A variant of Zinner syndrome with ectopic ureteral insertion into the seminal vesicle.

Zinner syndrome comprises a triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ejaculatory duct obstruction, which can be accompanied by additional abnormalities of the genitourinary tract in some cases. Patients may be asymptomatic or present with urinary, reproductive, and/or local pain symptoms. Diagnosis is most commonly achieved via MRI. Here, we present the case of an 18-year-old male previously diagnosed with unilateral renal agenesis, who presented with testicular and penile pain, along with urinary urgency and frequency. MRI of the abdomen and pelvis revealed all three components of Zinner syndrome as well as an ectopic ureter emptying into the seminal vesicle. Our case adds to the existing limited literature on this rare syndrome and broadens the understanding of how this syndrome can present both clinically and radiologically.

A Single-System Ectopic Ureter in a Child: A Challenge for Early Diagnosis.

An ectopic ureter is an uncommon anomaly, usually associated with a duplicated urinary system. Up to 20% of ectopic ureters occur in a single system. In females, only 25% of ectopic ureters insert into the vagina and usually cause urinary incontinence, which can be confused with vaginal discharge. The diagnostic investigation includes urinary tract ultrasound, DMSA, and urethrocystography, which evaluate renal morphology and function, determining factors for surgical treatment decision that aims to preserve renal function, prevent the recurrence of infections, and reestablish urinary continence. The rarity of this anomaly and the delay in recognizing symptoms are factors related to late diagnosis.

Persistent urinary incontinence in female Golden Retrievers following laser ablation of intramural ectopic ureters may be associated with the presence of historical urinary tract infection.

OBJECTIVE: To identify predictive factors for postoperative continence in female Golden Retrievers following cystoscopic-guided laser ablation of intramural ectopic ureters (CLA-EU). ANIMALS: 41 client-owned female entire Golden Retrievers with uni- or bilateral intramural ectopic ureter(s) were retrospectively enrolled. METHODS: Patients were diagnosed with ectopic ureters with a combination of ultrasonography and cystoscopy. CLA-EU was performed for all dogs so that each ureteral opening was considered to be in an appropriate position by a single operator. All dogs had short-term follow-up 4 weeks and long-term follow up > 10 weeks after the procedure via telephone, which included urinary continence scoring. Clinical factors and ultrasonographic and cystoscopic findings from initial presentation were evaluated to identify predictive factors for postoperative continence. RESULTS: Short-term urinary continence was achieved in 46.3% of dogs with no additional medical therapies. Presence of historical urinary tract infections prior to CLA-EU (OR, 0.130; 95% CI, 0.020 to 0.621; P = .018) was negatively correlated and ureteral dilatation (OR, 34.260; 95% CI, 1.813 to 2,143; P = .043) was positively correlated with likelihood of urinary continence. Long-term urinary continence was achieved in 63.4% of dogs, and presence of historical urinary tract infections was negatively prognostic (OR, 0.173; 95% CI, 0.023 to 0.856; P = .048). CLINICAL RELEVANCE: Female Golden Retrievers undergoing CLA-EU have similar outcomes to those reported for other mixed-breed cohorts with > 30% of dogs failing to regain urinary continence. Historical urinary tract infections were significantly associated with both short- and long-term urinary continence in our population.

Personalized Surgical Management Offers Full Restitution and Unimpaired Quality of Life to Patients with Duplex Kidneys and Associated Pathologies: 30-year Follow-up at a Tertiary Referral Center.

