|Year : 2016 | Volume
| Issue : 1 | Page : 39-41
Solitary ureteric ectopia with incontinence: A case report and review of literature
Mamatha Basavaraju, Ninan Zachariah
Department of Pediatric Surgery, Pondicherry Institute of Medical Sciences, Puducherry, India
|Date of Submission||31-Mar-2016|
|Date of Acceptance||02-Jun-2016|
|Date of Web Publication||16-Jun-2016|
PIMS Quarters, C Block No 14, Ganapathichettikulam, Puducherry - 605 014
Source of Support: None, Conflict of Interest: None
A 5-year-old girl presented with continuous wetting with normal voiding pattern since birth. Ultrasonography (USG) showed a solitary left kidney. Excretory urography showed a normal left kidney. Right kidney was not seen but lower ureter was faintly delineated. Hence, contrast enhanced computed tomography (CECT) was done to look for any right kidney which was not seen on USG. CT showed a normal left kidney with a small ectopic kidney in right hemipelvis but ureteric orifice could not be traced to its insertion. Dimercaptosuccinic acid confirmed it to be a poorly functioning (6%) ectopic right kidney. Cystourethroscopy and vaginoscopy done could not localize the right ureteric orifice. The child underwent right nephrectomy along with excision of ectopic ureter inserting into vagina with complete resolution of her symptoms.
Keywords: Nephroureterectomy, solitary ectopia, urinary incontinence
|How to cite this article:|
Basavaraju M, Zachariah N. Solitary ureteric ectopia with incontinence: A case report and review of literature. J Curr Res Sci Med 2016;2:39-41
|How to cite this URL:|
Basavaraju M, Zachariah N. Solitary ureteric ectopia with incontinence: A case report and review of literature. J Curr Res Sci Med [serial online] 2016 [cited 2020 Nov 28];2:39-41. Available from: https://www.jcrsmed.org/text.asp?2016/2/1/39/184128
| Introduction|| |
In most cases, ectopic ureters arise from the upper pole of duplex moiety (80%) and single-system ectopias are very rare (20%). Compared to ectopic ureters of duplicated systems, the diagnosis of single-system ectopias can be delayed because they drain small dysplastic poorly functioning kidneys, which may be difficult to detect even with conventional imaging and cystourethroscopy/vaginoscopy. Our child is a case of single-system ureteric ectopia presenting with incontinence and our case report highlights the challenges in the diagnosis and treatment of this rare condition.
| Case report|| |
A 5-year-old girl presented with urinary incontinence since birth. She had constant wetting but also had a normal voiding pattern. Genital examination revealed a normal urethral and vaginal opening with pooling of urine in vagina. Ultrasonography (USG) showed only a solitary left kidney. Excretory urography (EU) showed a left bifid pelvis with normal ureter. Right kidney was not visualized but lower part of right ureter was seen [Figure 1]. Hence, the patient was taken for contrast-enhanced computed tomography (CECT) which showed a very small right kidney of 3 cm × 2 cm in ectopic position [Figure 2]. Micturating cystourethrogram was normal. Dimercaptosuccinic acid (DMSA) confirmed a right ectopic kidney in pelvis with 6% function. The patient underwent a cystovaginoscopy which showed a normal left ureteric orifice but right ureteric orifice could not be visualized. On exploration, a small right kidney was found in lower lumbar region [Figure 3] and a guidewire passed through the ureter exited out of the vagina which confirmed the ectopic insertion [Figure 4]. She underwent right nephroureterectomy in view of poorly functioning kidney with ectopic ureter and has attained normal continence postsurgery. HPE showed it to be a hypoplastic kidney with fibrosis of pelvicalyceal system and there were no dysplastic elements.
|Figure 1: Intravenous pyelogram showing lower part of right ureter (red arrow) but right kidney not visualized. Left kidney with bifid pelvis (black arrow) single ureter|
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|Figure 2: Contrast-enhanced computed tomography shows contrast in pelvis of ectopic small right kidney|
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|Figure 4: Guidewire passed through right ureter (white arrow) exiting out of vagina (black arrow)|
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| Discussion|| |
Many of the ectopic ureters remain asymptomatic and so the true incidence is not known. In one autopsy series, it was 1 in 2000. Most of the ectopic ureters are associated with complete duplication and single ureteric ectopias are very rare.  According to the China Birth Defect Monitoring Center, the incidence is 29 per million.  About 80% of female with ectopic ureters are associated with duplex systems. A majority of ectopia in males (75%) are singlets. Ectopic ureters always insert proximally to urinary sphincter in males so usually are continent. In females, those that are at or above bladder neck have continence. In females, the urethra and vestibule are the more common draining site (69%) and vaginal ectopia as similar to our case occurs only in 25%. 
Embryologically, if the ureteral bud arises from a more cranial position of the Wolffian duct, the ectopic ureteral orifice is carried caudally with the mesonephric duct and may get incorporated into any of the nearby müllerian duct structures. In females, it may get inserted to the vagina, uterus, cervix, or Fallopian tube More Detailss and in males, into the prostatic urethra, seminal vesicles, ejaculatory duct, or vas. Renal dysplasia is often associated with ureteral ectopia. According to the hypothesis by Stephens, the further the ureteral orifice is located from the trigone of the bladder the more severe is the ipsilateral renal dysplasia. 
