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

Abstract

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.

Highlights

• •Ectopic ureter is defined as any ureter, single or duplex, that opens in a location other than trigone of bladder.
• •Unlike functional problems of urinary incontinence, organic causes like EU have potential cure through surgical repair.
• •Diagnosis of EU as a cause of urinary incontinence is delayed.
• •When suspected, it has pathognomonic feature and can be confirmed with contrast study especially MRI.
• •Surgical management depends on the degree of renal function and location of EU.
• •Either nephroureterectomy or ureteric reimplantation are curative for incontinence.

1. Introduction

Urine incontinence in children is frequently due to functional problems and because of these treatments are frequently unsatisfactory. However, incontinence from organic causes, like an ectopic ureter (EU), is especially relevant since it has potential cure through surgical repair [1]. Ectopic ureter is defined as any ureter, single or duplex, that opens in a location other than trigone of bladder (i.e, urethra, vagina, bladder neck, prostate or other sex organs).

The prevalence of EU is uncertain Because many people with EU are asymptomatic and the diagnosis is frequently missed [1]. The male to female ratio is l/2. Ureteral duplication accounts for 80 % of ectopic ureter occurrences in women [3]. Ectopic ureters draining a single-system ectopic ureter with dysplastic kidneys, on the other hand, are uncommon, particularly in females [1]. A single renal collecting system is often present in men. In clinical settings, women are more likely to experience it than males do, with a ratio of 2.9 to 1 in an autopsy series. Bilateral ectopic ureter instances only make up 7.5–17 % of all cases. Meyer-Weigert-Rule predicts the draining pattern of duplex ureters in bipolar renal duplications. The upper pole is normally seen as ectopic and therefore dysplastic due to obstruction, whereas the lower pole is related to vesicoureteral reflux.

Pathognomonic feature of an infrasphincteric ureteral opening is normal urination together with persistent incontinence [2]. Due to insufficient assessment, the diagnosis of ectopic ureteral insertion is delayed. In fact, the majority of diagnostic techniques don't offer enough details about ectopic ureteral openings [3]. Imaging tests are used to diagnose the ectopic ureter and any related defects. The first imaging technique to demonstrate a dilated ureter down to its course and a hydronephrotic collecting system is ultrasound (US). However, in single-system ectopias where the kidney may be dysplastic and challenging to view, US may not be helpful. In this situation, computed tomography (CT) and magnetic resonance imaging (MRI) provide a better delineation of the ectopic kidney and non-dilated upper pole ureter draining ectopically. Prior to surgery, the renal function is evaluated by a renal scan [1].

The degree of renal function, accompanying anomalies, and the location of the EU all influence the surgical approaches chosen to treat a single-system EU. When the kidney is dysplastic and its function is limited (function of less than 10 %) with the contralateral kidney normal, nephroureterectomy is indicated. The preferred procedure for restoring continence and sparing the function of the remaining nephrons when kidney function is retained is ureteric reimplantation [1].

In this article, we want to introduce a 24-year-old patient with incontinence who has had it since she was a toddler but whose condition was difficult to diagnose using various diagnostic instruments and tests, such as CT and ultrasound. MRI, however, could confirm the diagnosis [3].

This case is reported on accordance with scare criteria [4].

2. Presentation of case

24 years old nulligravid Ethiopian woman came to our hospital outpatient department with a complaint of insensible continuous urinary leak since childhood. The leak was evidenced by wetness of her underwear. She had normal voiding pattern with normal sensation of her bladder fullness. The urine leak doesn't appear to be affected by cough or stressful activities. The delay in seeking medical care is due to fear of abandonment from family and society. The family was not aware of her condition since she was 10 years old with the assumption of her being cured of it.

She visited our hospital 2 years back but was lost from follow up for about a year due to lack of clear diagnosis for her condition. She had no previous history of pelvic surgery, trauma, lower urinary tract symptoms, or treatment for urinary tract infections. She had no history of previous known chronic medical illness. On physical examination of the genitourinary system, she had no costovertebral angle tenderness. She has normal urethral opening into vestibule and normal external genitalia. Per-Vaginal examination was unremarkable. No leak could be demonstrated with increased intraabdominal pressure.