Background: Duplex kidneys may be associated with additional pathologies with an indication for surgery. Various surgical approaches have been described. However, little is known about long-term outcomes and quality of life (QoL) for these patients. Objective: To present long-term outcomes and QoL data up to 30 yr after surgical treatment of duplex kidneys and associated pathologies. Design setting and participants: We collected clinical and operative data for all patients who underwent surgery for complicated duplex kidney at our institution from 1990 to 2018. All patients were invited for a follow-up examination or telephone interview. Outcome measurements and statistical analysis: We evaluated renal function, clinical outcomes, residual dilation of the upper urinary tract, and health-related QoL. Results and limitations: Of the 176 patients included, 173 were available for follow-up (mean 140.5 mo). Surgical treatment involved an upper-tract, lower-tract, or combined approach in 11%, 56%, and 33% of cases, respectively. Rates of perioperative complications (8%) and secondary surgery (10%) were low. Overall, 95% of our patients achieved full restitution. Renal function was preserved in all cases, with recurrent urinary tract infections reported by just 2% and urinary incontinence by 1%. Good health-related QoL was reported by 98% of patients. Those without full restitution included six patients who underwent total nephrectomy and two boys who underwent multiple surgeries and urinary diversion. Our results are limited by their retrospective nature, including partly incomplete data sets. Conclusions: Management of duplex kidneys and associated pathologies is complex and highly individual. By planning a personal approach for each patient it is possible to achieve full bodily integrity and good QoL for most of these patients. Patient summary: Almost all patients undergoing surgery for duplex kidneys and associated pathologies will lead a life without body impairment and good quality of life.This trial is registered in the German Clinical Trials Register as DRKS00022542.

Urinary Incontinence Due to Complete Ureteral Duplication With an Ectopic Vulvar Ureteral Orifice: A Case Report.

Ureteral duplication is one of the most common congenital malformations of the urinary tract and may be complete or incomplete. One of the complications of complete ureteral duplication is an ectopic ureter orifice, which, depending on the opening site, may cause urinary incontinence in females, a condition with potentially serious repercussions on the woman's quality of life. Thus, the present study aims to report the case of a 24-year-old female patient with a complaint of urinary incontinence since childhood. After a physical examination and imaging tests, she was diagnosed with complete ureteral duplication on the left side, associated with sequelar parenchymal atrophy of the upper pole of the left kidney, and ectopic vulvar ureter. The patient underwent a videolaparoscopic left upper polar nephrectomy, and her symptoms improved after surgery. This report intends to add to already available data in the literature, highlighting the relevance of anamnesis and physical examination in reaching a diagnosis and implementing appropriate treatment, thus improving the quality of life of individuals with this condition. In addition, these data should be useful both for the medical community and for future studies on this malformation.

Obstructed hemivagina and ipsilateral renal agenesis: magnetic resonance imaging findings in young Nepali females - a report of four cases.

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome also known as the Herlyn-Werner-Wunderlich Syndrome is a rare embryological disorder associated with Mullerian and mesonephric duct abnormality. Case presentation: The cases presented describe the imaging (ultrasound and MRI) findings of four young females who presented with dysmenorrhea and urinary complaints. All of them had solitary kidneys with a didelphic uterus and unilateral hematometrocolpos. A proximally blind-ending ureter with distal ectopic insertion, transverse vaginal septum, and left-sided endometrioma was seen. Clinical discussion: OHVIRA syndrome is associated with duplicated uterovaginal structure with OHVIRA. Ultrasound is the first line of investigation; however, MRI better delineates the anatomy and assists in preoperative planning. Conclusion: This report highlights that earlier clinical suspicion and imaging diagnosis of OHVIRA is crucial to prevent adverse outcomes and treating complications.

Bilateral single system ectopic ureters with bilateral urethral insertion and horseshoes kidneys in a female child.

A 14-months old female child was diagnosed with bilateral single system ectopic ureters opening into the urethra, with small bladder capacity, horseshoes kidneys, and bilateral hydronephrosis, presenting recurrent febrile UTI accompanied by continuous incontinence and elevated renal function. Early bilateral re-implantation of the ureters (modified Lich-Gregoir) was done in one setting, resulting jn no recurring febrile UTIs and continuous wetting, improving renal function parameter, competent bladder neck, and 10 folds increased in bladder capacity after 1-year follow up. We showed that earlier treatment enables patient to preserve both renal and bladder function without involving complex reconstructive surgery.

