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.

Spontaneous resolution and the role of endoscopic surgery in the treatment of primary obstructive megaureter: a review of the literature.

The megaureter accounts for almost a quarter of all urinary tract dilations diagnosed in utero and is the second leading cause of hydronephrosis in newborns, following pyeloureteral junction obstruction. The current standard treatment for progressive or persistent, symptomatic primary obstructive megaureter is ureteral anti-reflux reimplantation, which can be associated with ureteral remodeling or plication. Due to the associated morbidity, postoperative recovery challenges, and the complications that may arise from the open surgical approach, there has been a natural inclination towards validating new minimally invasive techniques. This study reviews the literature, extracting data from three major international databases, from 1998 to 2022. Out of 1172 initially identified articles, only 52 were deemed eligible, analyzing 1764 patients and 1981 renal units. Results show that 65% of cases required surgical intervention, with minimally invasive techniques constituting 56% of these procedures. High-pressure endoscopic balloon dilation was the preferred endourologic technique. The degree of ureterohydronephrosis is considered one of the factors indicating the need for surgery. There is an inverse relationship between the diameter of the ureter and the likelihood of spontaneous resolution. Conditions such as renal hypoplasia, renal dysplasia, or ectopic ureteral insertion strongly indicate a poor prognosis. Endoscopic surgical techniques for treating primary obstructive megaureter can be definitive, firstline treatment options. In selected cases, they might be at least as effective and safe as the open approach, but with advantages like quicker recovery, fewer complications, shorter hospital stays, and reduced costs.

A rare case of right renal ectopia with ureteral triplication in a 37-year-old male: a case report.

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.

A Case of Solitary Kidney and Single-system Ectopic Ureter Draining Into the Vestibule in an Infant With Müllerian Duct Defect: Was the Bladder Missing?

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.

A Single-Center Study of the Efficacy of Transvesicoscopic Ureterovesical Reimplantation: With or Without Ureteral Tailoring in Children with Congenital Megaureter.

Objective: To investigate the efficacy of ureteral tailoring with or without ureteral bladder reimplantation in the treatment of primary megaureter (MGU) in children by transvesicoscopic ureteral reimplantation. Methods: Clinical data from 21 children with primary MGU who were treated with pneumo-bladder ureterovesical reimplantation in Anhui Children's Hospital from January 2019 to August 2022 were retrospectively analyzed. Thereafter, children were divided into two groups according to whether intraoperative ureteral tailoring was performed, including 9 in the trimmed group and 12 in the nontrimmed group. In the meantime, there were 16 males and 5 females, with the age of 4.7 ± 2.12 years. Furthermore, there were 17 obstructive MGU cases, 2 reflux MGU cases, and 2 obstructive with reflux MGU cases, with 16 cases on the left side (including 1 case of Y-shaped ureter in the left duplicated kidney), whereas 5 on the right side. All children underwent transvesicoscopic ureteral reimplantation (the Cohen procedure). Thereafter, changes in operation time, duration of retained catheterization, hematuria time, renal pelvis and ureteral dilatation, and tortuosity between two groups were compared 3 months after Double-J tube removal. Results: All children were effectively treated with transvesicoscopic ureteral reimplantation, the operation time (p = 0.02, p < 0.05), postoperative hematuria time (p = 0.00, p < 0.05), and retained urinary catheter time (p = 0.00, p < 0.05) were shorter in the nontrimmed group, and the mean postoperative follow-up time was 27.6 (3-22) months. Moreover, the postoperative anteroposterior diameters of the affected renal pelvis in both groups were 0.31 ± 0.39 cm and 0.27 ± 0.29 cm, respectively, whereas the postoperative maximum ureteral diameters were 0.33 ± 0.31 cm and 0.27 ± 0.36 cm, separately, which were significantly reduced compared with the preoperative counterparts. All children recovered well, with no postoperative complications. Conclusion: Whether intraoperative ureteral clipping is performed or not does not significantly affect the outcome of transvesicoscopic laparoscopic ureteral bladder reimplantation for primary MGU in children. Both groups of children improved with satisfactory surgical results, but the nontrimmed group has shorter operation time, simpler operation, and less trauma.

Congenital venous anomalies associated with retrocaval ureter: evaluation using computed tomography.

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.

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.

Inverted-Y Ureteric Duplication With Paraureteric Diverticulum Presenting With Bladder Outlet Obstruction in an Infant- A Diagnostic Dilemma.

The ectopic ureter and paraureteric diverticulum are 2 known common urological anomalies of pediatric patients. Another rare entity is inverted-Y ureteric duplication. We report a case of a 3-month-old boy presented with bladder outlet obstruction, where surgical excision of large bladder diverticulum with left ureter and small kidney was done. Histopathology confirmed the presence of inverted-Y ureteric duplication with left dysplastic kidney. The report defines the first case of infantile bladder outlet obstruction having the co-existing congenital genitourinary anomaly of inverted Y-partial ureteric duplication with obstructive ectopic ureter and ipsilateral paraureteric diverticula.

Prognostic factors for continence after surgical correction of ectopic ureters of 51 dogs with long-term follow-up.

