Case in Point Ectopic in Adulthood

Angelo R. DeRosalia, MD and Wellman W. Cheung, MD

While this congenital anomaly usually is detected in childhood, it is occasionally overlooked. This case illustrates the importance of thoroughly evaluating the adult with and recurrent urinary tract infections.

ctopic ureter is a congeni- initial exam left ureter; complete duplication of tal anomaly in which one or A 47-year-old woman presented to the right renal collecting system, with more drain into an ori- the urology department of a large both ureters draining into the blad- fice abnormally located within teaching hospital with a one-year his- der; and a large, fibroid uterus (13 E 1 or outside the bladder (Figure 1). It tory of recurrent urinary tract infec- cm x 11 cm x 8 cm). may be associated with a duplicated tions associated with stress urinary The patient underwent cystoscopy, renal collecting system, especially in incontinence. She had been referred which showed bilateral ectopic ure- female patients.1 to urology by her gynecologist, who teral orifices. On the right side, con- Ectopic ureter usually is diagnosed had diagnosed her with a urethral di- sistent with the CT findings, there in childhood, either incidentally or verticulum. were two orifices within the bladder when a patient presents with such During the urologic evaluation, ad- trigone. On the left side, there was symptoms as urinary incontinence ditional questioning revealed that the one ureteral orifice within the trigone or . If routine patient had a six-month history of in- and an opening on the posterior ure- examinations fail to reveal the abnor- termittent, painless, gross hematuria; thra that corresponded to the urethral mal structures and childhood symp- urinary frequency and urgency; noc- diverticulum diagnosed previously by toms are absent or masked, however, turia; and lower abdominal pain. She the gynecologist. A voiding cystoure- the condition may go undetected for stated that her left ureter had been throgram revealed grade II vesicoure- years. tied off 15 years earlier during sur- teral reflux on the left side (Figure 2) In this article, we describe the case gery for left flank pain and frequent and a grade I cystocele. Video urody- of a middle-aged woman who pre- urinary tract infections. No operative namics demonstrated a normal blad- sented with recurrent urinary tract in- reports were available for this proce- der capacity, no instability, and no fections and stress urinary continence dure, which the patient said had been stress, with an abdominal pressure of

that were found to be secondary to performed in Central America. 90 cm H2O. an undiagnosed ectopic ureter and Physical examination revealed pal- Bilateral retrograde pyelography duplicated renal collecting system. pable uterine fibroids. An empty su- confirmed the presence of two right This case illustrates the importance of pine stress test yielded negative results. ureteral orifices and a single left ure- performing a thorough evaluation— Laboratory testing showed a normal teral orifice within the bladder, as including a detailed history, physical serum creatinine level, negative urine well as mild left hydroureteronephro- examination, and appropriate imag- cultures, and normal urine cytology. sis. A large, left, posterior, urethral ing studies—of adult urinary inconti- cyst was identified, which, after con- nence, especially when the patient has Imaging studies trast injection, proved to be a blind- a long history of urinary problems. A renal ultrasound revealed moder- ending stump similar to the refluxing ate left hydroureteronephrosis, and segment visualized on video urody-

Dr. DeRosalia is the chief resident and Dr. a transvaginal ultrasound showed a namic studies (Figure 3). Cheung is an assistant professor, both in the cystic structure arising from the pos- departments of urology and obstetrics and gy- terior . A computed tomogra- treatment Course necology at the State University of New York Downstate Medical Center and the Brooklyn VA phy (CT) scan of the abdomen and Exploratory laparoscopic surgery re- Medical Center, both in Brooklyn. pelvis demonstrated a single, patent vealed that the patient actually had

