Should a Refluxing Internal Diversion Be Considered a Temporizing Procedure? Extended Follow-up and Outcomes After Side-to-Side Ureterovesicostomy for Primary Obstructive Megaureter in Young Children.

PURPOSE: We describe long-term outcomes, including UTIs and need for reimplantation, after ureterovesicostomy (UV) as a lasting surgical procedure for children with primary obstructive megaureter (POM). MATERIALS AND METHODS: Children referred to our institution between 2016 and 2023 who underwent refluxing UV were analyzed. POM was defined as hydroureteronephrosis with distal ureteral dilatation > 7 mm and a negative workup for other etiologies of hydronephrosis. We assessed for surgical outcomes, complications, rate of UTI, and improvement in upper tract dilatation. Statistical analyses assessed for change in hydronephrosis metrics over follow-up. RESULTS: Among 183 patients diagnosed with POM, 47 (24%) underwent UV. Median age of presentation, surgery, and follow-up was 2, 9, and 43 months, respectively. A total of 7 patients developed 30-day complications: Clavien-Dindo grade 1 in 2 (transient urinary retention) and grade 2 in 5 (UTIs). During monitoring 14 (30%) developed UTIs and 7 (15%) required ureteral reimplant or UV takedown. After surgery there was a significant decrease in the proportion of patients with high-grade hydronephrosis, anteroposterior renal pelvis diameter, and maximum ureteral dilatation. CONCLUSIONS: Refluxing UV is a safe alternative to cutaneous diversion in POM. Most patients had improvement in upper tract dilatation with an acceptable short-term complication rate and need for reoperation (in comparison to routine later reimplantation). Our experience suggests that monitoring alone after UV is feasible, and that selective subsequent reconstruction is a reasonable strategy.

Primary non-refluxing megaureter: Natural history, follow-up and treatment.

Primary non-refluxing megaureter (PMU) is a congenital dilation of the ureter which is not related to vesicoureteral reflux, duplicated collecting systems, ureterocele, ectopic ureter, or posterior urethral valves and accounts for 5 to 10% of all prenatal hydronephrosis (HN) cases. The etiology is a dysfunction or stenosis of the distal ureter. Most often PMU remains asymptomatic with spontaneous resolution allowing for non-operative management. Nevertheless, in selective cases such as the development of febrile urinary tract infections, worsening of the ureteral dilatation, or reduction in relative renal function, surgery should be considered. CONCLUSION: Ureteral replantation with excision of the dysfunctional ureteral segment and often ureteral tapering is the gold-standard procedure for PMU, although endoscopic treatment has been shown to have a fair success rate in many studies. In this review, we discuss the natural history, follow-up, and treatment of PMU. WHAT IS KNOWN: • PMU is the result of an atonic or stenotic segment of the distal ureter, resulting in congenital dilation of the ureter, and is frequently diagnosed on routine antenatal ultrasound. WHAT IS NEW: • Most often, PMU remains asymptomatic and clinically stable, allowing for non-operative management. • Nevertheless, since symptoms can appear even after years of observation, long-term ultrasound follow-up is recommended, even up to young adulthood, if hydroureteronephrosis persists. • Ureteral replantation is the gold standard in case surgery is needed. In selected cases, however, HPBD could be a reasonable alternative.

Disruption of Gen1 causes ectopic budding and kidney hypoplasia in mice.

Congenital anomalies of the kidney and urinary tract (CAKUT) are a family of often-concurrent diseases with various anatomical spectra. Null-mutant Gen1 mice frequently develop multiple urinary phenotypes, most commonly duplex kidneys, and are ideal subjects for research on ectopic budding in CAKUT development. The upper and lower kidney poles of the Gen1PB/PB mouse were examined by histology, immunofluorescence, and immunohistochemistry. The newborn Gen1PB/PB mouse lower poles were significantly more hypoplastic than the corresponding upper poles, with significantly fewer glomeruli. On embryonic day 14.5, immediately before first urine formation, the upper pole kidney was already larger than the lower pole kidney. In vivo and in vitro, embryonic kidney upper poles had more ureteric buds than lower poles. Gen1PB/PB embryos exhibited ectopic ureteric buds, usually near the original budding site, occasionally far away, or, rarely, derived from the primary budding site. Therefore, ectopia of the ureteric buds is the core of CAKUT formation. Further studies will be needed to investigate the regulatory roles of these genes in initial ureteric budding and subsequent ontogenesis during metanephros development.

Sonographic differential diagnosis of duplex kidneys.

