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Ectopic Ureter and Ureterocele

Ectopic ureter and ureterocele are congenital anomalies of ureteral development in which the ureteral orifice or the terminal ureter is malpositioned, most often in association with a duplicated collecting system. Both are usually diagnosed and treated in childhood, but reconstructive urologists and urogynecologists also see adults with these anomalies: women with lifelong urinary leakage from an undiagnosed ectopic ureter, adults with an incidentally discovered ureterocele, and adults with recurrent infection, incontinence, or impaired emptying after a childhood ureterocele repair.[1][2] This page is framed for the adult presentation; childhood decompression technique and outcomes are summarized only as background to explain how a patient arrives at adult reconstructive care.

Anatomy and Embryology​

Both anomalies arise from abnormal development of the ureteral bud and its relationship to the trigone. In a duplicated system, the Weigert-Meyer relationship places the lower-pole ureter laterally and cephalad on the trigone (at risk for reflux) and the upper-pole ureter medially and caudad, tracking distally toward the bladder neck or urethra, where it may end in a ureterocele or as a frankly ectopic orifice.[1] See GU Embryology for the underlying developmental anatomy.

  • A ureterocele is a cystic dilation of the submucosal terminal ureter. An intravesical (orthotopic) ureterocele lies entirely within the bladder; an ectopic ureterocele extends through the bladder neck into the proximal urethra.
  • An ectopic ureter opens outside the normal trigonal location: in women, classically into the urethra, vagina, or vestibule, distal to the external sphincter, so that it continuously leaks urine independent of voiding; in men, the ectopic orifice typically terminates in the prostatic urethra, seminal vesicle, or vas deferens, proximal to the external sphincter, so continence is usually preserved and the anomaly instead presents with infection.[3]

Adult Presentations​

Ectopic ureter in women: lifelong continuous leakage​

The classic adult presentation of an ectopic ureter is a woman with normal voiding and a pattern of continuous urinary leakage that she describes as lifelong, often attributed for years to another cause of incontinence. In the largest reported adult case series (9 women and 1 man referred over 10 years, mean age 37, range 20-58), all nine women presented with this lifelong leakage pattern; three had already undergone heminephrectomy or nephrectomy elsewhere with the distal ureteral stump left in place.[4] Fine-slice MRI was the predominant diagnostic tool in this series but often required senior radiology review before the diagnosis was confirmed, underscoring that a reflexive "normal" pelvic MRI read does not exclude a small ectopic ureter terminating in the vaginal wall or vestibule.[4]

Ectopic ureter in men​

Men are far less often affected. A classic description is a nonduplicated system draining a dysplastic or poorly functioning kidney, with the ectopic orifice terminating in the seminal vesicle or vas deferens; presentation is usually with recurrent epididymitis, a boggy periprostatic mass on rectal examination, or obstructive and irritative voiding symptoms rather than incontinence, since the ectopic orifice lies proximal to the external sphincter.[5]

Ureterocele discovered in adulthood​

An adult-presenting ureterocele is most often an incidental finding on imaging obtained for an unrelated reason, since many ureteroceles that caused no childhood symptoms are never previously diagnosed. Reported adult presentations include incidental bilateral ureteroceles found during the workup of hypertension and a ureterocele found at the time of staging or treatment for prostate cancer.[6][7] An asymptomatic, nonobstructed, nonrefluxing ureterocele with a functioning moiety can reasonably be observed; a ureterocele causing obstruction, recurrent infection, or stone formation, or one found incidentally during planned pelvic surgery such as radical prostatectomy, is managed according to its anatomy and the function of the affected renal unit, as described below.[1][2][3]

