequently during routine human cadaveric dissection and surgical practice; however, their coexistence with accessory or aberrant renal arteries is exceptionally rare. Accordingly, this study was designed to evaluate the prevalence of anatomical and developmental abnormalities of ureters and renal pelvis existing with accessory renal arteries in human cadavers., Materials and Methods: This study was carried out on 50 human cadavers including dissected specimens (25 males and 25 females) the kidneys, renal pelvis, and ureters along with their arteries were exposed and the anomalous abnormalities of the renal pelvis and ureters existing with accessory renal arteries were observed. Photographs of the anomalous and developmental variations were taken for proper documentation., Results: Among the 50 cadavers studied, unilateral double ureters were found in 5 cadavers (10%), rare bilateral "S-"shaped loop of ureter with quadruple uretic constrictors in the abdominal segment of the ureter was observed in one female cadaver (2%), accessory or aberrant renal arteries were found in 15 cadavers (30%), hydronephrosis involving the renal pelvis and ureters was observed in 9 cadavers (18%). Interestingly, this prevalence was higher among males (28%) compared to females (8%). Moreover, the occurrence of bilateral hydronephrosis of the kidneys, renal pelvis, and ureters was identified in a single male cadaver, representing 2% of the sample. Notably, the prevalence of double ureter, hydronephrosis accompanied by congenital double and triple accessory renal arteries was documented in nine cadavers, accounting for 18% of the cohort., Conclusion: Anatomical and developmental variations of the ureters, renal pelvis, and renal vasculature, as well as their relationships to surrounding structures, hold clinical significance due to their impact on various surgical procedures, including kidney transplantation, abdominal aorta reconstruction, interventional radiology, and urologic operations. Therefore, identifying these potential developmental variations is essential for effective surgical management to preserve renal function and ensure optimal patient outcomes., (Copyright © 2024 Copyright: © 2024 Annals of African Medicine.)

Background: Crossed fused renal ectopia (CFRE) is a common congenital anomaly where one kidney is positioned abnormally on the opposite side of the midline, often fused with the other kidney. However, single ureter draining crossed fused renal ectopia is a rare occurrence., Case Report: Here, we report a case of crossed fused renal ectopia with a single ureter in a 46-year-old Nepali male who presented with history of lithuria. Computed tomography revealed that the left kidney was situated on the right side and fused with the right kidney. The renal pelvises of both kidneys were fused, and a single ureter, located on the right side, was draining both kidneys into the bladder. The patient was advised to have regular follow-ups., Conclusion: Crossed fused renal ectopia with a single ureter represents a rare renal anomaly. Asymptomatic patients can typically be managed conservatively. Regular follow-up is recommended to monitor renal function, calculus formation, infections, and malignant changes., (© 2024. The Author(s).)

Detecting ureteral injury-a serious intraoperative and postoperative complication of hysterectomy-is a high level of difficulty if ureteral malformations are not identified preoperatively. A 39-year-old Japanese woman underwent total laparoscopic hysterectomy for uterine adenomyosis. During surgery, ureteral peristalsis was absent on the right side, whereas the left ureter was visible under the peritoneum. Transabdominal ultrasonography failed to locate the right kidney, with cystoscopy showing no flow from the right ureteral orifice. The ureteral tunnel was identified by tracing the vascular path of the internal iliac and uterine arteries, confirming that the corded tissue was the right ureter. Postoperative contrast-enhanced CT revealed severe right kidney atrophy with no contrast effect or connected ureter. Based on intraoperative laparoscopy and postoperative examination, a diagnosis of a unilateral ureteral scar with a proximal blind end and ipsilateral renal dysgenesis was established, marking the first reported instance of endoscopic visualization of this anomaly. In addition to normal pelvic anatomy, surgeons must consider embryological abnormalities to prevent intraoperative complications., (© 2024 Japan Society of Obstetrics and Gynecology.)

Introduction: Duplex renal collecting system or duplex kidney (DK) commonly is associated with uropathy, with upper pole obstruction or lower pole vesicoureteral reflux (VUR). Its management is variable, ranging from therapeutic abstention to total nephrectomy. In case of damage to a single renal pole, uretero-ureteral anastomosis (UUA) is one of the surgical techniques for preserving the pathological pole. It can be performed by laparotomy, laparoscopy, or both. The aims of this study are to report the results of UUA in pathological DK, and compare outcomes depending on the surgical approach., Methods: This is a retrospective analysis study over 20 years, from April 2002 to July 2022, including all children from 0 to 15 years old who underwent UUA for a DK. Outcome measure included per- and post-operative complications, the occurrence of urinary tract infections and ultrasound measurements during follow-up., Results: Thirty-three children underwent an UUA over 20 years. The median age at diagnosis was 25 days (range: 1 day-12 years). The median age at surgery was 13 months (range: 2 months - 13 years). The sex ratio was 0.22. Prenatal diagnosis was made in 87.9 % of cases (n = 29). The left kidney was affected in 72.7 % of cases. Twenty-seven children (81.8 %) had an ectopic ureteral opening of the upper pole ureter; four children (12.1 %) had ureterocele of the upper pole and 2 children (6.1 %) had vesicoureteral reflux of the inferior pole. UUA was performed by inguinal approach (laparotomy) in 17 children (51.5 %), by laparoscopy in 9 cases (27.3 %), and by laparoscopy combined with laparotomy in 7 cases (21.2 %). The complication rate was 12.1 % (n = 4), including recurrent pyelonephritis with superior polar hydronephrosis (n = 2); iatrogenic injury of the lower pole ureter (n = 1) and a urinoma (n = 1), that all required surgical management with a polar nephrectomy. Between each surgical approach, there were no significant differences in operative time, hospital stay, complications., Conclusion: UUA is an effective therapeutic option in duplex kidneys as it allows the preservation of the pathological pole with low complication rates, regardless of the surgical approach., Competing Interests: Conflict of interest No conflicts of interest., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Renal transplantation is the best modality of treatment for patients with end-stage renal disease. Donor shortage remains a substantial problem, for which different strategies are employed, including acceptance of marginal donors and donor kidneys with anatomic variations. We performed a successful kidney transplant of a donor kidney that had complete duplication of the ureter. After transplant, the recipient had no urinary complications.

