Ureteral Duplication, Ureteral Ectopia, and Ureterocele Clinical Presentation

  • Author: John M Gatti, MD; Chief Editor: Marc Cendron, MD   more...
 
Updated: Aug 11, 2011
 

History

Ureteral duplication and ectopic and nonectopic ureterocele tend to present early in life, even prenatally.

  • Prenatal presentation: Prenatal maternal ultrasonography has led to earlier and increased detection of ureteral abnormalities. Hydronephrosis that is identified prenatally warrants postnatal follow-up imaging.
  • Neonatal presentation: Urinary tract infection in the first few months of life remains the most common presentation. An infected obstructed system may lead to life-threatening urosepsis. Infants may also exhibit failure to thrive or nonspecific GI symptoms.
  • Older childhood presentation
    • Urinary tract infection is a common presentation at any age. Ureteral duplication and ectopic ureters can be associated with both reflux and obstruction, predisposing to urinary tract infection. Hematuria or pain in the abdomen or flank may also occur.
    • Ureteral ectopia in females can present as continuous drip incontinence in cases in which the ureteral orifice is located in the vagina, septum, or the urethra, below the sphincter mechanism. Persistently damp underwear (day and night) is a typical history finding.
    • Girls can present with prolapse of the ureterocele through the urethra, which may lead to bladder outlet obstruction and/or incontinence. This is the most common cause of urethral obstruction in girls.[1] Ureteroceles may be seen upon careful physical examination of the genitalia.
    • Ureteroceles in boys may prolapse and occasionally obstruct the urethra, but this is very rare.
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Physical

  • A hydronephrotic kidney is the most common palpable abdominal mass in the newborn and suggests an obstructed kidney, possibly due to an ectopic ureter or ureterocele.
  • Occasionally, urine that drips from the meatus of an ectopic ureter at the urethrovaginal septum can be detected. Close inspection of the genitalia in a female with urinary retention may reveal a prolapsing ureterocele.
  • The images below illustrate duplicated ectopic ureter to the urethrovaginal septum.Ectopic ureter to urethrovaginal septum. This patiEctopic ureter to urethrovaginal septum. This patient presented with continuous drip incontinence. The blue catheter is positioned in the urethra. The gray catheter is positioned in the orifice of the ectopic ureter. Duplicated ectopic ureter to the urethrovaginal seDuplicated ectopic ureter to the urethrovaginal septum. Retrograde injection of contrast into the orthotopic lower pole (white arrow) ureteral orifice and ectopic upper pole (black arrow) orifice simultaneously opacifies both systems.
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Contributor Information and Disclosures
Author

John M Gatti, MD  Associate Professor and Director of Minimally Invasive Urology, Department of Pediatric Surgery and Urology, Children's Mercy Hospital; Assistant Professor, Department of Pediatric Surgery and Urology, University of Missouri School of Medicine at Kansas City, Missouri; Assistant Clinical Professor, Division of Pediatric Urology, University of Kansas School of Medicine at Kansas City, Kansas

John M Gatti, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Urological Association, Society for Fetal Urology, and Society for Pediatric Urology

Disclosure: Nothing to disclose.

Coauthor(s)

J Patrick Murphy, MD  Professor of Surgery, University of Missouri at Kansas City School of Medicine; Section Chief of Urological Surgery, Children's Mercy Hospital

J Patrick Murphy, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Urological Association, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey F Williams, MD  Consulting Staff, Private Practice Urologist

Jeffrey F Williams, MD is a member of the following medical societies: American Urological Association

Disclosure: Nothing to disclose.

Harry P Koo, MD  Chairman of Urology Division and Director of Pediatric Urology, Professor of Surgery, Virginia Commonwealth University School of Medicine, Medical College of Virginia; Director of Urology, Children's Hospital of Richmond

Harry P Koo, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, and American Urological Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Bartley G Cilento, Jr, MD  Instructor, Department of Surgery, Division of Urology, Children's Hospital of Boston and Harvard Medical School

Bartley G Cilento, Jr, MD is a member of the following medical societies: American Academy of Pediatrics, American Urological Association, and Massachusetts Medical Society

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Martin David Bomalaski, MD, FAAP  Pediatric Urologist, Alpine Urology

Martin David Bomalaski, MD, FAAP is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, and American Urological Association

Disclosure: Nothing to disclose.

Daniel Rauch, MD, FAAP  Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine

Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine

Disclosure: Baxter Honoraria Consulting

Chief Editor

Marc Cendron, MD  Associate Professor of Surgery, Harvard School of Medicine; Consulting Staff, Department of Urological Surgery, Children's Hospital Boston

Marc Cendron, MD is a member of the following medical societies: American Academy of Pediatrics, American Urological Association, European Society for Paediatric Urology, Johns Hopkins Medical and Surgical Association, New Hampshire Medical Society, Society for Fetal Urology, and Society for Pediatric Urology

Disclosure: Nothing to disclose.

References

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Ectopic ureter. Cystoscopic view of an ectopic ureter entering the bladder neck.
Single-system ectopic ureter. A retrograde pyelogram of the ectopic ureter opacifies the dilated system. The ureter is obstructed when the sphincter is closed but opens to drain when the sphincter opens during voiding.
Ectopic ureter to urethrovaginal septum. This patient presented with continuous drip incontinence. The blue catheter is positioned in the urethra. The gray catheter is positioned in the orifice of the ectopic ureter.
Duplicated ectopic ureter to the urethrovaginal septum. Retrograde injection of contrast into the orthotopic lower pole (white arrow) ureteral orifice and ectopic upper pole (black arrow) orifice simultaneously opacifies both systems.
A large ureterocele is seen as a filling defect on the early filling images of this cystogram.
Bilateral single-system ureteroceles. The collecting systems and their associated ureteroceles are opacified on intravenous pyelography (IVP). Multiple stones in the ureteroceles may be discerned within the ureteroceles (white arrows) as filling defects.
Bilateral ureteroceles with stones. This ultrasonogram at the bladder level depicts thin-walled, bilateral ureteroceles. Echogenic stone material can be seen in the left ureterocele.
Left duplicated kidney with upper pole ureterocele. This renal scan shows the typical findings of an upper pole duplicated system subtended by a ureterocele. The left upper pole (black arrow) shows minimal uptake when compared with the left lower pole or right kidney.
Renal duplication. Marked upper pole hydronephrosis with minimal dilation of lower pole, indicative of a duplicated collecting system.
Reflux into lower pole: A voiding cystourethrography (VCUG) that demonstrates reflux into the lower pole ureter with classic "drooping lily" configuration.
Female infant with acute pyelonephritis. The ultrasonography findings are notable for left hydronephrosis.
 
 
 
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