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Table of Contents
CASE REPORT
Year : 2022  |  Volume : 5  |  Issue : 1  |  Page : 40-42

Laparoscopic Nephroureterectomy for Dysplastic Kidneys with Ectopic Ureters Opening Inside the Vagina


1 Department of Pediatric Surgery, Faculty of Medicine, Sirimavo Bandaranaike Specialized Children's Hospital, University of Peradeniya, Kandy, Sri Lanka
2 Department of Pediatrics, Faculty of Medicine, Teaching Hospital Peradeniya, University of Peradeniya, Kandy, Sri Lanka

Date of Submission14-Mar-2021
Date of Decision30-May-2021
Date of Acceptance04-Nov-2021
Date of Web Publication28-Jun-2022

Correspondence Address:
Mathula Hettiarachchi
Department of Paediatric Surgery, Faculty of Medicine, Sirimavo Bandaranaike Specialized Children's Hospital, University of Peradeniya, Kandy
Sri Lanka
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ajpn.ajpn_11_21

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  Abstract 


Urinary incontinence in girls could be due to ectopic ureteral insertion inside the vagina, a condition that is difficult to diagnose. In these patients, the urinary tract may be duplicated or single and be associated with a dysplastic kidney. We report two girls with dribbling of urine due to single ectopic ureteral insertion into the vagina. Computerised tomography urogram, retrograde contrast study and isotope scan revealed poorly functioning ectopic kidney. Hence, both patients underwent laparoscopic nephroureterectomy which led to the resolution of symptoms.

Keywords: Duplex kidney, ectopic ureter, laparoscopic nephroureterectomy, renal dysplasia


How to cite this article:
Hettiarachchi M, Thalgahagoda S. Laparoscopic Nephroureterectomy for Dysplastic Kidneys with Ectopic Ureters Opening Inside the Vagina. Asian J Pediatr Nephrol 2022;5:40-2

How to cite this URL:
Hettiarachchi M, Thalgahagoda S. Laparoscopic Nephroureterectomy for Dysplastic Kidneys with Ectopic Ureters Opening Inside the Vagina. Asian J Pediatr Nephrol [serial online] 2022 [cited 2022 Aug 18];5:40-2. Available from: https://www.ajpn-online.org/text.asp?2022/5/1/40/348521




  Introduction Top


Urinary incontinence in girls could be due to ectopic ureteral insertion into the vagina, either in a duplicated system or a single system. Continuous dribbling of urine after the age of toilet training should raise the suspicion of an ectopic ureter. A completely duplicated system with an ectopic ureter account for approximately 80% of cases while the remainder of patients have a single system. In most cases, the ipsilateral kidney is dysplastic and may be difficult to locate or identify on conventional imaging and nuclear scintigraphy due to poor radiotracer uptake. We describe two children with continuous urinary incontinence in whom ectopic ureteral insertion was detected into the vagina. While one child had a single system ectopic ureter, the other had a duplex system with a single ectopic ureter. Both children had ipsilateral poorly functioning dysplastic kidney and underwent laparoscopic nephroureterectomy, leading to resolution of symptoms.


  Case Reports Top


Case 1

A 7-year-old girl presented with persistent dribbling of urine noted since toilet training. She had been diagnosed with urinary tract infection once at 9 months of age. An ultrasound scan revealed normal left kidney and smaller right kidney with mild hydronephrosis. Computed tomography with urography revealed a small right kidney with minimal contrast excretion into the ureter, indicating reduced function. Since the kidney was poorly functioning, a diuretic renogram was not performed and the mild hydronephrosis was not considered indicative of downstream obstruction.

Since the dribbling was persistent, cystoscopic examination was performed. While the left ureteric orifice was normal, the right ureteric orifice was not visualized in the bladder. On suspicion of an ectopic ureter, vaginoscopy was performed, which revealed an abnormal slit-like opening on the anterior wall of the vagina. The opening was cannulated by a ureteric catheter to perform a retrograde contrast study, which confirmed that the right ureter was ectopically inserted into the vagina [Figure 1].
Figure 1: Retrograde contrast study performed through the ureteric catheter inserted into the ectopic ureteral opening in the anterior wall of the vagina. The ectopic right ureter and the collecting system are seen as opacified

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To assess the function of the kidneys, a Tc99m dimercaptosuccinic acid (DMSA) scan was performed. The right kidney was visualized only faintly in the right loin and had a differential function of 3%. During laparoscopic exploration, the right ureter was traced upwards to detect a contracted right kidney (size 3.5 cm × 2 cm) located at a relatively low level in the loin. Nephroureterectomy was performed as definitive management. Dribbling of urine resolved completely following this intervention.