Isthmin-1 (Ism1) was first described to be syn-expressed with Fgf8 in Xenopus. However, its biological role has not been elucidated until recent years. Despite of accumulated evidence that Ism1 participates in angiogenesis, tumor invasion, macrophage apoptosis, and glucose metabolism, the cognate receptors for Ism1 remain largely unknown. Ism1 deficiency in mice results in renal agenesis (RA) with a transient loss of Gdnf transcription and impaired mesenchyme condensation at E11.5. Ism1 binds to and activates Integrin α8β1 to positively regulate Gdnf/Ret signaling, thus promoting mesenchyme condensation and ureteric epithelium branching morphogenesis. Here, we propose the hypothesis underlying the mechanism by which Ism1 regulates branching morphogenesis during early kidney development., (© 2023 The Authors. BioEssays published by Wiley Periodicals LLC.)

Purpose of Review: In this study, we describe a rare case of a male with a duplex renal collecting system with ectopic ureter insertion into the prostate who presented with sepsis and required urgent laparoscopic radical nephrectomy. This study offers urologists and emergency care practitioners a great overview of the variety of manifestations, anatomic differences, and therapeutic approaches for similar patients., Recent Findings: modified reconstructive strategy could be a method of choice when addressing particular anatomies, such as incomplete duplex kidneys with ureteropelvic junction obstruction in both upper and lower poles when the obstruction is close to the confluence of the two ureters. There is a spectrum of acute presentations and management strategies in adult patients with duplex collecting systems. The majority of patients presenting with obstruction and infection are managed surgically with hemi-nephrectomy; stable patients who present with less extensive anomalies were managed conservatively or with ureteral dilatation or deroofing., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

We report a case of a solitary kidney and a single-system ectopic ureter draining into the vestibule in an infant with a Müllerian duct defect. Due to the absence of the upper vagina and uterus, an extremely underdeveloped bladder masqueraded as the vagina, and bladder agenesis was suspected preoperatively. Urinary continence was achieved using staged bladder surgery without augmentation or urinary diversion., Competing Interests: Declaration of Competing Interest The authors have no conflict of interest to declare., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Ureteral triplication is one of the least encountered congenital malformations of the upper urinary tract. We report a case of a 37-year-old male patient with right renal ectopia with triplication of the ureter which was diagnosed via computed tomography (CT) urography. This is an intriguing example because, as we discovered after reviewing the literature, the presentation is distinctive., Competing Interests: The authors declare no competing interests., (Copyright: Sejal Sanjeev Joshi et al.)

Ureteropelvic junction obstruction (UPJO) is a congenital or acquired functionally significant impairment of urinary transport from the renal pelvis to the ureter. Congenital UPJO typically results from intrinsic disease such as the presence of an aperistaltic segment of the ureter, aberrant vessels or kidney abnormalities. Rare conditions can sometimes mimic an UPJO. We present a case of an 86-year-old woman with a UPJO diagnosed on CT. The patient was counseled on treatment options and elected to undergo a left uretherorenoscopy (URS) plus left laparoscopic pyeloplasty. The definitive histopathologic diagnosis was perinephric myxoid pseudotumor of fat, an extremely rare neoplasm, mass-forming. To the best of our knowledge, this is the first known case of a pseudotumor of fat causing UPJO. 6-month follow-up showed neither recurrence nor residual UPJO. We describe a rare presentation of extrinsic perinephric myxoid pseudotumor of fat causing UPJ obstruction. In elderly patients with no history of malignancy, UPJ obstruction can occur because of atypical masses., (© 2022. The Author(s) under exclusive licence to The Japan Society of Nephrology.)

Congenital anomalies of the kidney and urinary tract (CAKUT) are frequently associated with Mullerian anomalies. This can be explained by the fact that Mullerian duct elongation depends on the preformed Wolffian duct during embryogenesis. While CAKUT such as unilateral renal agenesis and multicystic dysplastic kidney are commonly identified prenatally by routine ultrasound, the diagnosis of Mullerian anomalies is often delayed, increasing the risk of complications such as endometriosis or pelvic inflammatory disease. Herein, we report a case of a premenarchal girl who had initially been diagnosed with right multicystic dysplastic kidney. She presented with continuous urinary incontinence at 4 years old and further evaluation by contrast-enhanced computed tomography, cystoscopy, colposcopy, ureterography, and hysterosalpingography led to the final diagnosis of right hypodysplastic kidney and ectopic ureter associated with bicornuate uterus. A strong family history of uterine malformations prompted the examination of the uterus. Genetic testing was suggested but the family declined. She is planned to be referred to a gynecologist at puberty for further assessment. The recognition and screening rate of concurrent Mullerian anomalies in CAKUT patients varies between institutions. Screening for Mullerian anomalies in prediagnosed CAKUT girls may enable to provide timely counseling and to prevent gynecological complications., (© 2022. The Author(s) under exclusive licence to The Japan Society of Nephrology.)