Challenges in diagnosing vaginal mass in a pregnant woman with an underlying duplex collecting system disease: a case report.

Congenital renal anomalies are a sequence of defective renal parenchymal or collecting system development and migration that may be discovered during the prenatal period or incidentally among adults. Duplex collecting system diagnosing in adults represents a challenge to physicians. Long-term history of urinary tract infections besides vaginal mass in pregnant women should raise the suspicion of underlying urinary tract malformation. Case presentation: In this case, a 23-year-old pregnant woman at 32 weeks came to the clinic for a routine check-up. A vaginal mass had been noticed during the examination and was punctured, revealing unknown fluid. Further investigations revealed left duplex collecting system consisted of an upper moiety opening with a ureterocele in the anterior wall of the vagina and a lower moiety ending with an ectopic orifice near the right ureter orifice. Therefore, the modified Lich-Gregoir procedure was done to reimplant the ureter of the upper renal moiety. Postoperative following-up investigations affirmed improvement without complications. Clinical discussion: The duplex collecting system disease may remain asymptomatic until adulthood or present with unexpected symptoms. The subsequent workup in the duplex kidney disease depends on the moieties' function and the ureter orifice opening site. Although the Weigert-Meyer rule is usually used to describe the typical pattern of duplex collecting system ureters opening sites, it has many expectations in the literature. Conclusion: This case shows how some common symptoms may lead to finding an unexpected urinary tract abnormality.

Detection and management of a case of single system ectopic ureteral insertion to vagina with atrophic ectopic kidney.

INTRODUCTION AND IMPORTANCE: Ectopic ureter is defined as any ureter, single or duplex, that opens in a location other than trigone of bladder. Continuous urine leakage and regular intentional voiding must point to the diagnosis of an ectopic ureter, particularly in females (Singh et al., 2022). Following successful repair of ectopic ureter, the overall long-term continence rate is satisfactory. CASE PRESENTATION: This case is reported to discuss a case of 24 yrs. old woman presenting with a complaint of insensible continuous urinary leak with normal intentional voiding since childhood. Ultrasound and CTU showed left solitary kidney with normal insertion of its ureter; but failed to demonstrate right system. MRI showed Right EU with ectopic dysplastic right kidney. Renal scintigraphy was unavailable at the time of evaluation and IVP was suggestive of NEK. Nephroureterectomy done. Her subsequent follow up was satisfactory. CLINICAL DISCUSSION: Because many people with EU are asymptomatic and the diagnosis is frequently missed, the prevalence of EU is uncertain. Preferred mode of diagnosis is pelvic MRI. Ureteral duplication accounts for 80 % of ectopic ureter occurrences in women (Demir et al., 2015). Ectopic ureters draining a single-system ectopic ureter with dysplastic kidneys, on the other hand, are uncommon, particularly in females (Amenu et al., 2021) Despite this rarity, we have found single system with atrophic kidney. CONCLUSION: This instance suggests to us that in cases of urinary incontinence especially in women, congenital anomalies of the genitourinary tract should be taken into consideration. Surgical management depends on the degree of renal function and location of EU. Either nephroureterectomy or ureteric reimplantation are curative for incontinence.

Surgical management of complicated duplex kidney: A tertiary referral centre 10-year experience.

