Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Megaureter and Other Congenital Ureteral Anomalies

  • Author: Christopher S Cooper, MD, FACS, FAAP; Chief Editor: Edward David Kim, MD, FACS  more...
 
Updated: Apr 08, 2015
 

Background

Developmental abnormalities of the ureter encompass a wide range of disorders. Ureteral anomalies are a common cause of morbidity in children and frequently require surgical intervention.

Next

History Of The Procedure

Interestingly, Leonardo da Vinci and Galen were the first to begin to recognize the importance of the ureterovesical junction (UVJ) and to identify vesicoureteral reflux (VUR). Semblinow's 1883 animal experiments renewed enthusiasm for the study of reflux and began the modern era of research to clarify the anatomy, function, and pathophysiology of UVJ anomalies.

Previous
Next

Problem

Congenital ureteral anomalies include the following:

  • Duplex (duplicated) ureters
  • Ectopic ureter
  • Megaureter
  • Ureterocele
  • Vesicoureteral reflux (VUR)<>

Duplex (duplicated) system

The duplex system is a kidney with two ureters. A patient with a duplication anomaly may have bifid ureters (partial or incomplete duplication) or two ureters that empty separately into the bladder (complete duplication). The upper ureter is more likely to be associated with ectopic insertion, ureterocele, and/or obstruction, whereas the lower ureter is more frequently associated with VUR.

The upper pole is one of the components of the duplex kidney. The upper pole ureter drains the upper pole of a duplex kidney. Similarly, the lower pole of the kidney is drained by the lower-pole ureter.

Caudal or medial ectopia describes the ureteral orifice when located at the proximal lip of the bladder neck or more distal.

Ectopic ureter

Ectopic ureter occurs when the ureter drains to an abnormally located (ectopic) orifice.

Bilateral single-system ureteral ectopia is rare and usually coexists with a multitude of other urinary tract abnormalities (eg, VUR, renal dysplasia, rudimentary bladder development).

Megaureter

A megaureter is a wide ureter, greater than 7 mm in diameter.

Megaureters may be classified into the following four categories:

  • Obstructed
  • Refluxing
  • Obstructed and refluxing
  • Nonobstructed/nonrefluxing

Ureterocele

A ureterocele is a congenital saccular dilatation of the distal segment of the ureter.

This anomaly most frequently involves the upper pole of a duplex system. If the ureterocele extends beyond the bladder into the urethra, it is considered an ectopic ureterocele. A ureterocele contained within the bladder is considered an orthotopic ureterocele.

Ureteroceles are often associated with obstruction and VUR.

Vesicoureteral reflux

VUR is retrograde passage of urine from the bladder into the ureter and/or kidney. Reflux-induced renal injury is usually caused by the association of VUR with urinary tract infection (UTI).

Reflux-induced renal injury was previously thought to occur primarily in children younger than 2 years. However, the risk of renal injury from pyelonephritis associated with VUR may occur in individuals well beyond this age.

Reflux may also be associated with regions of renal dysplasia or hypoplasia in the absence of UTI and is thought to be secondary to abnormal development.

Reflux that is secondary to high bladder pressures such as those occurring in patients with posterior urethral valves (PUV) or bladder outlet obstruction (BOO) is frequently associated with renal injury.

Reflux-induced renal injury may range from clinically silent focal scars to generalized scarring and renal atrophy (reflux nephropathy), which may lead to morbidity during pregnancy, renin-mediated hypertension, renal insufficiency, and even end-stage renal disease.

Previous
Next

Epidemiology

Frequency

Ureteral duplication

  • Ureteral duplication is the most common anomaly of the urinary tract.
  • Incidence is as high as 8% in children being evaluated for UTI.
  • Incomplete ureteral duplication is observed in approximately 1 in 25 individuals.
  • Complete duplication is present in approximately 1 in 125 individuals.
  • Complete duplication on one side results in a 40% chance of a complete duplication abnormality on the other side.
  • Approximately 10% of siblings may also be affected by complete duplication.

Ectopic ureters

  • Ectopic ureters occur in 0.025% of the population.
  • Approximately 10% are bilateral.
  • Ectopic ureters occur more frequently in females than in males (by a ratio of 6:1).
  • In females, more than 80% of ectopic ureters drain duplicated systems. In males, most ectopic ureters drain a single system.
  • Approximately 80% of all ectopic ureters drain the upper pole of a duplex kidney.
  • In males, the ureters always terminate proximal to the external sphincter and may be found at the bladder neck/prostatic urethra (48%), seminal vesicle (40%), ejaculatory duct (8%), vas deferens (3%), or epididymis (0.5%).
  • In females, the ureters may terminate at the bladder neck/urethra (35%), vestibule (30%), vagina (25%), or uterus (5%).