Background: Retrocaval ureter is a rare congenital anomaly resulting from anomalous development of inferior vena cava (IVC) and not from anomalous of the ureter. The anomaly always occurs on the right side due to regression of right supracardinal vein and persistence of right posterior cardinal vein. Retrocaval ureter tends to be associated with various vena cava anomalies because of the embryogenesis. We aimed to identify the prevalence of associated congenital venous anomalies (CVA) resulting from cardinal vein development in adults with retrocaval ureter using computed tomography (CT) images., Materials and Methods: The study included 22 adults with retrocaval ureter. We evaluated CT findings and determined the incidence of associated CVA using thin slice data sets from CT scanner with 64 or more detectors. We compared the prevalence of CVA in the retrocaval ureter group (mean age: 57 ± 19 years) and in the control group of 6189 adults with normal ureter (mean age: 66 ± 14 years)., Results: In the retrocaval ureter group, 4 (18.2%) adults had CVA including double IVC, right double IVC, preisthmic IVC with horseshoe kidney, and preaortic iliac confluence. One of 2 adults with preaortic iliac confluence had right double right IVC. In the control group, 49 (0.79%) adults had CVA including 37 double IVC, 11 left IVC, and 1 IVC interruption azygos continuation. Fifteen horseshow kidneys were found. The prevalence of associated CVA in the retrocaval ureter group was higher than that in the control group (p < 0.001)., Conclusions: Retrocaval ureter is frequently associated with CVA. Various CVA with retrocaval ureter could happen because of abnormal development of not only the right posterior or supra cardinal vein but also other cardinal veins.

Introduction: Duplex collecting system is a common congenital urinary system anomaly and is usually asymptomatic. Vascular variations associated with a duplex system are common but haven't been reported as obstructive., Case Report: 14-month-old female had a right sided incomplete ureteral duplication complicated by lower pole hydroureteronephrosis due to distal ureteral obstruction by an aberrant vessel crossing the bifid ureters at ureteric junction of these bifid ureters. Prenatal imaging detected right hydronephrosis. Magnetic resonance suggested a diagnosis of duplicated ureters. At surgery, an aberrant artery compressed the lower moiety ureter at the bifid ureteric junction. The stenotic section was resected and ureter segments were anastomosed. The occluding small artery was not resected to preserve vascularization., Discussion/conclusion: An anatomical vascular variation can cause proximal ureteral dilatation and segmental hydronephrosis in a bifid system.

Introduction: Obstructed hemivagina ipsilateral renal agenesis (OHVIRA) is a rare syndrome with limited data on both treatment and postoperative sequelae, specifically of ectopic ureters to the vagina., Case Description: This case study describes a 22-year-old patient with OHVIRA syndrome presenting with pelvic pain and drainage from a chronic vaginal abscess secondary to a remnant distal ectopic ureter after nephrectomy and proximal ureterectomy. Imaging was significant for a right paravaginal abscess. Previously, the patient was treated with conservative therapy and on presentation her evaluation confirmed a right paravaginal abscess. She subsequently underwent a robot-assisted incision and drainage of the paravaginal abscess and excision of the remnant distal ectopic ureter. She did well postoperatively without recurrence., Discussion: We discuss the successful surgical technique used to identify and excise a paravaginal abscess. We also highlight the unique anatomy of a patient with OHVIRA syndrome. Lastly, we underline the importance of a complete resection of an ectopic ureter to the vagina at the time of nephrectomy, given the potential risk of ascending chronic infection and recurrent abscess formation., Competing Interests: Conflict of interests: none., (© 2022 by SLS, Society of Laparoscopic & Robotic Surgeons.)

The duplex kidney is one of the common congenital anomalies of the kidney and urinary tract. Meanwhile, renal cell carcinoma is also the most typical variant constituting more than 90% of cancers of the kidneys. However, renal cell carcinoma is rare in a duplicated collecting system, and literature regarding its association is very sparse. Herein, we present a 28-year-old male patient who presented with abdominal pain and hematuria and was diagnosed with left upper pole renal cell carcinoma coexisted with a duplicated collecting system by computed tomography scan. The patient underwent a left partial nephrectomy followed by meticulous suturing of opened pelvicalyceal system. The histopathology result showed clear cell carcinoma. The postoperative period was uneventful, and the patient was discharged without complications. In conclusion, the coexistence of the duplex system and renal cell carcinoma in the ipsilateral kidney is rare and maybe more than a coincidence, requiring a deeper insight and further elucidation., Competing Interests: The authors declare no competing interests., (Copyright: Rajesh Domakunti et al.)

A 1-year-old female spayed French Bulldog was referred to the Interventional Radiology and Endoscopy Service for evaluation of urinary incontinence with partial response to medical management (phenylpropanolamine). Cystourethroscopy and retrograde ureteropyelogram were performed and revealed multiple congenital abnormalities including; a duplex renal system of the left kidney with one normal ureter with an intravesicular ureteral orifice and one extramural ectopic ureter with an associated branching and ureteral diverticulum. A dual vagina was also noted. Left neoureterocystostomy of the extramural ectopic ureter was performed and a temporary ureteral stent was concurrently placed and removed cystoscopically one month later, leaving the patient with two patent left ureters. Thirty-four months later the patient is mildly incontinent with no other comorbidities., (© 2021 British Small Animal Veterinary Association.)