Aim: The management of a complicated duplex kidney remains a challenge for paediatric urologists. The aim of this study is to report a 10-year experience of the surgical management of complicated duplex kidney in a single tertiary care paediatric referring hospital. Materials and Methods: Clinical records of all children who undergone a surgical procedure for complicated duplex systems between January 2009 and March 2019 at our institution were retrospectively reviewed. Clinical manifestations, surgical procedures, complications and follow-up were collected and analysed. Logistic regression was performed to explore if any patient's characteristic or underlying associated comorbidity (ureterocoele, ectopic ureter, obstruction, etc.,) could be positively linked to the chance to develop recurrent urinary tract infections (UTIs). Results: We have identified 95 children who received a surgical treatment for 102 complicated duplex kidneys. The presence of an ureterocoele was recorded in 41 (43.2%) patients, an ectopic ureter in 25 (26.3%), a vesicoureteral reflux (VUR) in 40 (42.1%), a vesicoureteric junction obstruction in 24 (25.3%) and an ureteropelvic junction obstruction in 3 (3.2%). An invasive approach such as an heminephrectomy (71.6%) was required in the majority of cases. Higher risk of developing a UTI has been demonstrated in children diagnosed postnatally (P < 0.001) and in those with an associated obstruction (P < 0.05). Conclusions: No standardised management could be recommended for the surgical treatment of complicated duplex kidney. Children without antenatal diagnosis and with either an upper tract obstruction or VUR are at greater risk to develop UTI and need to be looked after more closely.

A case of right hypodysplastic kidney and ectopic ureter associated with bicornuate uterus in a prepubertal girl.

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.

Bilateral Single-System Ectopic Ureter: When Can We Avoid Bladder Neck Reconstruction?

Bilateral single-system ectopic ureter (BSSEU) is often associated with underdeveloped incompetent bladder neck; hence, to achieve continence, bladder neck reconstruction (BNR) is usually advocated with ureteric reimplantation. Presented here is a 14-year-old girl with BSSEU who achieved continence without BNR. An attempt is made to look at factors that could identify patients in whom BNR could be avoided.

Misplaced Urinary Catheter in the Ectopic Ureter in a Female With Previously Undiagnosed Duplex Ureter: A Rare Case.

Unintentional Foley catheter placement into a ureter is an extremely rare and life-threatening complication that can be easily prevented by early diagnosis. A misplaced Foley catheter should be suspected in the presence of abdominal pain and oliguria after bladder catheterization, and congenital variation of the urinary tract can complicate this procedure. We reported the third case of misplaced insertion of a Foley catheter into the upper moiety ureter of a duplex kidney in a 19-year-old post-partum woman. The patient complained of the right flank and pelvic pain and oliguria one day after urinary catheter insertion. Duplicated right kidney, upper pole moiety mild hydronephrosis and malpositioned Foley catheter balloon in the right ectopic ureter were reported on ultrasonography which was confirmed by a CT scan. The catheter was removed easily without any complications.

Gynecological Diagnosis and Treatment of Ectopic Ureter Insertion into Vagina: Analysis of Five Cases and a Literature Review.

An ectopic ureter is a ureter that does not correctly connect to the trigone of the bladder and drains outside of the bladder. Here, we presented five cases of ectopic ureter opening into the vagina, whose clinical symptoms and malformations were rarely described in previous case reports. All five patients were hospitalized with complaints of gynecologic disease. Three of the five cases did not present the typical symptoms of urinary incontinence. Three of these cases showed congenital malformations of the female genital tract. Four cases were diagnosed in adulthood. All patients were analyzed using various imaging examinations. This study suggests that the ectopic ureter should be considered in the differential diagnosis of a pelvic mass in a patient with urinary and reproductive system abnormalities. It is essential to comprehensively evaluate complex malformations of the genitourinary system with multiple imaging tests.

Preoperative MRI presentations of Herlyn-Werner-Wunderlich syndrome.

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare complex female urogenital anomaly, with diverse anatomical presentations. Due to obstruction, most patients with HWW syndrome need to be addressed surgically. The treatment strategy should be tailored to the different anatomical variants of each patient. Therefore, a detailed and comprehensive preoperative evaluation is needed. In this review, we describe the embryology and clinical manifestations of HWW syndrome and discuss and illustrate its diverse preoperative magnetic resonance imaging presentations to guide clinical treatment.

Left ectopic ureteral insertion into seminal vesicle detected after robotic assisted laparoscopic radical prostatectomy.

