Ureteral Duplication, Ureteral Ectopia, and Ureterocele Workup

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

Laboratory Studies

  • Rarely, bilateral renal dysplasia or significant renal scarring may be present, resulting in impaired renal function. In this scenario, renal function and electrolyte levels must be monitored. These patients must also be assessed for hypertension.
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Imaging Studies

  • Ultrasonography
    • Prenatal fetal ultrasound is often the initial study suggesting the diagnosis based on hydroureteronephrosis. The natural history of prenatally diagnosed duplication anomalies is that approximately half of these infants develop urinary tract infection, and about 25% require surgical intervention in the first few years of life.[2]
    • Postnatal abdominal ultrasonography is the best initial screening study.
    • Renal measurements in duplicated collecting systems are often longer than those of a contralateral nonduplicated system. See the image below. Renal duplication. Marked upper pole hydronephrosiRenal duplication. Marked upper pole hydronephrosis with minimal dilation of lower pole, indicative of a duplicated collecting system.
    • Disparate hydronephrosis in the upper and lower pole of a kidney suggests ureteral duplication, especially with upper pole dilation associated with an obstructed or ectopic ureter or with a ureterocele.
    • A ureterocele can usually be seen at the bladder level and appears as a well-defined cystic intravesical mass that can be proximally followed into a dilated ureter. If the bladder is decompressed, visualization of a ureterocele is more difficult.
    • Ultrasonography provides imaging for gross evaluation of the renal parenchyma. Increased echogenicity and renal cysts are sonographic signs that suggest renal dysplasia.
    • The image below illustrates bilateral single-system ureteroceles.Bilateral ureteroceles with stones. This ultrasonoBilateral ureteroceles with stones. This ultrasonogram at the bladder level depicts thin-walled, bilateral ureteroceles. Echogenic stone material can be seen in the left ureterocele.
  • Intravenous urography
    • Intravenous pyelography (IVP) is not commonly used. IVP generally reveals duplicated collecting systems and their level of confluence. In young girls with persistent incontinence, IVP may be helpful in defining ureteral anatomy and the level at which an ectopic ureter may be found.
    • Typically, a ureterocele has been described as having a "cobra head" or " spring onion" configuration at the bladder level.
    • Stones that collect in the ureterocele, as shown below, may be visible on the scout film.Bilateral single-system ureteroceles. The collectiBilateral 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.
    • Because duplicated systems generally have poorly functioning renal moieties associated with ectopia or ureteroceles, these distinct images are usually observed with single-system intravesical ureteroceles. Delayed images are helpful in identifying poorly functioning renal units.
  • Voiding cystourethrography
    • Duplicated collecting systems with lower pole reflux can be visualized using voiding cystourethrography (VCUG). The configuration of the kidney lacks opacification of the nonrefluxing upper pole, giving it the appearance of a "drooping lily." See the image below. Reflux into lower pole: A voiding cystourethrograpReflux into lower pole: A voiding cystourethrography (VCUG) that demonstrates reflux into the lower pole ureter with classic "drooping lily" configuration.
    • Ectopic ureters generally do not reflux unless they are ectopic to the bladder neck. In this case, the refluxing unit opacifies only during voiding, when the bladder neck is open. Occasionally, the radiologist may inadvertently pass a catheter transurethrally up the ectopic ureter. The initial films then opacify only that collecting system and not the bladder.
    • Ureteroceles are best imaged at initial filling and appear as a filling defect in the bladder base (see the image below). Identifying which side large ureteroceles are associated with can be difficult. Reflux of the ipsilateral lower pole is observed in approximately 50% of cases. Contralateral reflux may be observed in 25% of cases, and reflux into the ureterocele may be observed in 10% of cases. A large ureterocele is seen as a filling defect onA large ureterocele is seen as a filling defect on the early filling images of this cystogram.
  • Renal scintigraphy
    • Mercaptotriglycylglycine (MAG-3) renal scintigraphy is the most commonly obtained renal scan used to evaluate relative renal function and drainage. It provides information on segmental renal function, allowing comparison of the upper pole moiety to the lower. It may aid in the determination of salvageability and selection of operative technique. MAG-3 Lasix renography may also quantitate the degree of obstruction in moieties with preserved function.
    • Technetium-99m (Tc99m) diethylenetriaminepentaacetic acid (DTPA) is similar to MAG-3 but is less efficacious in patients who have kidneys with poor function and in newborns because of their relatively impaired glomerular filtration rates. Radiation exposure is fairly high with this compound.
    • Tc99m dimercaptosuccinic acid (DMSA) is an agent that is taken up by the renal tubular cells and is used for renal scintigraphy.[3] DMSA resting can be used reliably to assess renal function but is not useful in evaluating urinary drainage of the upper urinary tract because it is secreted slowly into the urine.
    • The image below illustrates pical findings of an upper pole duplicated system subtended by a ureterocele.Left duplicated kidney with upper pole ureteroceleLeft 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.
  • Axial Imaging
    • Axial imaging using CT scanning or MRI is rarely the initial study of choice.
    • Gadolinium-enhanced MRI may be valuable in opacifying an ectopic ureter that causes incontinence in a female, whereas other studies may not be diagnostic.
    • In one study, contrast CT scanning was the most reliable study in demonstrating the poorly functioning upper pole renal moiety and the associated ectopic ureter causing continuous drip incontinence.[4]
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Procedures

  • Although sometimes used as an investigative modality, cystoscopy is usually performed during surgical intervention to verify the diagnosis. See the image below. Ectopic ureter. Cystoscopic view of an ectopic ureEctopic ureter. Cystoscopic view of an ectopic ureter entering the bladder neck.
  • The search for the orifice of an ectopic ureter may be extremely difficult. Tedious probing of any small dimple along the urethra or anterior vaginal wall with a whistle-tip catheter may be rewarded with visual confirmation of the offending moiety upon the retrograde injection of contrast. The use of indigo carmine or methylene blue injected intravenously may help in locating an ectopic ureteral orifice at the time of cystoscopy.
  • Findings with large or ectopic ureteroceles, which can distort and obscure the entire field of vision, can be confusing. Finding the ureterocele orifice may be challenging.
  • Small ureteroceles may not be evident, especially when the bladder is distended, which causes the ureterocele to flatten or evert. When associated with duplication, large ureteroceles may obscure visualization of any ipsilateral ureteral orifice.
  • Retrograde pyelography can define the ureteral anatomy, and puncture of the ureterocele with instillation of contrast may aid in defining the origin of the lesion.
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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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