Bilateral single-system ectopic ureters: a case report

Abstract

Introduction

While 20% of ectopic ureters are bilateral, bilateral single-system ectopic ureters constitute a rare occurrence. They present as continuous incontinence in females owing to the ectopic ureteral openings being located outside the bladder. Managing bilateral single-system ectopic ureters can be challenging owing to the reduced bladder capacity and incompetent bladder neck. Major reconstructive procedures, such as bladder augmentation and bladder neck reconstruction, may be necessary in addition to ureteral reimplantation to address these issues.

Case presentation

A 14-year-old Ethiopian female adolescent presented with lifelong urinary incontinence and recurrent urinary tract infections. Physical examination was unremarkable, with no signs of neurologic disorder. Abdominal ultrasound imaging showed bilateral moderate hydroureteronephrosis. The voiding cystourethrogram showed a bladder capacity of 150 ml and a grade 4 vesicoureteral reflux in the left ureter. Computed tomography urography revealed a low-capacity bladder with bilateral ectopic ureteral openings and bilateral moderate hydroureteronephrosis. Cystoscopic examination revealed a wide urethra, with both ureteric orifices located within the urethra and an indistinct trigone. Bilateral Politano-Leadbetter ureteric reimplantation was then performed, which resulted in satisfactory continence during follow-up.

Conclusion

Bilateral single-system ectopic ureters represent an infrequent clinical entity. In such cases, modern imaging techniques can guide surgical planning, and in selected patients, ureteral reimplantation may allow normal bladder function and satisfactory continence without major reconstructive surgery.

Introduction

Bilateral single system ectopic ureters constitute one of the most uncommon urological conditions, with fewer than 80 reported cases documented in literature [1]. Urinary incontinence and recurrent urinary tract infections are the commonly reported main complaints. The most significant challenge for these cases is how to maintain bladder size and functional development as the outcome of the surgical intervention while preserving renal function [2].

We present the case of a 14-year-old female adolescent diagnosed with bilateral ectopic ureters who underwent bilateral ureteric reimplantation, achieving satisfactory continence without the need for major reconstructive surgery.

Case report

A 14-year-old Ethiopian female adolescent presented with continuous dribbling of urine without any voiding stream since birth. She also has a history of recurrent urinary tract infections. The patient had undergone surgical repair for an anorectal malformation with a rectovestibular fistula at the age of 1 year at another hospital, after which she was unable to continue follow-up care owing to financial constraints. She experienced occasional soiling associated with this. Otherwise, she had no other complaints. She had no known history of similar illness in her family.

Physical examination was unremarkable, with normal external genitalia and no neurologic abnormalities. All routine investigations, including hemoglobin, total leukocyte count, renal function tests, and urine microscopic examination, were within the normal range. Abdominal sonography revealed bilateral moderate hydronephrosis and hydroureters.

Computed tomography (CT) urography revealed a low-capacity bladder with bilateral ectopic ureteral openings, accompanied by a moderate bilateral hydroureter. Coronal and three-dimensional reconstruction images facilitated the assessment of the anatomical orientation of the kidneys, ureters, and bladder (Fig. 1). The voiding cystourethrogram revealed a bladder capacity of 150 ml, with a grade 4 vesicoureteral reflux observed in the left ureter (Fig. 2).

Fig. 1

Computed tomography scan (coronal view A) and three-dimensional reconstruction image B showing bilateral single-system ectopic ureters and lumbar scoliosis

Fig. 2

Voiding cystourethrogram showing A distended urinary bladder and B high grade left vesicoureteral reflux (black arrow: left ureter)

Upon examination under anesthesia, the urethral and vaginal openings appeared normal, but the patient was continuously leaking urine from the urethral opening. No other abnormal openings were observed. Cystoscopic examination revealed a wide urethra, with both ureteric orifices located within the urethra and an indistinct trigone (Fig. 3).

Fig. 3

Cystoscopy showing both ectopic ureteric orifices opening in the urethra and bladder neck. Arrows indicate lumen of urethra, ureter, and bladder neck

After a diagnostic cystoscopy confirmed the diagnosis, surgical intervention in the form of bilateral ureteric reimplantation was planned. Through a Pfannenstiel incision, the pelvic organs were explored, and the bladder and both ureters were located. Bilateral Politano-Leadbetter ureteric reimplantation was then performed (Fig. 4). During the early postoperative period, the patient maintained daytime continence but needed to void frequently. Nocturnal incontinence was noted.