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four ureters: two on each side, with both right ureters and the lower pole left ureter terminating in the bladder, and the upper pole left ureter (pre- sumably the one that had been tied off previously) terminating at the location of the previously identified urethral cyst. Ureteral stents were Joe Gorman © 2007. placed in both right ureters and the lower pole left ureter, and a Fogarty balloon catheter was placed in the left urethral diverticulum for identifica- tion purposes. Further exploration revealed mild, left, upper pole hydro- nephrosis, with a large, ectopic ure- terocele at the bladder neck. The surgeons immediately per- formed lysis of adhesions, excision of the left upper pole duplicated ure- ter, a supracervical hysterectomy, an abdominal sacrocolpopexy, creation of a pubovaginal sling with autolo- gous fascia, and repair of the urethra with a Martius labial fat-pad graft and vaginal reconstruction. The patient’s incontinence re- solved immediately after the opera- tion. Three weeks later, the patient’s ureteral stents were removed. Re- peat cystoscopy confirmed that the original posterior urethral defect was closed, and uroflow voiding studies with postvoid residual showed good flow with adequate emptying. Figure 1. Ectopic ureter with bilateral duplication of the renal collecting system. ABOUT THE CONDITION Ectopic ureter results from abnor- mal development of the ureteric bud tient, the ectopic ureteral orifices and the intracellular matrix of vaginal tis- from the early in bilateral duplicated renal collecting sue that might have been masking the embryogenesis. Renal dysplasia also system went undiagnosed and un- defect. occurs secondary to inadequate ure- treated for many years despite recur- With this in mind, clinicians eval- teral bud-to-blastema interaction.2 In rent urinary tract infections and other uating incontinence in adult patients female patients, the urethra and the symptoms. should be sure to elicit an adequate vestibule are the most common loca- Notably, it was the late occurrence medical history, particularly with re- tions of ectopic ureteral orifices.3 of urinary incontinence that led to gard to the duration of urinary symp- The classic presentation of ectopic recognition of this patient’s condi- toms. An adult with ectopic ureter ureter is continuous incontinence in a tion. It is possible that this delayed often will have a long history of uri- pediatric, female patient with an oth- symptom may have been triggered by nary symptoms, including inconti- erwise normal voiding pattern after a decrease in estrogen associated with nence, that dates back to childhood. toilet training. In the case of our pa- aging, which could have decreased Additionally, a thorough physical

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toration of continence, preservation of renal and sexual function, correc- tion of reflux, and elimination of in- fection. As this case illustrates, adults with ectopic ureter can be treated suc- cessfully, with resolution of persistent, bothersome symptoms and improve- ment in quality of life. ●

Figure 2. The patient’s voiding cystoure- Author disclosures throgram, showing grade II vesicoureteral The authors report no actual or poten- reflux on the left side. tial conflicts of interest with regard to this article.

Disclaimer The opinions expressed herein are those of the authors and do not necessarily reflect those of Federal Practitioner, Quadrant HealthCom Inc., the U.S. government, or any of its agencies. This article may discuss unlabeled or investigational use of certain drugs. Please review complete prescribing in- formation for specific drugs or drug combinations—including indications, contraindications, warnings, and ad- verse effects—before administering pharmacologic therapy to patients. References 1. Minevich E, Tackett L. and other con- Figure 3. The patient’s left retrograde py- gential ureteral anomalies. eMedicine web site. elogram, showing a large, posterior cyst http://www.emedicine.com/med/topic3196.htm. Updated February 9, 2007. Accessed May 14, 2007. that proved to be a blind-ending stump. 2. Mackie GG, Stephens FD. Duplex kidneys: A cor- relation of renal dysplasia with position of the ure- teral orifice. J Urol. 1975:114(2):274–280. 3. Ellerker AG. The extravesical ectopic ureter. Br J Surg. 1958;45(192):344–353. examination—which includes, in particular, a close inspection of the vaginal wall and perineum—may re- veal a visible ectopic ureteral orifice. If ectopic ureter is associated with a duplicated collecting system, reflux may be noted on voiding cystoure- throgram. Cystoscopy may aid in vi- sualization of ectopic or duplicated ureteral orifices. Once diagnosed, correction of ec- topic ureter in the adult should adhere to same principles as the pediatric population. Goals should include res-

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