Ectopic ureters are rare congenital malformations of the urinary tract, more frequent in females and most commonly associated with single collecting system in males. We report a case of a prostate cancer patient undergoing robotically assisted laparoscopic radical prostatectomy. Duplication of vas deferens was thought to be found during surgery. Postoperatively, patient developed fevers. CT showed incidental finding of duplex collecting system on the left with dilatation of the upper moiety. Percutaneous nephrostomy was placed but an attempt at antegrade insertion of ureteric stent was unsuccessful. Robotic reimplantation of the ectopic ureter was successfully performed on day six post prostatectomy.

[Outcome of selective mating in the Entlebucher Mountain Dog for reduction of ureteral ectopia]. / Ergebnis der selektiven Verpaarung beim Entlebucher Sennenhund zur Reduktion der ureteralen Ektopie.

INTRODUCTION: The Entlebucher Mountain Dog is predisposed to ureteral ectopia and associated diseases of the urinary tract as well as the kidneys, which can have severe to lethal consequences. Due to the clustered occurrence of clinical signs in 11 % of Entlebucher Mountain dogs in the absence of a genetic test for ureteral ectopia, screening was introduced in 2008 to allow phenotype-based breeding selection. The ureteral orifices of the dogs are visualized by ultrasound and existing urinary retention or urinary incontinence is documented. The diagnostic findings were evaluated centrally with assignment to one of five phenotypes depending on the localization of the ureteral orifices and the renal and ureteral shape. Breeding approval and mating restrictions are the responsibility of the respective breeding associations and predominantly Entlebucher Mountain Dogs with extravesical ectopic ureters and/or clinical signs were excluded from breeding. The effect of phenotype-based selective mating on the incidence of ureteral ectopia and its clinical signs, as well as possible factors influencing the expression of the phenotype, were determined in the birth cohorts after the introduction of screening. Analysis of the data set of 1456 phenotyped Entlebucher Mountain Dogs showed, that at 11 % versus 5 %, males were more frequently assigned to the extravesical phenotype than females. The effect of phenotype-based breeding selection was examined in a subpopulation consisting of phenotyped parents and their offspring (n = 876). The prevalence of the extravesical phenotype decreased from 24 % in the 2005 to 2007 birth cohorts to 1,4 % in the 2015 to 2017 birth cohorts. Since 2015 almost no Entlebucher Mountain Dogs with incontinence, hydroureter or hydronephrosis have been recorded. It was feared that the additional selection measures to control ureteral ectopia in the small Entlebucher Mountain Dog population would intensify the inbreeding increase. However, this has so far remained absent. Therefore, as long as no genetic test is available, it is recommended to continue phenotype-based breeding selection with exclusion of dogs with extravesical ureteral ectopia and/or hydroureter/hydronephrosis/urinary incontinence, while keeping an eye on the development of the inbreeding coefficient.