Duplex kidneys have two renal pelvises and two ureters, which can join on the way to the urinary bladder but can also enter the bladder separately. A distinction must be made between normal and pathological duplex kidneys. In normal duplex kidneys, both renal pelvises are normal in width, and the upper and lower poles are approximately the same size. Furthermore, ureters are not dilated, and the upper and lower poles of the kidneys are not cystically altered. In contrast, pathological duplex kidneys, occurring in about 50 % of cases, are characterized by dilation of one or both renal pelvises. Additionally, one or both ureters may be dilated. Megaureters may be obstructive or refluxive. If a megaureter is present, a ureterocele must be ruled out, as well as an ectopically opening ureter. A pathological duplex kidney must always be assumed if one pole of the kidney is hypoplastic. Hypoplasia of the upper renal pole is often associated with an obstructive megaureter. Vesicoureteral reflux into the lower pyelon is common in hypoplasia of the lower pole. In the presence of vesicoureteral reflux, the associated (lower) pyelon is dilated when the bladder is full or during micturition. In addition, there is a dilated ureter. On the other hand, the pyelon can have a normal width when the bladder is empty. In rare cases, one pole may be cystically altered in pathological duplex kidneys. In this instance, segmental multicystic dysplastic duplex kidney must be differentiated from segmental multicystic nephroma.

Surgery of the Equine Urinary Tract.

Urinary surgery in the horse may be challenging. More straightforward procedures, such as urinary bladder or urachal defects, do not usually require specialized equipment or imaging, although laboratory work is helpful. Congenital or acquired conditions of the ureters or kidneys may necessitate advanced diagnostic work-ups including advanced imaging /or and minimally invasive procedures. Some surgery of the lower urinary tract is done in the sedated, standing adult horse. Surgery involving the kidney typically requires general anesthesia. Laparoscopy and associated tools are frequently used. Although many of the surgical procedures discussed are quite involved, they are becoming more commonplace.

Incomplete Renal Duplex System with Lower Moiety Hydroureteronephrosis Due to Aberrant Blood Vessel.

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.

[Bradis anti-reflux ureterocysto-anastomosis in children with recurrent obstructive megaureter].

INTRODUCTION: Congenital ureterovesical junction (UVJ) obstructions quite rarely serve as an indication for ureteral reimplantation, and recurrent obstructions resulting from surgical treatment are even less frequent. Cases of acquired UVJ obstruction following endoscopy and ureteral reimplantation done for vesicoureteral reflux correction are fairly rare. The lack of known publications analyzing treatment of recurrent obstructive megaureter predetermines topicality of our research. PURPOSE: Evaluate the efficiency and safety of extravesical anti-reflux uretero-cysto-anastomosis performed using Bradis technique for children with recurrent obstructive megaureter (OM). MATERIALS AND METHODS: The work is based on the experience of surgical OM correction in 5 children aged 1 to 4 years, 4 boys and a girl with a recurrent UVJ obstruction following prior ureteral reimplantation for correction of UVJ obstruction (4 children) or vesicoureteral reflux (1 patient). Antenatal UVJ obstruction had been diagnosed in 2 patients. In all cases the examination included urine analysis, its culture study, ultrasonography of the kidneys and urinary tracts, dynamic renal scintigraphy, micturating cystography, and, where indicated, intravenous urography and SCT of the kidneys. All patients underwent Bradis extravesical reimplantation. RESULTS: During postoperative care period, a considerable reduction of the dilatation of renal collecting system was accomplished in all five patients along with the differential renal function preserved on the pre-surgery level and a steady remission of urinary tract infection based on clinical manifestations and laboratory findings. CONCLUSIONS: The simplicity of Bradis extravesical ureteral reimplantation represents its advantage over intravesical methods. Extravesical approach allows easy ureteral length and width correction. Limited experience of Bradis surgery in pediatric cases of recurrent OM requires confirmation with further randomized clinical trials.

Multifaceted Hoxa13 function in urogenital development underlies the Hand-Foot-Genital Syndrome.