BACKGROUND: An ectopic ureter is a congenital malformation characterized by caudal displacement of one or both ureteral orifices and is the most common cause of urinary incontinence in young dogs. Complete resolution of incontinence after surgery has been reported in 25-82% of dogs. The aim of this study was to identify preoperative prognostic factors for continence after surgical treatment of dogs with an ectopic ureter. Dogs were included if surgical correction of an ectopic ureter was performed and at least 1 year follow-up was available. RESULTS: Fifty-one dogs met the inclusion criteria. The ectopic ureters were either intramural (91%) or extramural (9%). The ectopic ureters were bilateral in 49% of cases. Overall median follow-up time after surgery was 6.5 years (range 1-13 years). Surgical correction alone resolved urinary incontinence in 47% of cases. Low grade pre-operative incontinence, male sex and pre-operative presence of ureteral or renal pyelum dilation were significantly associated with urinary continence after surgery. CONCLUSIONS: Dogs with severe preoperative incontinence were less likely to become completely continent after surgery, whereas male sex and preoperative dilation of the ureter or renal pyelum were positive prognostic indicators for continence. These results may assist in predicting outcome after surgical correction of ectopic ureters and suggest assessment of pre-operative urethral pressure profiling in future studies.

Renal cell carcinoma coexistence with ipsilateral renal duplex system: a case report.

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.

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.

[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.

Concomitant occurrence of pyelocalyceal diverticulum and ipsilateral large sized orthotopic ureterocele in an adult: a rare association.

Pyelocalyceal diverticulum is an incidental finding detected on routine ultrasonography scans which may also show symptoms of recurrent urinary tract infections secondary to urinary stasis and calculi formation within the diverticulum. Orthotopic type of ureterocele occurs as an isolated abnormality and is mostly confined to adults as opposed to ectopic type of ureterocele which is congenital in origin and commonly seen in children and is almost always associated with a duplicated collecting system. We report a case of concomitant occurrence of pyelocalyceal diverticulum and ipsilateral large sized orthotopic ureterocele diagnosed on high-resolution ultrasonography and confirmed on contrast enhanced computed tomography in a 54-year-old adult female. The patient underwent resection of the ureterocele with insertion of left sided double-J ureteral stent which was left in place for 4 weeks following which the patient's symptoms improved and she was discharged after an uneventful post-operative recovery period.

Ectopic Vas Deferens in a Male Infant.

Vas deferens ectopia is a rare congenital anomaly associated with urinary tract and anorectal anomalies. It is important to recognize the complex embryology of the reproductive, urinary, and lower enteric systems to understand how these processes can go awry.1 As the ureteric bud arises from the mesonephric duct, a failure of the channel that connects the two to obliterate can produce an ectopic vas deferens connected to the ureter or bladder.2 We present a 2-month-old male with vas deferens ectopia. Our patient uniquely did not present with associated anorectal anomalies or urinary tract symptoms.

Cystoscopic-guided scissor transection of intramural ectopic ureters as a novel alternate minimally invasive treatment option to laser ablation in female dogs: 8 cases (2011-2020).

OBJECTIVE: To evaluate the safety and efficacy of cystoscopic-guided scissor transection of ectopic ureters (CST-EU) in female dogs. ANIMALS: 8 incontinent female dogs with intramural ectopic ureters. PROCEDURES: For this retrospective case series, data were collected from medical records of dogs that underwent CST-EU to relocate the ectopic ureteral orifice to an anatomically normal trigonal location between June 2011 and December 2020. Outcome after hospital discharge was determined using owner telephone questionnaires. RESULTS: Ectopic ureters were bilateral in 4 of the 8 dogs, and all dogs had other urogenital tract anomalies. Owner questionnaire follow-up was available for 7 dogs, and results indicated 6 dogs had improved urinary continence immediately following the procedure. At the last follow-up (44 to 3,384 days after CST-EU), 3 of the 7 dogs were completely continent with CST-EU alone, 3 others became continent or were markedly improved with the addition of medications for urethral sphincter mechanism incompetence, and 1 required ureteroneocystostomy, colposuspension, and an artificial urethral sphincter to become fully continent. Owners of 5 of the 7 dogs reported that they considered the outcome of CST-EU as good to excellent, and all owners reported that they would consider having CST-EU performed again should they have another incontinent dog. Complications were minor, and only 3 dogs showed transient lower urinary tract signs after CST-EU. CONCLUSIONS AND CLINICAL RELEVANCE: Results indicated CST-EU could provide a safe, effective, minimally invasive alternative in the absence of laser technology for the treatment of intramural ectopic ureters in female dogs.

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.

Duplex Collecting System with Ectopic Ureters Opening into Vagina: A Case Report.

Continuous urinary leakage, along with normal deliberate voiding, must suggest diagnosis of ectopic ureter, specifically in girls. Combination of a duplicated collecting system with distal, infra-sphincteric, vaginal insertion of ureter is an uncommon congenital anomaly and rare cause of urinary incontinence. We present a case report of a 7-year-old girl who presented to the urology department with urinary incontinence despite successful toilet training and history of recurrent urinary tract infections. Right duplex collecting system was seen on ultrasound. Magnetic resonance urography revealed a near complete right duplex collecting system with ectopic insertion of ureter into vagina, and aplastic uterus with bilateral normal ovaries suggestive of Mayer-Rokitansky-Kuster-Hauser syndrome. Surgical treatment consisted of "common sheath" reimplantation of ectopic ureters into bladder, with complete resolution of symptoms. This case suggests to us that congenital abnormalities of the genitourinary tract should be considered in case of urinary incontinence and recurrent urinary tract infections.

A Novel Transvesicoscopic Ureteral Reimplantation Technique to Treat an Infantile Ectopic Ureter in a Single System.

An ectopic ureter is a significant urinary tract malformation often treated during early childhood and is surgically managed based on its condition. Generally, only extravesical ureteral reimplantation is available as a treatment option for an ectopic ureter with well-preserved renal function. This report describes a case of a 28-months-old girl with an ectopic ureter in a single system who experienced repeated febrile urinary tract infections, successfully treated using a transvesicoscopic ureteral reimplantation as a minimally invasive treatment. To the best of our knowledge, this is the first report of transvesicoscopic ureteral reimplantation for an ectopic ureter in a single system.

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.