Ultrasound can be useful in identifying a hydronephrotic collecting system and dilated ureter but may not be useful in ectopic and small dysplastic kidney. In most instances, the diagnosis of an ectopic ureter is confirmed by EU which may show a duplex hydronephrotic system or ectopic kidney with some function. However, EU may not be of much help in solitary ectopia with dysplasia which is poorly functioning as there may not be any excretion of contrast. Our case had some functioning parenchyma so some contrast was seen in right ureter. CECT gave a better delineation of the right ectopic kidney which was not seen on USG or intravenous urogram. However, there is usually a long delay in the diagnosis of an ectopic ureter. In a study by Carrico, in seven females with ectopic ureters draining dysplastic kidneys, he found that each patient underwent a mean of 10 diagnostic procedures, delay in diagnosis for a mean of 5.7 years, and were diagnosed and treated inappropriately. 
In a study by Milind Joshi et al., on eight girls with solitary ectopia, they stated that EU and renal scans have no role to play in diagnosing this rare condition and concluded that magnetic resonance urography is investigation of choice for localizing the ectopic ureteric coarse.  However, in our case, the conventional imaging still helped localize the ectopic poorly functioning kidney and clinical examination showing urine in vagina confirmed its insertion somewhere along the genital tract. In a study by Gangopadhyaya, he found that single ectopia was more common in females contrary to literature and were able to localize ectopic orifice in 77% of their patients by examination under anesthesia or cystovaginoscopy. Furthermore, intravenous pyelogram (IVP) was able to pick up an ectopic poorly functioning kidney in 61% of their patients whose function was 1-7% on DMSA.  This shows that IVP may still help in diagnosis even in a poorly functioning kidney as similar to our case.
If a girl child who is toilet-trained and has a normal voiding pattern presents with incontinence where there is a constant dribbling of urine during the night and day, it should raise a suspicion of ectopic ureter below the sphincter level. This along with a good clinical examination which may reveal pooling of urine in vagina or an ectopic orifice may be seen in the vestibule.
A micturating cystourethrogram must be performed to see for reflux. A Tc99 DMSA scan is required to give a clear picture about the function and salvageability and to decide whether the kidney has to be preserved or excised. If it is a poorly functioning kidney with ectopic ureter, a nephroureterectomy will be done. However, if the function is salvageable, the ureter will be reimplanted. ,, High index of suspicion along with appropriate investigations helps in diagnosing this rare condition. Solitary ectopia usually have dysplastic poorly functioning kidney and a nephroureterectomy will solve the problem in such cases.
| Conclusion|| |
Continuous urinary dribbling in a female child along with a normal voiding pattern should be investigated for ectopic ureter. Solitary kidney or ectopic kidney in the initial investigation in such children is highly suggestive of an ectopic ureter. Imaging studies and renal scans play an important role in deciding the management. Surgery depends on the degree of renal function. Nephroureterectomy is the treatment of choice for poorly functioning solitary ureteric ectopia.
We would like to acknowledge the Department of Radiodiagnosis for technical support in investigations.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Keating MA. Ureteral duplication anomalies: Ectopic ureters and ureterocele. In: Docimo SG, Canning DA, Khoury AE, editors. The Kelalis King Belman Textbook of Clinical Pediatric Urology. 5 th
ed. London: Informa Healthcare UK Ltd.; 2007. p. 600-1.
Liang J, Wang Y, Zhu J, Mu X. Epidemiological analysis of renal dysplasia. Modern Preventive Medicine 2003;11:200.
Mackie GG, Stephens FD. Duplex kidneys: A correlation of renal dysplasia with position of the ureteral orifice. J Urol 1975;114:274-80.
Carrico C, Lebowitz RL. Incontinence due to an infrasphincteric ectopic ureter: Why the delay in diagnosis and what the radiologist can do about it. Pediatr Radiol 1998;28:942-9.
Joshi M, Parelkar S, Shah H. Renal dysplasia with single system ectopic ureter: Diagnosis using magnetic resonance urography and management with laparoscopic nephroureterectomy in pediatric age. Indian J Urol 2009;25:470-3.
Gangopadhyaya AN, Upadhyaya VD, Pandey A, Gupta DK, Gopal SC, Sharma SP, et al
. Single system ectopic ureter in females: A single center study. J Indian Assoc Pediatr Surg 2007;12:202-5.
Ahmed S, Barker A. Single-system ectopic ureters: A review of 12 cases. J Pediatr Surg 1992;27:491-6.
Li J, Hu T, Wang M, Jiang X, Chen S, Huang L. Single ureteral ectopia with congenital renal dysplasia. J Urol 2003;170(2 Pt 1):558-9.
Borer JG, Bauer SB, Peters CA, Diamond DA, Decter RM, Shapiro E. A single-system ectopic ureter draining an ectopic dysplastic kidney: Delayed diagnosis in the young female with continuous urinary incontinence. Br J Urol 1998;81:474-8.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]