Introduction: Zinner syndrome is a rare congenital abnormality defined by a clinical triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction., Case Presentation: Most patients are asymptomatic, but if the cystic dilatation of the seminal vesicle becomes significant, it can result in urinary symptoms such as dysuria and urinary retention. This rare developmental anomaly related to mesonephric duct can also present with other abnormalities., Conclusion: Here, we report our experience of Zinner syndrome with bladder outlet obstruction and an ectopic ureter remnant., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Abstract: Chapman and Hellstrom techniques are typically employed to transpose renal lower pole crossing vessels (LPCVs). Both procedures have certain limitations. We investigated the midterm outcomes in pediatric patients in whom LPCV-induced ureteropelvic junction obstruction was treated with either dismembered Anderson-Hynes pyeloplasty or upward transposition coupled with a new technique to fix the LPCV.We retrospectively compared Anderson-Hynes pyeloplasty to the new technique in terms of outcome. LPCV transposition was considered feasible in patients in whom the diuretic loading test revealed a decrease in the pelvic volume after correction of vascular compression as well as absence of structural changes in the ureteropelvic junction (UPJ) and hemodynamic compromise of the lower renal pole. The fascial flap was passed below the LPCV to form a "hammock". The free edge of the flap was sutured to its base.Group 1 consisted of 102 (69.9%) patients (median age: 7.9 years) undergoing dismembered Anderson-Hynes pyeloplasty, while group 2 included 44 (30.1%) patients (median age: 8.4 years) treated with upward transposition and the new technique to fix the LPCV. No intra-operative complications or conversions occurred in either group. Redo-pyeloplasty was performed in 3 (2.9%) children of group 1 and 1 (2.3%) child of group 2. Renal ultrasonography conducted 12 months after surgery revealed similar anteroposterior diameters of the renal pelvis in groups 1 (7.9 ± 8.1 mm) and 2 (6.0 ± 2.9 mm). Patients in both groups showed a non-significant median increase in differential renal function at follow-up after at least 1 year after surgery (group 1: 36% [33.3; 40.5] vs 36.5% [35.3; 41.0]; group 2: 41% [37.5; 46.0] vs 43% [39; 46]).In our patients, the new technique for laparoscopic or open fixation of the obstructing vessel after transposition was effective, reproducible, and devoid of limitations typical for the Chapman and Hellstrom techniques. We recommend Anderson-Hynes pyeloplasty in children with a history of hydronephrosis diagnosed antenatally, recurrent abdominal pain, intra-operative absence of peristalsis across the UPJ, high location of the UPJ at the renal pelvis, or intra-operative absence of volume reduction of the renal pelvis upon furosemide testing., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Seminal vesicle cysts are usually congenital and frequently accompanied by upper urinary tract abnormalities due to mesonephric duct maldevelopment. Zinner syndrome, first described in 1914, refers to a triad of features consisting of seminal vesicle cysts, ejaculatory duct obstruction, and unilateral (mostly ipsilateral) renal agenesis. We herein present four pediatric patients with Zinner syndrome diagnosed at a children's medical center. A remnant ureteral structure was observed in three (75%) patients. Interestingly, a multicystic dysplastic kidney was present in one (25%) patient before it eventually disappeared. These findings suggest possible involvement of renal dysgenesis rather than agenesis in Zinner syndrome., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Competing Interests: Competing interests: None declared.

Development of the mammalian kidney is orchestrated by reciprocal interactions of stromal and nephrogenic mesenchymal cells with the ureteric bud epithelium. Previous work showed that the transcription factor Wilms tumor 1 (WT1) acts in the nephrogenic lineage to maintain precursor cells, to drive the epithelial transition of aggregating precursors into a renal vesicle and to specify and maintain the podocyte fate. However, WT1 is expressed not only in the nephrogenic lineage but also transiently in stromal progenitors in the renal cortex. Here we report that specific deletion of Wt1 in the stromal lineage using the Foxd1 cre driver line results at birth in cryptorchidism and hypoplastic kidneys that harbour fewer and enlarged ureteric bud tips and display an expansion of capsular stroma into the cortical region. In vivo and ex vivo analysis at earlier stages revealed that stromal loss of Wt1 reduces stromal proliferation, and delays and alters branching morphogenesis, resulting in a variant architecture of the collecting duct tree with an increase of single at the expense of bifurcated ureteric bud tips. Molecular analysis identified a transient reduction of Aldh1a2 expression and of retinoic acid signalling activity in stromal progenitors, and of Ret in ureteric bud tips. Administration of retinoic acid partly rescued the branching defects of mutant kidneys in culture. We propose that WT1 maintains retinoic acid signalling in the cortical stroma, which, in turn, assures proper levels and dynamics of Ret expression in the ureteric bud tips, and thus normal ramification of the ureteric tree. © 2020 The Authors. The Journal of Pathology published by John Wiley & Sons, Ltd. on behalf of The Pathological Society of Great Britain and Ireland., (© 2020 The Authors. The Journal of Pathology published by John Wiley & Sons, Ltd. on behalf of The Pathological Society of Great Britain and Ireland.)