The initial imaging done is abdominopelvic ultrasound which localized left normal kidney with non-visualized right kidney. Normal appearing pelvic organs. With suspicion of ectopic ureteric orifice, we ordered CTU and diagnostic cystourethroscopy. CTU showed left solitary kidney with normal course and insertion of ureter (Fig. 1). It failed to demonstrate the right kidney or ectopic ureter. Cystoscopic findings are normal urethra and bladder neck. The left ureteric orifice has normal insertion and morphology. The right UO couldn't be found in its normal insertion area or caudally inside urethra. Complete blood count, renal function test, serum electrolytes and urinalysis were all normal.

Fig. 1.

CT with contrast showed left solitary kidney (white arrow) with its ureter (black arrow) in normal position. It failed to demonstrate the right kidney or ectopic ureter.

Since CT failed to demonstrate ectopic UO, we ordered pelvic MRI (not dedicated MRU). It showed an ectopic insertion (vaginal) of Right ureter with an ectopic and possibly dysplastic right kidney (at the pelvic inlet - with a dilated calyx and a bifid pelvis) (Fig. 2). Even though the right kidney had dysplastic features on MRI, we wanted to assess functionality of the kidney before intervention. Since we didn't have renal scintigraphy (nuclear study) at the time of evaluation, we did IVP up to 24 h of contrast administration. There was no obvious nephrogram or contrast excretion despite waiting for long hours including 24 h. These IVP findings were suggestive of non-excreting kidney (NEK).

Fig. 2.

Right ureter is seen dilated and ends in the anterior wall of distal vaginal canal. A small right kidney (l = 4 cm) is seen at the pelvic inlet with a dilated bifid pelvis (scan was not a dedicated MRU to further comment on the kidneys).

With the information we had, we prepared her for surgery and did extirpation of right renal moiety along with the accessible ureter. Due to lack of less-invasive approaches like laparoscopy in our facility at the time of management, our approach was open surgery. Intraoperative findings were the kidney had no appreciable parenchyma (likely dysplastic/atrophic) (Fig. 3A). The pelvis was bifid with dilated ureter throughout its entire course (Fig. 3). Ureteric dissection was not extensive enough to appreciate its insertion to vagina. Intraoperative course was smooth, minimal blood lose and no drain was left. Post op course was smooth, and she was discharged on the 3rd post OP day. After discharge she was seen twice (1st and 4th month) at outpatient clinics. Follow up ultrasound and RFT were unremarkable. She doesn't have any urinary complaint. She has resumed her routine work.

Fig. 3.

Intraoperative findings: The right renal moiety had no appreciable parenchyma (likely dysplastic/atrophic). It has lateral (B-4) and proximal attachment (B-5) with visible vessels in it. The pelvis was bifid (A & B - 2) with dilated ureter throughout its entire course (A & B – 1). Cecum and Appendix are denoted with no 3.

Histologic section through the atrophied kidney showed areas of normal glomeruli with adjacent normal tubules (Fig. 4A & B) and focus of tubular thyroidization (filled with colloid casts), tubular atrophy, interstitial fibrosis and inflammation (Fig. 4C) consistent with chronic pyelonephritis. Histologic section through the ureter showed urothelium lining with underlying lamina propria, and thick muscularis propria (Fig. 4D).

Fig. 4.

Histologic section through the atrophied kidney showed areas of normal glomeruli (arrows) with adjacent normal tubules (A & B) and focus of tubular thyroidization (filled with colloid casts), tubular atrophy, interstitial fibrosis and inflammation (C) consistent with chronic pyelonephritis. Histologic section through the ureter showed urothelium lining with underlying lamina propria, and thick muscularis propria (D).