Hand-Foot-Genital syndrome is a rare condition caused by mutations in the HOXA13 gene and characterized by limb malformations and urogenital defects. While the role of Hoxa13 in limb development has been extensively studied, its function during the development of the urogenital system remains elusive mostly due to the embryonic lethality of Hoxa13 homozygous mutant mice. Using a conditional inactivation strategy, we show that mouse fetuses lacking Hoxa13 function develop megaureters, hydronephrosis and malformations of the uterus, reminiscent of the defects characterizing patients with Hand-Foot-Genital syndrome. Our analysis reveals that Hoxa13 plays a critical role in Müllerian ducts fusion and in ureter remodeling by regulating the elimination of the caudal common nephric duct, eventually preventing the separation from the nephric duct. Our data also reveal a specific role for Hoxa13 in the urogenital sinus, which is in part mediated by Gata3, as well as Hoxa13 requirement for the proper organization of the ureter. Finally, we provide evidence that Hoxa13 provides positional and temporal cues during the development of the lower urogenital system, a sine qua non condition for the proper function of the urinary system.

Expansion of the renal capsular stroma, ureteric bud branching defects and cryptorchidism in mice with Wilms tumor 1 gene deletion in the stromal compartment of the developing kidney.

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 Foxd1cre 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.

Antireflux endoscopic injection therapy in post-pubertal patients via techniques adopted for the dilated ureteral orifice: a retrospective single-center study.

BACKGROUND: To investigate the efficacy and safety of endoscopic injection therapy for vesicoureteral reflux in post-pubertal patients with dilated ureteral orifice via modified hydrodistension implantation techniques. METHODS: We retrospectively reviewed medical records including operational procedure and clinical course of all consecutive patients over 12 years old with a history of injection therapy. Endoscopic injection of dextranomer/hyaluronic acid copolymer was performed under hydrodistension implantation technique with some modifications in order to inject through dilated ureteral orifice align with the intramural ureter. Technical selections were done according to hydrodistension grade of the ureteral orifice. Voiding cystourethrography was evaluated at 3 months postoperatively. Hydronephrosis was evaluated using ultrasonography preoperatively until 6 months postoperatively. RESULTS: From 2016 to 2019, 12 patients (all female, 16 ureteral units; median age 32 [range 15-61] years) underwent endoscopic injection therapy at one of our institutions. We have identified grade II vesicoureteral reflux in 5 ureters, grade III in 8, and grade IV in 3 ureters. Grade 3 ureteral-orifice dilation were presented in 12 ureters (75%), grade 2 in 3 and grade 1 in 1 ureter in the present cases. Postoperatively, vesicoureteral reflux was diminished to grade 0 in 12 ureteral units (75%), decreased to grade I in 3 (9%), and remained grade III in 1 (6%). Three patients reported dull flank pain for several days postoperatively and there was 1 case of acute pyelonephritis. Temporary hydronephrosis was confirmed in 3 ureteral units (19%) at 1 month postoperatively. Median follow-up duration was 23 (range 13-63) months long. Although, 3 patients were experienced f-UTI 1-2 times, repeated VCUG showed no VUR recurrence. CONCLUSIONS: According to hydrodistension grade of the ureteral orifice, endoscopic injection therapy via modified hydrodistension implantation technique is an effective and safe treatment for vesicoureteral reflux in post-pubertal female patients with dilated ureteral orifice. While ureteral deformities or a history of anti-reflux surgery may increase the risks, these can be managed with appropriate methods that ensure sufficient mound appearance and height.

Relation between Cajal Cell Density and Radiological and Scintigraphic Outcomes in Patients with Ureteropelvic Junction Obstruction.

INTRODUCTION: In this study, we aimed to investigate the correlation between Cajal cell density and preoperative and postoperative radiological and scintigraphic parameters in ureteropelvic junction obstruction (UPJO). METHODS: The study group consisted of 41 renal units (38 consecutive patients; 13 female and 25 male) surgically treated for UPJO. UPJ specimens from patients were immuno-stained with CD117 (c-kit) antibody for interstitial Cajal cells (ICCs). The relation between Cajal cell density and preoperative and postoperative radiological and scintigraphic parameters was evaluated. RESULTS: The mean age of the patients was 8.52 ± 8.86 (0-35) years. The density of Cajal cells was defined in 2 groups for convenient analysis as 0-5 cells (low) in 19 (46.3%) patients and >5 cells (moderate-high) in 22 (53.6%). There was significant difference between the preoperative and postoperative anteroposterior diameters of the related kidneys in both Cajal groups (p = 0.001-low, p = 0.000-moderate-high) independent of Cajal cell density. Regression in hydronephrosis postoperatively was determined in both Cajal groups (77.8%-low, 64.7%-moderate-high); however, there was no difference between them (p = 0.39). Preoperative T1/2 was significantly longer in the low Cajal group (p = 0.02). Postoperative T1/2 decreased in both low (p = 0.000) and moderate-high (p = 0.001) Cajal groups, but no difference was found between them (p = 0.24). There was significant improvement in the kidney differential function after surgery in the low Cajal density group (p = 0.015) while there was no correlation between the scintigraphic success or improvement and Cajal cell density (p = 0.51). DISCUSSION/CONCLUSION: ICC deficiency/density could not be shown as a predictive factor for the determination of success rate of pyeloplasty. Despite the lack of any evidence for the degree of deficiency as an indicator for the severity of obstruction and prediction of surgical success, further studies are needed for confirmation.