Sequelae of childhood ureterocele repair​

Adults who had a ureterocele decompressed or excised in childhood can present years later with recurrent infection, incontinence, or difficulty emptying. One proposed mechanism is inadequate trigonal support left behind by the anomaly itself: a weak bladder base can balloon behind the bladder neck and obstruct emptying, and a urethral or cecoureterocele component can contribute further.[9] Reported series disagree on how often this occurs. In one series, bladder dysfunction (infrequent voiding with possible infection) was described in 19 of 36 patients and incontinence in 3 of 36, while other published series of ureterocele excision found no significant bladder dysfunction; these are differing single-center observations, not a consistent rate.[9][10] When this picture is suspected, evaluation has been described with lateral-view VCUG and videourodynamics, and with antegrade cystoscopy performed through a suprapubic puncture to directly assess the bladder neck and trigone.[9] Depending on findings, management options include bladder or trigonal reconstruction, bladder-neck repair, intermittent catheterization, or injection of a bulking agent for demonstrated sphincteric incompetence.[9]

Background: why some adults have already had more than one childhood procedure​

Endoscopic transurethral incision or puncture is the standard first-line childhood treatment for an obstructing ureterocele, but it carries a real reoperation risk that helps explain why an adult patient may arrive with a history of more than one prior procedure. A systematic review and meta-analysis of pediatric series found that ectopic ureterocele location, a duplex (versus single) collecting system, and preoperative reflux were each associated with a significantly higher reoperation rate after endoscopic incision, though having more than one of these risk factors did not further increase the risk.[8] A single-institution review of 83 children undergoing transurethral incision found "cure" (resolved hydronephrosis without reflux) in 55.6% of single-system ureteroceles versus only 14.9% of duplex-system ureteroceles, de novo reflux into the ureterocele moiety in 27.8% versus 56.2%, and a second surgery required in 3.8% versus 73.7%, respectively.[11] These are pediatric outcome data presented as background, not as a current recommendation for how to treat a ureterocele found in an adult.

Evaluation in the Adult​

  • Fine-slice MRI is the preferred imaging study when an ectopic ureter is suspected, particularly in a woman with lifelong leakage and an otherwise unremarkable standard workup; senior radiologic review is often needed to identify a small terminal ectopic segment.[4]
  • CT or MR urography and renal scintigraphy define the anatomy of the affected renal unit or moiety, document split function, and identify an associated dysplastic or nonfunctioning segment.
  • VCUG and, when indicated, videourodynamics assess reflux, bladder-neck competence, and emptying, particularly when evaluating bladder dysfunction after a prior childhood repair.[9]
  • Cystoscopy confirms orifice position and the bladder-neck relationship; an overdistended bladder can collapse a ureterocele and obscure it on both imaging and cystoscopy, so this is considered when a suspected ureterocele is not seen as expected.

Management in the Adult​

Ectopic ureter​

Treatment choice depends mainly on whether the renal segment drained by the ectopic ureter retains useful function.

  • Nephroureterectomy or heminephrectomy with excision of the distal ureteral stump is preferred when the affected renal segment is nonfunctioning or minimally functioning, which is common given the often dysplastic nature of the ectopic moiety.
  • Excision of the ectopic ureter with bladder-neck reconstruction is used when continence, rather than renal salvage, is the primary problem and when leaving a functioning segment undrained is not acceptable, or when a nonfunctioning segment has already been removed elsewhere and the residual distal ureteral stump is the source of ongoing leakage. In the largest adult series, excision with bladder-neck reconstruction (with a concomitant autologous fascial sling in one patient) was performed in 6 of 9 women; stress incontinence was cured by the bladder-neck reconstruction alone in 4 of those 6 (67%), while the remaining 2 required further incontinence surgery. Three women were treated with nephroureterectomy alone, of whom 2 were cured.[4]
  • Malignancy risk in a retained stump. When a nonfunctioning moiety has been removed elsewhere with the distal ureteral stump left in situ, that retained segment is not inert: malignancy arising in a retained ureteral remnant has been described in the world literature at a rate the same series's authors estimated at 8%, and patients in whom the stump is left in place should be counseled about this risk and followed accordingly.[4]

