Surgical Management of Bilateral Single-System Ectopic Ureters (BSSEUs) in a Pediatric Patient: A Report of a Rare Case and Literature Review.

Bilateral single-system ectopic ureters (BSSEUs) are among the rarest entities encountered in pediatric urology. A BSSEU occurs when the ureteric buds originate cranially from the mesonephric ducts, causing a delay in their integration into the urogenital sinus. It presents as continuous incontinence in females, whereas symptoms like infection and discomfort are present in males. We describe a case involving a BSSEU opening into the vagina and urethra, with the patient experiencing continuous urinary incontinence, and its diagnosis and management. Here, We discuss a rare case of a four-year-old girl exhibiting continuous urinary incontinence or dribbling associated with recurrent urinary tract infections (UTIs) attributed to bilateral ectopic ureters. Imaging modalities, including contrast-enhanced computed tomography(CECT) and MRI, revealed the presence of BSSEUs accompanied by hydroureteronephrosis. The condition was managed with prompt surgical intervention involving bilateral ureteric reimplantation. Subsequent to the procedure, the patient experienced a significant improvement in continence mechanism and bladder capacity, obviating the requirement of urinary diversion procedure appendicovesicostomy or bladder neck reconstruction. Notably, while BSSEUs are an uncommon presentation, their timely and appropriate management is paramount in preventing potential renal damage. This case underscores the significance of vigilant monitoring and proactive intervention in addressing such complex urological anomalies in pediatric patients.

Ectopic Kidney with an Ectopic Ureter Masquerading Adnexal Mass: A Case Report and Literature Review.

An ectopic kidney is a rare congenital defect that is often asymptomatic, but can be incidentally discovered during imaging examinations. Moreover, the morphological characteristics and laboratory features of ectopic kidneys are nonspecific, which may lead to misleading diagnostic approaches, particularly when there are additional factors, such as infection, obstruction, or other anomalies. A 43-year-old female presented with a mass in the left adnexal area. She had septate uterus and a history of congenital urinary incontinence. Ultrasound and MRI findings indicated that the mass was a cyst originating from the ovary. However, it is possible that the lump was derived from the urinary system. To confirm the diagnosis, laparoscopy was performed, followed by pathological examination, which confirmed the presence of an ectopic kidney with a single-system ectopic ureter. The patient underwent nephroureterectomy, and her symptoms successfully resolved, leading to a favorable prognosis. This case report highlights a rare case involving an ectopic kidney with a vaginal ectopic ureter that initially presented as an adnexal cyst and caused urinary dribbling. This case emphasizes the importance of early recognition and accurate diagnosis in women with similar symptoms.

Ectopic Ureter Opening in Vagina: A Rare Cause of Nonfunctional Kidney and Urinary Incontinence in a Pediatric Patient.

An ectopic ureter is a condition characterized by a ureter, whether single or duplex, that fails to open in the trigone area of the urinary bladder but instead drains outside of it. This anomaly arises congenitally due to abnormal migration of the ureteric bud during its insertion into the urinary bladder. Here, we present a case involving an ectopic ureter draining into the vagina, with continuous urinary incontinence. We discuss the diagnosis, evaluation, and management of urinary incontinence in a female because of an ectopic ureter. A 9-year-old girl child presented with a continuous urinary leak or incontinence requiring the use of one to two pads per day that progressively became wetter throughout the day. Physical examination revealed a normal urethral meatus and vagina without obvious visible dribbling of urine at the introitus. CT urography showed significant dilation of the right ureter, causing hydroureter and ectopic insertion of the tortuous right ureter near the external urethral orifice at the vaginal vestibule, along with an atrophic right kidney. A DTPA (diethylenetriamine pentaacetate) scan indicated the nonfunctional status of the right kidney. The patient underwent a right nephroureterectomy, leading to a complete resolution of urinary incontinence. Ectopic ureter causing nonfunctional kidney and urinary leak or incontinence is rare. This case emphasizes the importance of a comprehensive diagnostic workup for achieving a better prognosis and initiating early treatment of ectopic ureter.

Ectopic Ureter: Spectrum of Magnetic Resonance Imaging Findings.

OBJECTIVE: This study aims to describe the MRI findings of six patients with ectopic ureters in a tertiary care institute. METHODS: A retrospective analysis was conducted on six patients presenting to the Department of Radiodiagnosis at Sawai Man Singh (SMS) Hospital, Jaipur, India, with ectopic ureters. Data were collected from the 3 Tesla (3T) Philips MRI scanner (Koninklijke Philips N.V., Amsterdam, Netherlands) from 2021 to 2023. RESULTS: The mean age was 21.6 years, with an equal male-to-female ratio (1:1). Most patients presented with urinary incontinence (50%, n = 3), followed by abdominal pain. Only 16.6% (n = 1) were associated with a duplex collecting system. In males, the most common site was the seminal vesicle, observed in 66.6% (n = 2), followed by the urethra in 33.4% (n = 1) of cases. One patient with an ectopic opening into the seminal vesicle had Zinner syndrome. In females, the ectopic site was found to be the vagina in all three patients. CONCLUSION: Ectopic ureter is a rare anomaly of the urinary system, often associated with other urinary system anomalies and a few syndromes. Clinical presentations range from the patient being asymptomatic to renal failure; therefore, a high index of suspicion and appropriate imaging are necessary for early diagnosis and timely treatment.

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.

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.

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.

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.

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.

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.

Ectopic Ureter—A Retrospective Analysis, Symptom and Treatment

Background: The objective of our study was to share the experience of the ectopic ureter and to characterize associated clinical features, medical images, and management. Methods: A retrospective analysis was performed on 13 cases of ectopic ureter confirmed by surgery. The gender, age, abnormal urinary leakage, the site of the ectopic ureter, renal dysplasia, and surgical methods of the patients were compared and analyzed. Results: Eleven females and 2 males with ectopic ureters were analyzed. Eight cases had the clinical symptom of persistent or intermittent urine leakage since birth. One case was with complete persistent urine leakage, and 4 cases with no urine leakage.The site of the ectopic ureters was mostly unilateral. Ectopic ureters were located in the vagina in 6 cases, urethra in 4 cases,prostate in 1, and uncertain in 2 cases. There were 3 cases with ectopic kidney and renal atrophy, 3 cases had “Y” ureter, and 8 cases had repeated ureters with duplex kidneys. Twelve cases were treated with surgery, and 1 case underwent bilateral ureteral stent implantation. Three cases were complicated with ureteral stump syndrome after surgery. Conclusions: The classic symptom of the ectopic ureter is continuous wetting with intermittent normal micturition in most young girl patients. Ectopic ureter is often associated with duplex kidneys, ectopic kidneys and atrophic kidneys. The surgical approach should be personalized. The aim of the surgery is to make the patient without urine leakage and preserve kidney function. (AU)