Ectopic ureter is a rare but pertinent cause of incontinence in young women. We report a 12-year-old girl who presented with reports of incontinence since birth. She was evaluated and found to have complete duplication of the left ureter, with the upper moiety ureter opening into the vestibule of the vagina just below the external urethra meatus. She was managed surgically by dismembered extravesical reimplantation of the upper moiety ureter instead of the conventional method of common sheath reimplantation, sparing the patient a wide cystostomy and intravesical dissection. One year postsurgery, the patient is asymptomatic and dry. Dismembered reimplantation of the ectopic ureter is a simple and reproducible technique which avoids manipulation of the normal lower moiety ureter and its associated potential complications. At the same time, it ensures that the patient is dry without any adverse effect on the lower moiety or its ureter., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Kidney triplication is a rare urological abnormality. Association of triplex kidney and ureterocele is out of ordinary. Treatment of such patients usually implies heminephrureterectomy of the upper moiety. We report a case of a saved function of the upper moiety after minimal invasive surgical procedure., Case Presentations: 5-year old girl complained for continuous wetting. Examination revealed 3 - segmented left kidney with pelvi-ureteric dilation of the upper moiety, IV grade vesicoureteral reflux in the upper moiety, cervical ectopic ureteral orifice of the upper moiety and a commune ureteral orifice of the lower segments. An endoscopic laser dissection of ureterocele was performed. Drainage of the upper moiety of triplex kidney was restored. Examination 18 months later showed no wetting and infection symptoms. Pelvi-ureteric dilation of the upper moiety and cavity of ureterocele decreased to minimal. Grade of vesicoureteral reflux decreased to I., Conclusion: Minimal invasive elimination of obstruction of the upper moiety of triplex kidney was successful and led to regress of vesicoureteral reflux, urinary incontinence and let to avoid heminephrectomy.

Duplicated collecting systems with minimal-to-no function of the upper moiety are often treated surgically with upper pole heminephrectomy and ureterectomy. Complications associated with the ureteral stump can occur and are collectively known as ureteral stump syndrome. We present a case of ureteral stump syndrome treated with percutaneous transgluteal access into the stump which aided in identification of an ectopic ureteral opening that could not be identified by surgical means alone., (Copyright © 2019 Elsevier Inc. All rights reserved.)

A 5-year-old girl underwent an elective left pyeloplasty for pelvoureteric junction obstruction (PUJO). At laparoscopy, two ureters were seen to be draining a left duplex kidney, with a PUJO of the lower moiety. Interestingly, however, the upper moiety ureter was bifurcating to drain both upper and lower moiety as well. All the preoperative investigations were suggestive of a single system. The ureteric bifurcation of this type has not been reported so far. It is of paramount importance to demonstrate anatomy clearly at an operation to prevent risk to the less defined ureters., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

A 5-month-old, male French bulldog was presented with a history of urinary incontinence. Abdominal ultrasound showed changes compatible with a ureterocele and a bilobed right kidney. Excretory computed tomographic urography was consistent with right-sided duplex kidney, ureter duplex and ectopic ureterocele. To the authors' knowledge, this is the first description of a duplex kidney with an ectopic ureterocele diagnosed with ultrasound and contrast enhanced computed tomography in a dog. After the imaging diagnosis, a neoureterocystostomy was performed. In the follow-up examination the dog presented with mild incontinence which was treated medically using phenylpropanolamine., Competing Interests: The authors confirm that they do not have any conflict of interest., (© Georg Thieme Verlag KG Stuttgart · New York.)

In case of stricture of the lower third of ureter in patients with duplex kidney, ureteroneocystostomy or transureteroureterostomy can be used. Anastomosis of the injured ureter of the upper pole with the pelvis of the lower pole is considered nontypical. In this clinical observation, a 46-year-old patient with iatrogenic trauma of the pelvic part of the upper pole of a duplex kidney successfully undergone laparoscopic ureteropyelostomy. There were no complications; the stent was removed after 4 weeks. During the follow-up examination, the function of both kidneys was within normal range and there were no disturbances in the urine passage in the upper urinary tract. Thus, laparoscopic ureteropyelostomy provided an adequate urine passage from the duplex system kidney through the healthy ureter.

Purpose: The literature about ectopic ureters in anorectal malformations is limited. Repair of an anorectal malformation may require dissection near the normal or abnormal insertion of the ureters. Knowledge of the presence and location of ectopic ureters may prevent intraoperative injury. We aim to describe the incidence and location of ectopic ureters in patients with anorectal malformations and to characterize associated renal anomalies., Methods: This is an IRB-approved retrospective study of patients with anorectal malformations and ectopic ureters identified in our colorectal database., Results: Of 2283 patients with anorectal malformation, 79 (3.5%) had ectopic ureter(s). Of those, 29% had bilateral ectopic ureters. Nearly all (87%) of bilateral ectopic ureters occurred in females. Ectopic ureters most commonly inserted into the bladder neck (33%), vagina (15%), or urethra (13%). Renal dysfunction was noted in a high proportion of patients. The majority (59%) of ectopic ureters were associated with dysfunction of the ipsilateral kidney. Interestingly, 29% of patients with unilateral ureteral ectopia had an abnormal contralateral kidney. Only 22 patients (28%) had two normal kidneys, and 5 (6%) had documented renal failure with 2 (2.5%) requiring renal transplant. This compares to a transplant rate of 0.6% among anorectal malformation patients without ectopic ureter., Conclusions: The incidence of ectopic ureter is 3.5% among anorectal malformation patients. Cloaca and recto-bladder neck fistula are the types of anorectal malformation with higher incidence. Ureteral ectopia seems to confer an increased risk of renal failure. Identifying ectopic ureters is important for surgical planning and monitoring renal function vigilantly.