Ureterocele​

  • Observation is reasonable for a small, asymptomatic, nonobstructing, nonrefluxing ureterocele with preserved moiety function, particularly when found incidentally in an adult who has had no symptoms attributable to it.
  • Endoscopic decompression (transurethral incision or puncture) can relieve obstruction or infection in a symptomatic adult, following the same principle used in children, though adult-specific outcome data are limited to case reports.
  • Definitive reconstruction is selected by anatomy and the function of the affected moiety: heminephrectomy for a nonfunctioning upper-pole segment, or ureteroureterostomy or formal ureteral reimplantation with ureterocele excision when the segment is salvageable and definitive anatomic correction is indicated. Technique and outcomes for these reconstructions are covered on their own procedure pages rather than repeated here.
  • Incidental ureterocele during planned pelvic surgery. When a ureterocele is found during evaluation for an unrelated pelvic procedure, such as radical prostatectomy, treatment at the same setting (for example, unroofing an obstructed ectopic ureterocele) has been described, while an asymptomatic ureterocele with a normally functioning renal unit can reasonably be left untreated if it is not expected to complicate the planned surgery.[7]

See Also​

References​

1. Stanasel I, Peters CA. Ectopic Ureter, Ureterocele, and Ureteral Anomalies. In: Partin AW, Dmochowski RR, Kavoussi LR, Peters CA, eds. Campbell-Walsh-Wein Urology. 13th ed. Elsevier; 2025:1038-1063.

2. Lee UJ, Palmer JS. Endoscopic and Open Surgical Management of Ureteroceles and Ectopic Ureters. In: Montague DK, Gill IS, Angermeier KW, Ross JH, eds. Textbook of Reconstructive Urologic Surgery. Informa Healthcare; 2008:192-199.

3. Fahmy MAB. Ureterocele. In: Abdel-Gawad M, Ali-El-Dein B, Barry J, Stenzl A, eds. The Ureter: A Comprehensive Review. Springer; 2023:113-134.

4. Toia B, Pakzad M, Hamid R, Wood D, Greenwell T, Ockrim J. "Diagnosis and Surgical Outcomes of Ectopic Ureters in Adults: A Case Series and Literature Review." Neurourol Urodyn. 2019;38(6):1745-50. doi:10.1002/nau.24054

5. MacDonald GR. "The Ectopic Ureter in Men." J Urol. 1986;135(6):1269-71. doi:10.1016/s0022-5347(17)46068-x

6. Thilagarajah R, Meganathan V, Gleeson M. "Incidental Bilateral Ureteroceles Presenting During Investigation for Hypertension." Urology. 2000;55(6):947-8. doi:10.1016/s0090-4295(00)00482-9

7. Leventis AK, Miles BJ, Gonzales ET Jr, Slawin KM. "Diagnosis and Management of Incidental Ureterocele During the Treatment of Clinically Localized Prostate Cancer." World J Urol. 2000;18(6):444-8. doi:10.1007/s003459900080

8. Byun E, Merguerian PA. "A Meta-Analysis of Surgical Practice Patterns in the Endoscopic Management of Ureteroceles." J Urol. 2006;176(4 Pt 2):1871-7; discussion 1877. doi:10.1016/S0022-5347(06)00601-X

9. Stanasel I, Peters CA. Ectopic Ureter, Ureterocele, and Ureteral Anomalies. In: Partin AW, Dmochowski RR, Kavoussi LR, Peters CA, eds. Campbell-Walsh-Wein Urology. 13th ed. Elsevier; 2025:1059.

10. Abrahamsson K, Hansson E, Sillén U, Hermansson G, Hjälmås K. "Bladder Dysfunction: An Integral Part of the Ectopic Ureterocele Complex." J Urol. 1998;160(4):1468-70. doi:10.1016/s0022-5347(01)62593-x

11. Sander JC, Bilgutay AN, Stanasel I, Koh CJ, Janzen N, Gonzales ET, Roth DR, Seth A. "Outcomes of Endoscopic Incision for the Treatment of Ureterocele in Children at a Single Institution." J Urol. 2015;193(2):662-6. doi:10.1016/j.juro.2014.08.095