Fig. 4

Intraoperative pictures showing A both ureters and posterior wall of the urinary bladder (star), B opened bladder, and C completed Politano-Leadbetter reimplantation with stents in situ. Blue arrows indicate area of ureteric insertion

Over the initial 6-month period, the patient was followed monthly, during which time a gradual improvement in her continence and urinary voiding patterns was observed. The follow-up schedule has since transitioned to every 3 months. During the most recent evaluation, the patient reported experiencing 2–3 monthly episodes of nocturnal incontinence. Renal function tests were normal, and abdominal ultrasound findings were unremarkable. Cystourethrography was not feasible owing to financial constraints.

Discussion

Ectopic ureter is a condition in which the ureter does not open into the trigone region of the urinary bladder. Ectopic ureters have an incidence ranging from 1 in 2000–4000 individuals. The female-to-male ratio for this condition is 2–6:1, with a higher prevalence observed among females. Ectopic ureters are frequently observed in conjunction with a duplicated urinary tract system, which is the case in approximately 80% of incidents. In contrast, single-system ectopic ureters are less prevalent, constituting 10–20% of occurrences. Bilateral single-system ectopic ureters are exceedingly rare among all cases [3].

In female patients with bilateral single-system ectopic ureters, the development of the trigone region and bladder neck musculature is impaired. This is owing to the ectopic ureteral openings being located outside the bladder, which consequently lacks the ability to distend with urine. Furthermore, a poorly developed bladder neck and an improperly functioning urethral control mechanism prevent bladder growth [4]. The most common presenting symptoms are urinary tract infection (UTI), incontinence, and renal insufficiency [5]. Similarly, our patient presented with urinary incontinence and a history of recurrent urinary tract infections. She also had a history of occasional fecal soiling associated with a prior anoplasty procedure for an anorectal malformation with a rectovestibular fistula. According to a study by Sarah et al. analyzing 2283 patients with anorectal malformation, the incidence of ectopic ureter was found to be 3.5% among this patient population [6]. Diagnosing ectopic ureter can be challenging, as most conventional radiologic techniques, such as ultrasound and voiding cystourethrogram, often fail to clearly visualize the ectopic ureter. Excretory urography can visualize ectopic ureteral insertion. However, the renal unit from which the ureter originates must have functional parenchyma in order for the excreted contrast agent to opacify the ureter. Consequently, CT or magnetic resonance (MR) urography are the established techniques for the diagnostic evaluation of ectopic ureter. Of these options, MR urography demonstrates superior performance owing to its provision of clear anatomical details and avoidance of ionizing radiation exposure. Urodynamic studies and renal scintigraphy are also crucial for the perioperative evaluation of bladder and renal function, respectively [7,8,9]. CT urography in our case clearly demonstrated the ectopic insertion of the ureters and a small bladder size.

Surgery is the best treatment for this condition, as it resolves incontinence, preserves renal function, and eliminates recurrent UTIs [7]. Unused urinary bladder secondary to bilateral single system ectopic ureters has small capacity and is nonfunctional. ureteric reimplantation is diificult in small capacity bladder and the functional outcome is also poor secondary to poorly developed muscles and innervation [10].

The appropriate management of ectopic ureters remains controversial, with options including ureteral reimplantation alone, ureteral reimplantation with bladder augmentation and reconstruction, or bladder neck closure with continent urinary diversion [11]. There are also reports of staged operations. First, ureterovesicostomy between the dilated ureter and the lateral wall of the bladder was performed to increase bladder capacity. Second, ureterocystostomy was performed after the bladder capacity had increased enough to allow for reimplantation [4, 12].

There are few reported cases of successful management of bilateral ectopic ureters through bilateral reimplantation alone [2, 3, 10, 11, 13]. Stavrinides et al. studied eight children with bilateral single-system ectopic ureters (BSSEU) and found that continence was linked to preoperative bladder capacity. Children with bladder filling to at least 30% of expected bladder capacity for age on preoperative ultrasound or apposition of the Bladder neck (BN) at cystoscopy were able to achieve satisfactory continence after ureteric reimplantation alone (9). Our patient had a bladder capacity exceeding 30% of the expected volume, which may be attributed to the insertion of the ureters into the proximal urethra, leading to the reflux of urine into the bladder.

Our patient had an acceptable bladder capacity of 150 ml, and both ureters were opening in the urethra. We performed bilateral ureteral reimplantation, and the patient was able to achieve normal bladder function and satisfactory continence.

Conclusion

Bilateral single-system ectopic ureters are an uncommon condition. In such cases, modern imaging techniques can clearly delineate the anatomy and guide appropriate surgical planning. In selected cases, ureteral reimplantation can avoid the need for major reconstructive surgery, allowing patients to achieve normal bladder function and satisfactory continence.