Crossed renal ectopia is a rare congenital abnormality, in which the two kidneys are located in the same side. One of the ureters, whose length is adapted to the kidney seat, crosses the median line to implant in the bladder on the opposite side. Renal fusion of the two parenchymas is common and this abnormality is most often asymptomatic and fortuitously discovered. We report the case of a 36-year old patient with no previous pathological history, presenting with crossed renal ectopia incidentally discovered after acute cholecystitis complicated by perivescicular abscess. On the basis of this observation and of the data from the literature, we here discuss the etiopathogenic, clinical, radiological and therapeutic features of this malformation., Competing Interests: Les auteurs ne déclarent aucun conflit d'intérêts., (© Anass Elbouti et al.)

Objective: The objective of this video is to illustrate feasibility of the ureteral ligation for the treatment of massive ureteroceles associated with non-functioning upper kidney moieties in duplex kidneys., Materials and Methods: In this video, one case is presented to demonstrate this novel technique. A nine-year-old boy presented with progressive lower urinary tract symptoms. Radiological workup depicted a duplex kidney on the left side with the absence of function on the upper pole and hydroureteronephrosis with a massive ureterocele. Laparoscopic ureteral transection, drainage, and ligation of both extremities of the enlarged ureter (upper pole ureter) were performed., Results: Immediately after ureteral ligation, ultrasonic evidence of ureterocele decompression and improved hydroureteronephrosis was observed. This patient remained asymptomatic without postoperative complications after 3 years. De novo reflux was not observed., Conclusion: Ureteral ligation, as demonstrated in the video, is a good approach for the treatment of massive ureteroceles associated with non-functioning upper kidney moieties in duplex kidneys. Compared with the current standard techniques (incision/puncture), ureteral ligation has the clear advantage of not causing 'de novo reflux' or unsuccessful de-obstruction, and is not as surgically demanding as other reconstructive/ablative techniques. The disadvantage is the need of regular clinical and ultrasonographic follow-up., (Copyright © 2019 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

In the article a rare case of structural anomaly of ureter, namely obliteration, in 1-year-old infant is reviewed. The obliteration was located in the distal part of the left ureter. There was urine leakage through dilated and inflamed wall of the ureter towards right gluteal region with a formation of retroperitoneal abscess. A diagnosis was established based on preoperative MRI and intraoperative antegrade pyeloureterography. Extravesical Lich-Gregoir ureterocystoneostomy was successfully performed.

Introduction: Failure of kidney migration during embryonic life results in an ectopic kidney, with an incidence varying from 1 in 500 to 1 in 1200. Pelvic kidney can be a rare cause of recurrent urinary tract infection (UTI), warranting nephrectomy in some cases., Material and Methods: A 7-year-old girl with a history of recurrent UTIs and vaginal discharge was diagnosed with a dysplastic afunctional ectopic kidney located in the pelvis, with ureter draining into the cervix or vaginal wall., Results: A robot-assisted approach was chosen, with side docking of the robot to allow concurrent vaginoscopic/hysteroscopic exploration. A uterus bicornis was found. The ectopic ureter was dissected toward its drainage in the vaginal wall, where it was sutured and resected. The dysplastic kidney and ureter were removed. Compared to pure laparoscopic approach, dissection deep into the pelvis toward the vaginal wall is aided by the robotic dexterity and facilitates complete resection of the structure, which avoids leaving a ureteric stump into the vaginal wall., Conclusions: Robot-assisted surgery is a safe and effective option for resection of ectopic kidneys with ectopic ureter in children. Dissection up until the vaginal wall in children is aided by robotic dexterity, making this kind of surgery the ideal indication for robotic-assisted surgery., (Copyright © 2018 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Aims: (1) to identify the most dysregulated genes in ureter tissue affected by congenital anomalies of the kidney and urinary tract (CAKUT) and to extract the biological meaning of these markers; (2) to describe the key molecular networks in CAKUT and to provide expression validation of the genes selected from these networks., Main Methods: Transcriptome data was obtained from ureter samples of CAKUT patients and controls by Illumina iScan microarray. Identification of differentially expressed genes was coupled with subsequent bioinformatics analyses. Expression of candidate genes was validated by qRT-PCR., Key Findings: Analysis of the transcriptome led to the identification of 78 commonly dysregulated genes in CAKUT tissue compared to controls. Integrative bioinformatic analyses of differentially expressed genes identified 7 major networks. The targets for qRT-PCR validation were selected as members of the major molecular networks in CAKUT, which had both, the significant high fold change and biological relevance for CAKUT. By qRT-PCR the substantial increase of LCN2, PROM1, SOSTDC1, and decrease of INA, RASD1 and TAC3 mRNA levels was confirmed., Significance: Since CAKUT is a leading cause of end-stage renal disease in children, the search for molecular targets for postnatal therapy is of particular interest. Data described in this study represents the gene expression profile and significant molecular networks specific to human ureter affected by CAKUT. The discovery of impaired molecular factors and processes is the step towards the uncovering of the key mechanisms that reflect CAKUT postnatally and could lead to the affected tissue deterioration and end organ damage., (Copyright © 2018 Elsevier Inc. All rights reserved.)