MRI of female genital tract congenital anomalies: European Society of Urogenital Radiology (ESUR) guidelines.

OBJECTIVE: To develop imaging guidelines for the MR work-up of female genital tract congenital anomalies (FGTCA). METHODS: These guidelines were prepared based on a questionnaire sent to all members of the European Society of Urogenital Radiology (ESUR) Female Pelvic Imaging Working Group (FPI-WG), critical review of the literature and expert consensus decision. RESULTS: The returned questionnaires from 17 different institutions have shown reasonable homogeneity of practice. Recommendations with focus on patient preparation and MR protocol are proposed, as these are key to optimised examinations. Details on MR sequences and planning of uterus-orientated sequences are provided. CONCLUSIONS: The multiplanar capabilities and soft tissue resolution of MRI provide superb characterisation of the wide spectrum of findings in FGTCA. A standardised imaging protocol and method of reporting ensures that the salient features are recognised, contributing to a correct diagnosis and classification of FGTCA, associated anomalies and complications. These imaging guidelines are based on current practice among expert radiologists in the field and incorporate up to date information regarding MR protocols and essentials of recently published classification systems. KEY POINTS: • MRI allows comprehensive evaluation of female genital tract congenital anomalies, in a single examination. • A dedicated MRI protocol comprises uterus-orientated sequences and vaginal and renal evaluation. • Integration of classification systems and structured reporting helps in successful communication of the imaging findings.

Computed tomographic excretory urography for diagnosing ectopic ureter in a female rhesus macaque (Macaca mulatta).

Ectopic ureter is a congenital abnormality where the ureter terminates at a site other than the urinary bladder. A five-year-old female rhesus monkey presented with a urine odor, a wet perineum, and persistent dribbling of urine. An ultrasound examination revealed a cyst-like structure (1 × 0.75 cm) on the left side of the bladder. Computed tomographic excretory urography (CTEU) imaging revealed a left unilateral extramural ectopic ureter, which was connected to the vagina. The perineum and wet hair were dried and disinfected with 0.4% chlorhexidine for perineal hygiene and skin care. The animal was closely monitored for potential moist dermatitis near the perineum and for urinary tract infection. The complete blood count (CBC) and blood chemistry results showed no signs of inflammation during the observation period. This is the first report of detailed diagnosis of ectopic ureter by ultrasound and CTEU in a female rhesus monkey.

Delayed onset of smooth muscle cell differentiation leads to hydroureter formation in mice with conditional loss of the zinc finger transcription factor gene Gata2 in the ureteric mesenchyme.

The establishment of the peristaltic machinery of the ureter is precisely controlled to cope with the onset of urine production in the fetal kidney. Retinoic acid (RA) has been identified as a signal that maintains the mesenchymal progenitors of the contractile smooth muscle cells (SMCs), while WNTs, SHH, and BMP4 induce their differentiation. How the activity of the underlying signalling pathways is controlled in time, space, and quantity to activate coordinately the SMC programme is poorly understood. Here, we provide evidence that the Zn-finger transcription factor GATA2 is involved in this crosstalk. In mice, Gata2 is expressed in the undifferentiated ureteric mesenchyme under control of RA signalling. Conditional deletion of Gata2 by a Tbx18cre driver results in hydroureter formation at birth, associated with a loss of differentiated SMCs. Analysis at earlier stages and in explant cultures revealed that SMC differentiation is not abrogated but delayed and that dilated ureters can partially regain peristaltic activity when relieved of urine pressure. Molecular analysis identified increased RA signalling as one factor contributing to the delay in SMC differentiation, possibly caused by reduced direct transcriptional activation of Cyp26a1, which encodes an RA-degrading enzyme. Our study identified GATA2 as a feedback inhibitor of RA signalling important for precise onset of ureteric SMC differentiation, and suggests that in a subset of cases of human congenital ureter dilatations, temporary relief of urine pressure may ameliorate the differentiation status of the SMC coat. © 2019 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

Overactivity or blockade of transforming growth factor-ß each generate a specific ureter malformation.