3. Discussion

Ectopic ureter is a congenital anomaly, however, due to insufficient assessment of the patient and evaluations, a diagnosis cannot usually be determined until an advanced age. As the patient ages, various causes of urine incontinence are increasing, considered, and eventually, the diagnosis of the ectopic ureter is disregarded [3]. As in the case of our patient, adulthood, the diagnosis of ectopic ureter is entertained after thorough imaging and full investigations because of the patient's lack of adherence to clinical appointments, and financial instability.

In females, the ureteral opening may be located in the urethra, in the vagina, in the uterus, or in the fallopian tubes along the mullerian duct structures that bypass the urethral external sphincter [3]. Twenty-five percent of ectopic ureter open into the vagina [1].

Similar to the present patient, girls with ectopic ureters can display the typical symptom of continuous incontinence following toilet training with an otherwise typical voiding pattern. Additionally, they might have chronic vaginal dribbling or dampness, which might develop later in life. Urine can be seen coming from the vaginal orifice or vestibule under close observation [5].

Even though a wide range of important diagnostic techniques are available, such as ultrasound, voiding cystourethrography, intravenous urography, computed tomography, and cystoscopy, we believe that these imaging modalities fall short in terms of ectopic ureteral opening information [3]. In our case, The CTU revealed a left single kidney with a normal course and ureter insertion. The right kidney and ectopic ureter were not shown. MRI revealed an ectopic and possibly dysplastic right kidney along with the right ureter's vaginal insertion (at the pelvic inlet - with a dilated calyx and a bifid pelvis).

Ectopic ureters are frequently linked to renal maldevelopment, which is believed to be brought on by an aberrant interaction between the ureteral bud and the metanephric blastema; the further away the ureteral orifice, the more severe the renal dysplasia. About 2.5 % of renal abnormalities are dysplastic kidneys, and ectopic ureters are frequently seen in these cases [5]. The level of renal dysplasia can cause very small, dysfunctional kidneys that are challenging to see on imaging investigations in single-system ectopic ureters. When the dysplastic renal unit is located abnormally, as it was in this patient, whose remnant atrophic, ectopic kidney was near the pelvic inlet, radiographic localization becomes more difficult [5].

The goal of treatment for patients with ectopic ureters and dysplastic kidneys is to ameliorate their symptoms as expeditiously as possible [5]. Open surgery with extirpation of the right renal moiety with ureter is done to relieve her incontinence This patient had no incontinence after surgery. Histologic sections showed atrophied kidney with interstitial fibrosis and inflammation.

4. Conclusion

In conclusion, in the investigation for incontinence, proper diagnostic methods should be used, and in differential diagnosis, ectopic ureter openings should be kept in mind irrespective of the patient's age.

Delays in diagnosis often result from the difficulty in uncovering these dysfunctional renal units with standard imaging modalities. Surgical extirpation is curative but requires preoperative radiographic identification and localization of the offending renal moiety and its ectopic ureter.

In cases where an ectopic dysplastic kidney is suspected, MRI may increase diagnostic sensitivity as compared to other imaging modalities for detecting small, poorly functioning renal remains, guiding cystourethroscopy, and aiding both preoperative planning and intraoperative localization of the small piece of ectopic tissue.

Sources of funding

There is no source of funding found for this research paper.

Patient (parent's) consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Author contribution

• 1.Nuru Bedru, MD, Urology resident: conceived, wrote, and submitted the report. Involved in the diagnosis, management and follow up of the patient.
• 2.Ibsa Daba,MD, Urology resident: Operated on the patient. Involved in the writing of the report and in the follow up of the patient
• 3.Abeselom Lemma, MD (Assistant professor of urology): Operated on the patient. Reviewed the case report.

Guarantor

Abeselom Lemma Gebreamlak

abeselom.lemma@aau.edu.et

Disclosure statement

No competing financial interests exist.

Ethical approval

Ethical approval was provided by the author's institution.

Research registry

N/A

Declaration of competing interest

All authors declare that they have no conflict of interest.