Case 2

A 3.5-year-old girl presented with persistent dribbling of urine since the age of toilet training. There was no history suggesting urinary tract infections. Ultrasonography revealed a normal right kidney but the left kidney was not demonstrated. Cystoscopy revealed a single right ureteric opening. The left ureteric opening was not seen in the bladder. On vaginoscopy, an abnormal opening was seen on the left lower wall of the vagina. The insertion of ureteric catheter was attempted which did not progress, probably due to a kink in the ureter. A computerised tomography urogram, performed to delineate the left kidney, revealed a low-lying left kidney with poor excretion of contrast, with delayed images indicating a trace of contrast in the ureter, possibly ending up in an ectopic location. Laparoscopic exploration revealed a low-lying duplex kidney over the left iliac vessels, with a Y shaped configuration of the ureter. Since DMSA scan had not shown any demonstrable function of the left kidney, both moieties of the left kidney appeared dysplastic and nonfunctional. Laparoscopy had revealed a single ureter coursing over the pelvis into the vagina. Laparoscopic nephroureterectomy was performed with the ureter resected as low as possible in the pelvis. The dribbling resolved completely after the surgery.

The anthropometric parameters and blood pressure of both children were appropriate to the age. Neither girl had any suggestion of dysmorphism. Urinalysis did not reveal proteinuria and serum creatinine levels were normal.


  Discussion Top


Ectopic ureters are common in girls and are associated with a duplicated ureter in 80% of instances.[1] Patients with single system ectopic ureters usually have systemic anomalies, such as the VACTERL association.[1],[2] Embryologically, when the ureteric bud arises more proximally from the mesonephric bud, the ureteric insertion might be more distal in the bladder or lower.[2] Ectopic ureters may insert at any location between the bladder neck and seminal vesicles in boys or the mesonephric remnants in girls. The abnormal interaction of more caudally oriented abnormal ureteric buds with the mesonephric bud leads to renal dysplasia ipsilaterally.

The presenting symptoms depend on the location of the ectopic ureter. If the ureter opens within the bladder, ureteric obstruction, reflux, and recurrent urinary tract infections may occur.[3] In girls, ectopic ureters opening distal to the bladder neck cause persistent urinary dribbling between voids, as in the two patients presented here.[3]

The diagnosis of ectopic ureter is challenging due to the dysplastic nature of the kidney, which often evades detection in imaging. In one large series, cystoscopy and retrograde study of the kidney and the ectopic ureter were more conclusive for the diagnosis, like in the first patient presented.[4] Computed tomography with delayed images to visualize contrast excretion is a sensitive method to diagnose ectopic ureters and to localize the dysplastic kidneys prior to surgical intervention.[4],[5] However, the investigation exposes patients to radiation. Magnetic resonance urography may provide more specific anatomic and functional information about the kidneys and ureters in children with complex renal anomalies. A DMSA scan, as performed in both patients, helps assess the function and location of the kidney.

Once the diagnosis is made, the type of surgical intervention is decided based on the function of the ipsilateral kidney. Ureteric re-implantation to enable renal salvage should be considered if the differential function is more than 10%. If the function is <10%, as in both of our patients, nephroureterectomy is considered the definitive treatment.[5],[6] Laparoscopic examination and nephroureterectomy are invaluable and minimally invasive for the localisation as well as removal of the affected kidney.[6],[7] During laparoscopic exploration, the ureter is used as a guide to detect the dysplastic kidney. In both our patients, laparoscopic nephroureterectomy led to an immediate improvement in symptoms.

To conclude, single ectopic ureteric insertion into the vagina is a rare anomaly that is a diagnostic dilemma. The diagnosis depends on the use of multiple diagnostic modalities, including ultrasonography, contrast urography, nuclear imaging, and cystoscopic examination with retrograde contrast studies. Laparoscopy is useful in localizing the dysplastic kidney and can be combined with nephroureterectomy as a feasible and safe procedure in patients where the ectopic ureter is associated with a poorly functioning kidney.

Acknowledgments

We would like to extend our sincere gratitude to both patients and parents for giving their consent for the publication.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Blane CE, Ritchey ML, DiPietro MA, Sumida R, Bloom DA. Single system ectopic ureters and ureteroceles associated with dysplastic kidney. Pediatr Radiol 1992;22:217-20.  Back to cited text no. 1
    
2.
Wakhlu A, Dalela D, Tandon RK, Chandra H, Wakhlu AK. The single ectopic ureter. Br J Urol 1998;82:246-51.  Back to cited text no. 2
    
3.
Yan YJ, Feng ZX, Min ZH, Jin YX. Single-system ectopic ureters associated with renal dysplasia. Pediatr Surg Int 2004;20:851-4.  Back to cited text no. 3
    
4.
Chowdhary SK, Lander A, Parashar K, Corkery JJ. Single-system ectopic ureter: A 15-year review. Pediatr Surg Int 2001;17:638-41.  Back to cited text no. 4
    
5.
Hanson GR, Gatti JM, Gittes GK, Murphy JP. Diagnosis of ectopic ureter as a cause of urinary incontinence. J Pediatr Urol 2007;3:53-7.  Back to cited text no. 5
    
6.
Roy Choudhury S, Chadha R, Bagga D, Puri A, Debnath PR. Spectrum of ectopic ureters in children. Pediatr Surg Int 2008;24:819-23.  Back to cited text no. 6
    
7.
Wallis MC, Khoury AE, Lorenzo AJ, Pippi-Salle JL, Bägli DJ, Farhat WA. Outcome analysis of retroperitoneal laparoscopic heminephrectomy in children. J Urol 2006;175:2277-80.  Back to cited text no. 7
    


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