The pancake kidney (PK) is a rare type of renal anomaly in which both kidneys completely fuse without an isthmus. In the previous reports, PKs have double ureters and are located in the pelvic cavity. We encountered a rare case of PK with a single ureter, which is located in the left retroperitoneal space, in a 95-year-old female cadaver, which was detected during a dissection course. In our case, the major calyces joined to form a single renal pelvis, which continued as a single ureter. To the best of our knowledge, this is the first report on PK with a single ureter that is located not in the pelvic cavity but in the retroperitoneal space. The knowledge of such anomalous presentation is important to avoid any complications during retroperitoneal surgery.

Congenital abnormalities of the kidney and the urinary tract (CAKUT) belong to the most common birth defects in human, but the molecular basis for the majority of CAKUT patients remains unknown. Here we show that the transcription factor SOX11 is a crucial regulator of kidney development. SOX11 is expressed in both mesenchymal and epithelial components of the early kidney anlagen. Deletion of Sox11 in mice causes an extension of the domain expressing Gdnf within rostral regions of the nephrogenic cord and results in duplex kidney formation. On the molecular level SOX11 directly binds and regulates a locus control region of the protocadherin B cluster. At later stages of kidney development, SOX11 becomes restricted to the intermediate segment of the developing nephron where it is required for the elongation of Henle's loop. Finally, mutation analysis in a cohort of patients suffering from CAKUT identified a series of rare SOX11 variants, one of which interferes with the transactivation capacity of the SOX11 protein. Taken together these data demonstrate a key role for SOX11 in normal kidney development and may suggest that variants in this gene predispose to CAKUT in humans., (Copyright © 2017 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.)

Introduction: The duplex kidney is one of the most common congenital abnormalities of the urinary tract, and various surgical procedures exist for the management of this condition. Depending on the surgeon preferences, patients with duplex kidney may undergo heminephrectomy with ureterectomy using open or laparoscopic approach, uretero-cysto-anastomosis of the ectopic ureter or two ureters in a single block. Recently, there have been reports of inter-ureter anastomoses. Here we report our multicenter experience in open and laparoscopic uretero-ureteral anastomosis (UUA). The study aimed to improve the treatment results in children with urodynamic dysfunction due to the duplicated upper urinary tract., Materials and Methods: We retrospectively analyzed medical records of 64 children treated from 2007 to 2017. There were 22 (32.8%) boys and 43 (67.2%) girls with mean age 40.2 months at the time of surgery. All of them had duplex kidneys, including 27 (42.2%) right-sided and 37 (57.8%) left-sided. Of them, 15 (23.4%) children underwent distal UUA (DUAA), and 49 (76.6%) had proximal UUA (PUUA)., Results: After surgery, acute pyelonephritis occurred in three patients. In the early postoperative period, a prolonged urinary leakage from surgical site drainage was observed in three (6.1%) patients after laparoscopic PUUA. One (4.2%) child developed a stumpitis. Thus, complications occurred in 7 (10.9%) children; one of them (1.6%) needed additional treatment., Conclusion: Distal and proximal UUA is a safe and effective surgical treatment for urinary outflow disorders in patients with the duplicated upper urinary tract, minimizing the risk of the duplex kidney dysfunction.

Purpose: A growing body of evidence suggests that it is safe to ligate the ureter of poorly functioning renal moieties during renal transplantation. We present clinical outcomes and data on hydronephrosis progression in pediatric cases associated with ectopic ureters and obstructive ureteroceles., Materials and Methods: We prospectively collected data for 35 consecutive patients (23 females and 12 males) who underwent ureteral clipping between February 2011 and August 2016. Patients were divided into 4 groups consisting of 1) duplex system with ectopic ureter (45.7%), 2) duplex system with a large ureterocele (11.4%), 3) other duplex system (8.6%) and 4) single system kidneys (34.3%). Patients were followed for clinical outcomes and hydronephrosis trends. Comparisons included preoperative and postoperative anteroposterior diameter, maximal ureteral diameter and ureterocele size., Results: Median age at surgery was 59 months (IQR 11 to 120, range 5 to 216). Median ± SD operative time was 108.9 ± 31.1 minutes (range 20 to 180) and median length of stay was 7.5 hours (IQR 6 to 19, range 5 to 336). Immediate resolution of urinary incontinence was observed in all 16 ectopic ureter cases. After a median ± SD followup of 20.8 ± 13.8 months (IQR 8.5 to 30, range 6 to 50) 97.2% of the patients remained asymptomatic. No significant differences were observed between initial and last anteroposterior diameter measurements except in group 1 (p = 0.001). All ureteroceles demonstrated a significant decrease in median ± SD size after clipping (from 2.7 ± 0.41 to 0.53 ± 0.92 cm, p = 0.003). Pyonephrosis developed in 1 patient, who underwent laparoscopic nephrectomy., Conclusions: Ureteral clipping appears to be a reasonable, safe and effective option for pediatric patients in the reported settings, with the potential to be simpler and quicker than extirpative or reconstructive procedures., (Copyright © 2018 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Objectives: This study was performed to analyze the predictive factors of a contralateral operation after initial pyeloplasty in patients with antenatally detected bilateral ureteropelvic junction obstruction., Methods: Patients with prenatally diagnosed bilateral ureteropelvic junction obstruction who underwent initial pyeloplasty (aged <12 months at initial pyeloplasty) were offered to participate in the study. Patients were recruited from January 2012 to December 2015. The anteroposterior renal pelvic diameter, parenchymal thickness, and calyceal dilatation were evaluated. Predictive factors of contralateral pyeloplasty after initial unilateral pyeloplasty were also examined., Results: In total, 82 patients were included in the study (mean age, 2.8 months). Among all patients who underwent initial pyeloplasty, additional contralateral pyeloplasty was required in 11 patients (13.4%). The outcome of contralateral hydronephrosis was assessed as resolution, persistence, or surgery. The median anteroposterior renal pelvic diameter and calyceal dilatation were significantly different among the groups (p < 0.001). Calyceal dilatation of ≥10 mm and a calyceal dilatation/parenchymal thickness ratio of ≥5 strongly suggested the likelihood of a contralateral operation., Conclusions: In most patients with bilateral ureteropelvic junction obstruction, improvement or resolution of contralateral hydronephrosis following initial unilateral pyeloplasty can be expected. Patients with contralateral calyceal dilatation >10 mm and the calyceal dilatation/parenchymal thickness ratio >5 are at higher risk of surgery., (© 2018 S. Karger AG, Basel.)