Transforming growth factor-ß (TGFß) has been reported to be dysregulated in malformed ureters. There exists, however, little information on whether altered TGFß levels actually perturb ureter development. We therefore hypothesised that TGFß has functional effects on ureter morphogenesis. Tgfb1, Tgfb2 and Tgfb3 transcripts coding for TGFß ligands, as well as Tgfbr1 and Tgfbr2 coding for TGFß receptors, were detected by quantitative polymerase chain reaction in embryonic mouse ureters collected over a wide range of stages. As assessed by in situ hybridisation and immunohistochemistry, the two receptors were detected in embryonic urothelia. Next, TGFß1 was added to serum-free cultures of embryonic day 15 mouse ureters. These organs contain immature smooth muscle and urothelial layers and their in vivo potential to grow and acquire peristaltic function can be replicated in serum-free organ culture. Such organs therefore constitute a suitable developmental stage with which to define roles of factors that affect ureter growth and functional differentiation. Exogenous TGFß1 inhibited growth of the ureter tube and generated cocoon-like dysmorphogenesis. RNA sequencing suggested that altered levels of transcripts encoding certain fibroblast growth factors (FGFs) followed exposure to TGFß. In serum-free organ culture exogenous FGF10 but not FGF18 abrogated certain dysmorphic effects mediated by exogenous TGFß1. To assess whether an endogenous TGFß axis functions in developing ureters, embryonic day 15 explants were exposed to TGFß receptor chemical blockade; growth of the ureter was enhanced, and aberrant bud-like structures arose from the urothelial tube. The muscle layer was attenuated around these buds, and peristalsis was compromised. To determine whether TGFß effects were limited to one stage, explants of mouse embryonic day 13 ureters, more primitive organs, were exposed to exogenous TGFß1, again generating cocoon-like structures, and to TGFß receptor blockade, again generating ectopic buds. As for the mouse studies, immunostaining of normal embryonic human ureters detected TGFßRI and TGFßRII in urothelia. Collectively, these observations reveal unsuspected regulatory roles for endogenous TGFß in embryonic ureters, fine-tuning morphogenesis and functional differentiation. Our results also support the hypothesis that the TGFß up-regulation reported in ureter malformations impacts on pathobiology. Further experiments are needed to unravel the intracellular signalling mechanisms involved in these dysmorphic responses. © 2019 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of Pathological Society of Great Britain and Ireland.

Comparative outcome analysis of pediatric kidney transplant in posterior urethral valves children with or without pretransplant Mitrofanoff procedure.

BACKGROUND: Urinary diversion using catheterizable channel among posterior urethral valve (PUV) patients may help to alleviate the functional concerns of the bladder on the allograft. Herein, we review our series of PUV patients undergoing renal transplants at a single institution to determine outcomes between those with and without pretransplant Mitrofanoff. METHODS: PUV cases in our transplant database from 2000 to 2017 were identified. Data collected included clinical demographics, presentation of PUV, bladder function, implementation of dialysis and donor type. Post-transplant variables included graft function, and bladder function including incontinence and UTI development. Comparison between with vs without pretransplant Mitrofanoff patient groups was performed using Fisher's exact and t test analysis. RESULTS: Overall, 22 cases were analyzed. The majority (77%) had a prenatal diagnosis of PUV, and 23% had a symptomatic presentation. Pretransplant Mitrofanoffs were performed in 40.9% of cases. The overall age at transplant was 10 ± 5 years. Five percent experienced post-transplant incontinence and 23% UTIs. No difference between groups was noted in variables including age at transplant, the timing of PUV diagnosis. Mitrofanoff group patients had more symptomatic febrile UTIs than those without Mitrofanoff (67% vs 15%; P = .03); however, there was no difference in post-transplant UTI development. Furthermore, the Mitrofanoff group seems to have later onset of dialysis or need for transplant (age at the start of dialysis 36 ± 56 vs 156 ± 25 months, P < .001). CONCLUSION: Our study suggests that patients with early PUV diagnoses who develop symptomatic UTI may benefit from early creation of a Mitrofanoff cathetherizable channel, which is associated with delayed ESRD progression and need for dialysis.

Prenatal imaging features and postnatal outcomes of isolated fetal duplex renal collecting system: A systematic review and meta-analysis.