INTRODUCTION: Le Bouvier de l'Entlebuch est prédisposé à l'ectopie urétérale et aux maladies associées des voies urinaires ainsi que des reins, ce qui peut entraîner des conséquences fatales. En raison de l'apparition de signes cliniques chez 11 % des chiens et en l'absence d'un test génétique pour l'ectopie urétérale, un dépistage a été introduit en 2008 pour permettre une sélection d'élevage basée sur le phénotype. Les orifices urétraux des chiens ont été visualisés par échographie et la rétention ou l'incontinence urinaire existante documentée. Les résultats du diagnostic ont été évalués de manière centralisée avec attribution à l'un des cinq phénotypes en fonction de la localisation des orifices urétéraux ainsi que de la forme des reins et des uretères. L'approbation pour la reproduction et les restrictions d'accouplement relèvent de la responsabilité des associations d'élevage respectives et les bouviers de l'Entlebuch présentant des uretères ectopiques extravésicaux et/ou des signes cliniques ont majoritairement été exclus de la reproduction. L'effet de cet accouplement sélectif basé sur le phénotype sur l'incidence de l'ectopie urétérale et de ses signes cliniques ainsi que les facteurs possibles influençant l'expression du phénotype ont été déterminés dans les cohortes de naissance après l'introduction du dépistage. L'analyse de l'ensemble des données de 1456 Bouviers de l'Entlebuch phénotypés a montré que, à 11 % contre 5 %, les mâles étaient plus fréquemment affectés au phénotype extravésical que les femelles. L'effet de la sélection d'élevage basée sur le phénotype a été examiné dans une sous-population composée de parents phénotypés et de leur progéniture (n = 876). La prévalence du phénotype extravésical est passée de 24 % dans les cohortes de naissance de 2005 à 2007 à 1,4 % dans les cohortes de naissance de 2015 à 2017. Depuis 2015, presque aucun bouvier d'Entlebuch présentant une incontinence, un hydrouretère ou une hydronéphrose n'a été enregistré. Une possible augmentation de la consanguinité due aux mesures de sélection supplémentaires visant à contrôler l'ectopie urétérale ne s'est pas produite. Par conséquent, tant qu'aucun test génétique n'est disponible, il est recommandé de poursuivre la sélection d'élevage basée sur le phénotype avec exclusion des chiens présentant une ectopie urétérale extravésicale et/ou une hydrouretère/hydronéphrose/incontinence urinaire, tout en surveillant l'évolution du coefficient de consanguinité.

Upper pole pathologies in duplex kidneys: an analysis of predictive factors for surgery and urinary tract infections from the Mid-Atlantic Pediatric Academic Consortium.

BACKGROUND/OBJECTIVE: While there is significant data on the natural history and outcomes for prenatal hydronephrosis in simplex kidneys, duplex kidneys tend to be less studied. Management can be quite variable based on provider preference. We aimed to describe practice patterns from several tertiary academic institutions, identify clinical predictors for surgical intervention and urinary tract infection (UTI) for upper pole pathology, and demonstrate the natural history of lower pole vesicoureteral reflux (VUR). METHODS: We conducted a retrospective review of patients from 4 Mid-Atlantic institutions between 2015 and 2020. Inclusion criteria included patients with a duplex kidney with upper pole pathology and/or lower pole VUR. The primary outcome was predictive factors for surgical intervention and UTI. The secondary outcome was to assess the natural history of lower pole VUR including resolution rates by grade. Linear regression identified clinical predictors for UTI events. Multivariate logistic regression identified predictors of surgical intervention, UTI, and lower pole VUR resolution. Descriptive statistics and regression modeling analyses were performed using SAS. RESULTS: Two hundred forty-two patients were included with a total of 271 duplex renal units. Hydronephrosis grade (both SFU and UTD grading) and number of prior UTI events were statistically significant predictors for surgical intervention (p = 0.03/0.001 and p = 0.002 respectively). Ectopic ureter (p = 0.004), ureterocele (p = 0.02), and obstruction (p = 0.04) were the only pathologies predictive for surgery. Male gender and circumcision were significantly associated with decreased UTI risk (p = 0.03 and p = 0.01). On linear regression modeling, antibiotic prophylaxis after the first year of life was associated with decreased risk of further UTI events (p = 0.03); however, antibiotic prophylaxis within the first year of life did not decrease UTI risk (p = 0.14). For VUR outcomes, 65.0% of grades 1-3 VUR and 52.2% of grades 4-5 had resolution/improvement at mean time of 2.1 years. There were no predictive factors for resolution/improvement of VUR. CONCLUSIONS: Hydronephrosis grade and UTI events were significant predictors for surgical intervention for upper pole pathology. Pathologies that were predictive for surgery included ectopic ureter, ureterocele and obstruction. Male gender, circumcision and antibiotic prophylaxis after the first year of life were associated with a decreased UTI risk. Roughly 58% of lower pole VUR spontaneously improved/resolved. Identification of these risk factors aids in standardization of care practices to reduce long-term UTI risk and inform counseling with families about possible need for surgical intervention and expectations for long term outcomes.