Objective: To determine if the histopathology of the ureteropelvic junction differs between those with and without crossing vessels who present with a ureteropelvic junction obstruction (UPJO)., Materials and Methods: Our database was queried for patients undergoing pyeloplasty (Current procedural terminology 50400, 50405, and 50544) between June 1, 2002, and March 10, 2016. We excluded patients with other renal anatomic abnormalities and patients undergoing surgery for upper tract tumor. One genitourinary pathologist reviewed all slides for muscle hypertrophy, fibrosis, edema, and inflammation. Statistics were modeled in SAS 9.4 (SAS, Cary, NC) using logistic regression with maximum likelihood., Results: A total of 178 patients met the inclusion criteria, and pathology slides were available for 136 patients. Thirty-three patients had crossing vessels in association with a UPJO. The degrees of muscle hypertrophy (P = .89) and fibrosis (P = .17) were not predictive of etiology. The odds of a crossing vessel increased by 4.3 times (95% confidence interval 1.8-9.9) when edema was present (P = .009) and by 4.4 (95% confidence interval 1.4-13.7) times when inflammation was present (P = .0103)., Conclusion: In the largest pathology series to date, histopathology showed increased inflammation in the presence of a crossing vessel but a similar composition of muscle and fibrosis. These data suggest that UPJO with an associated lower-pole vessel may represent a chronic process, which would explain why patients with a crossing vessel present later in life., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: The aim of this study was to present a novel laparoscopic technique for persistent urinary incontinence in pediatrics due to ectopic ureter associated with poor functioning upper renal moiety., Methods: This technique consisted of laparoscopic clipping of the upper moiety artery and vein. The ectopic ureter was also clipped afterwards without upper pole partial nephrectomy., Results: The patient was a seven-year-old girl with persistent urinary incontinence and confirmation of duplex kidney with poor functioning upper moiety in pre-operative investigations. The upper moiety ureter was ectopically drained to the vaginal cavity. She was immediately dry after surgery and discharged on the second postoperative day. During the follow-up period of 14 months, she was continent and symptom-free. Hydronephrosis was not visualized in follow-up ultrasonography., Conclusions: This laparoscopic upper renal moiety vascular and ureteral clipping without partial nephrectomy could serve as a promising, safe and simple alternative in the treatment of patients with ectopic ureter associated with poor functioning renal moiety. Also, ipsilateral normal functioning moiety would not be associated with potential morbidity in this technique., (Copyright © 2017 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

A duplex kidney system with an ectopic ureter draining into the vagina is a congenital malformation that typically presents as refractory urinary incontinence. Diagnosis is often difficult to establish and delayed due to a low incidence. We present the case of a patient aged 26 years with a life-long history of persistent urinary incontinence. Initial presentation was at childhood; however, the diagnosis went undetermined for 22 years. CT urography revealed a duplex kidney with an atrophic upper pole associated with an ectopic ureter that drained into the vaginal vault. This is the first description of such a case being managed successfully via a robot-assisted partial nephrectomy approach. Ectopic ureteral duplication should be considered in the differential diagnosis for young women with refractory urinary incontinence. Robotic partial nephrectomy is a safe and effective technique to manage such cases., Competing Interests: Competing interests: None declared., (2017 BMJ Publishing Group Ltd.)

We report an extremely rare case of a pancake kidney with a single ureter. During the medicolegal autopsy on the body of a traffic accident victim, the kidneys were not located in the abdomen. The anterior surfaces of both kidneys were completely fused in the pelvic cavity, and the kidney was shaped like a pancake. This pancake kidney had accessory renal arteries around it and one renal vein from each kidney. The left renal vein was much smaller in diameter than the right one. There were two major calyces, one each from each kidney. The major calyces joined to form a single renal pelvis from both the kidneys, which continued as a single ureter. The renal pelvis and ureter lay posterior to the pancake kidney. Urologists and pelvic surgeons should be aware of the variant anatomy of the pancake kidney, particularly concerning its vasculature, to prevent unexpected catastrophic bleeding. If the kidneys are not located in the abdominal cavity, the autopsy surgeon must consider the possibility of its presence in the pelvic cavity. This could be especially important during medicolegal investigations into allegations of missing kidneys and unlawful kidney transplantations.

We reporting unilateral renal agenesis with ipsilateral ureterocoele, mega ureter and blind end proximal ureter in same patient first time as case report and has not been so far reported in local or international literature. Ultrasound, CT scan and intravenous pyelography performed which confirm the case. Patient presented with left lumber and pelvic pain on and off and history of recurrent urinary tract infection.