OBJECTIVES: To perform a systematic review of studies reporting the outcome of fetuses with a prenatal diagnosis of isolated duplex collecting system (DCS). METHODS: Inclusion criteria were studies reporting the outcome of fetuses with a prenatal diagnosis of isolated DCS, defined as DCS not associated with other major structural anomalies at the time of diagnosis. The outcomes observed were: imaging features of DCS on prenatal ultrasound, associated anomalies detected exclusively at prenatal follow-up ultrasound and at birth, abnormal karyotype, symptoms at birth (including vesicoureteral reflux [VUR] and urinary tract infections [UTI]), need for and type of surgical approach, complications after surgery, and accuracy of prenatal ultrasound in correctly identifying this anomaly. RESULTS: Eleven studies (284 fetuses with a prenatal diagnosis of DCS) were included. On ultrasound, DCS was associated with ureterocele in 70.7% and with megaureter in 36.6% of cases. Worsening of pelvic/ureteric dilatation was reported to occur in 41.3% of fetuses. At birth, 4.3% of fetuses affected by DCS showed associated renal anomalies. After birth, VUR and UTI presented in 51.3% and 21.7% of children respectively, while 33.6% required surgery. Prenatal diagnosis of DCS was confirmed in 90.9% of included cases. CONCLUSION: DCS diagnosed prenatally is associated with a generally good outcome. Prenatal ultrasound has a good diagnostic accuracy, while detailed postnatal assessment is required in order to identify associated renal anomalies.

Kidney triplication with ectopic ureterocele: a case report.

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.

The left ureterocele and stone of calyceal diverticulum in the patient with bilateral incomplete duplex kidneys managed by flexible ureteroscopy: a case report and literature review.

BACKGROUND: Duplex kidneys are one of the most common renal congenital abnormalities, mostly asymptomatic and of no clinical significance. There are little reports about the left ureterocele and stone of calyceal diverticulum in patients with bilateral incomplete duplex kidneys managed by flexible ureteroscopy. CASE PRESENTATION: A 69-year-old Chinese woman was presented with left waist pain for 1 month. A preoperative computed tomography (CT) scan and intravenous pyelogram revealed the left ureterocele which located in the left ureterovesical junction, and stone of calyceal diverticulum which located in the upper kidney of left incomplete duplex kidneys. The ureterocele was confirmed in view of ureteroscopy and the holmium laser was used for the resection of ureterocele. It took us a lot of efforts to find out the stone because of diverticular neck stenosis. Fortunately, when diverticular neck stenosis was incised internally by holmium laser, the stone was discovered clearly and removed using the holmium laser and nitinol stone basket through flexible ureteroscopy. A double-J ureteral stent was inserted and remained in place for 1 month. The symptom disappeared postoperatively and no complications were developed during the placement of the stent. There were no stone residents observed on CT scan before removing the ureteral stent 1 month later. CONCLUSIONS: Flexible ureteroscopy with holmium laser is feasible to manage the ureterocele and calyceal diverticulum stones in patients with bilateral incomplete duplex kidneys in one operation.

Sprouty1 Controls Genitourinary Development via its N-Terminal Tyrosine.

BACKGROUND: Studies in mice suggest that perturbations of the GDNF-Ret signaling pathway are a major genetic cause of congenital anomalies of the kidney and urinary tract (CAKUT). Mutations in Sprouty1, an intracellular Ret inhibitor, results in supernumerary kidneys, megaureters, and hydronephrosis in mice. But the underlying molecular mechanisms involved and which structural domains are essential for Sprouty1 function are a matter of controversy, partly because studies have so far relied on ectopic overexpression of the gene in cell lines. A conserved N-terminal tyrosine has been frequently, but not always, identified as critical for the function of Sprouty1 in vitro. METHODS: We generated Sprouty1 knockin mice bearing a tyrosine-to-alanine substitution in position 53, corresponding to the conserved N-terminal tyrosine of Sprouty1. We characterized the development of the genitourinary systems in these mice via different methods, including the use of reporter mice expressing EGFP from the Ret locus, and whole-mount cytokeratin staining. RESULTS: Mice lacking this tyrosine grow ectopic ureteric buds that will ultimately form supernumerary kidneys, a phenotype indistinguishable to that of Sprouty1 knockout mice. Sprouty1 knockin mice also present megaureters and vesicoureteral reflux, caused by failure of ureters to separate from Wolffian ducts and migrate to their definitive position. CONCLUSIONS: Tyrosine 53 is absolutely necessary for Sprouty1 function during genitourinary development in mice.