Megaureters

  • Bilateral involvement is present in about 20% of patients with primary obstructed megaureters.
  • Primary obstructed megaureter has a male-to-female ratio of nearly 4:1.
  • The left side is more often affected than the right.

Ureterocele

  • Incidence is approximately 1 in 4000 children.
  • Approximately 80% occur in females.
  • Approximately 10% are bilateral.
  • Approximately 80% are associated with duplicated collecting systems with single system ureteroceles being more common in males and adults.

Vesicoureteral reflux

  • Incidence in otherwise healthy children is approximately 1%.
  • The incidence is approximately 40% in patients undergoing evaluation for UTI.
  • The reported risk of reflux in a sibling is 27-43%.
  • Approximately 50% of the offspring of women with reflux also have VUR.
Previous
Next

Etiology

A ureteral bud, the early precursor of the ureter, branches off from the caudal portion of the wolffian (mesonephric) duct between the fourth and sixth week of gestation. The cranial portion of the ureteral bud joins with the metanephric blastema and begins to induce nephron formation. The bud subsequently branches into the renal pelvis and the calyces and induces nephron formation. Caudally, the mesonephric duct (along with the ureteral bud) is incorporated into the cloaca as it forms the bladder trigone. Alterations in bud number, position, or time of development result in ureteral anomalies.

Early branching of a single ureteral bud results in incomplete (partial) duplication, with a single ureteral orifice and bifid proximal ureters. An accessory ureteral bud creates complete duplication, with the upper ureter usually protruding into the bladder more medially and inferiorly than the lower ureter. Ectopic termination of a single system or of the ureter of a duplex system is the result of the high (cranial) origin of the ureteral bud from the mesonephric duct. Because of the delayed incorporation of the ureteral bud into the bladder, the resulting position of the ureteral orifice is more caudal and medial or in more severe cases it inserts into one of the Wolffian duct structures as noted above.

The function of the ureter is to effectively transport the urinary bolus from the minor calyces to the urinary bladder at acceptably low pressures. The efficiency of this task depends on adequate coaptation of the ureteral wall to propel the urinary bolus. If the ureter fails to propagate the peristaltic wave, the static urine distends the upper urinary tract and reduces luminal coaptation. Other factors that may affect ureteral transport include urinary volume and bladder pressure.

Previous
Next

Pathophysiology

Megaureters

The term megaureter refers to an enlarged ureter. The 4 categories of megaureters are refluxing, obstructing, refluxing/obstructing, and nonrefluxing/nonobstructing. Each category is further divided into primary or secondary, based on either intrinsic or extrinsic causes for their appearance.

  • Primary obstructed megaureter is most commonly caused by an adynamic juxtavesical segment of the ureter that fails to effectively propagate urine flow.
  • Secondary obstructed megaureter occurs usually when ureteral dilatation is the result of a functional ureteral obstruction associated with elevated bladder pressures secondary to PUV or an NGB that impedes ureteral emptying.
  • Primary refluxing megaureter is associated with severe VUR that alters ureteral efficiency by ureteral distention. The megaureter-megacystis syndrome is an extreme form of the primary refluxing megaureters in which massive reflux prevents effective bladder emptying because urine is passed back and forth between the ureters and bladder.
  • Secondary refluxing megaureter occurs secondary to PUV or NGB when elevated bladder pressures cause decompensation of the UVJ.
  • Primary nonrefluxing/nonobstructed megaureter is diagnosed when no evidence of obstruction or reflux can be demonstrated (diagnosis of exclusion).
  • Secondary nonrefluxing/nonobstructed megaureter occurs secondary to diabetes insipidus, in which high urinary flow rates may overwhelm the maximum transport capacity of the ureter by peristalsis or as the result of ureteral atony accompanying a gram-negative UTI.
  • Primary refluxing obstructed megaureter occurs in the presence of an incompetent VUJ that allows reflux through an adynamic distal segment.

Vesicoureteral reflux

A sufficient tunnel length of the submucosal ureter is the most important component of a competent UVJ, which provides a predominantly passive valve mechanism for ureteral compression and prevents retrograde urine passage. Factors that affect marginal tunnel pressure, causing either primary or secondary reflux, include the loss of UVJ compliance (during UTI), structural weaknesses in the detrusor floor (bladder diverticulum, ureterocele), or excessively high intravesical pressure resulting from neurovesical dysfunction or BOO. Reflux resulting from a congenitally deficient UVJ is referred to as primary reflux; reflux resulting from a BOO or NGB is referred to as secondary reflux.

Previous
Next

Presentation

No specific clinical signs are associated with ureteral anomalies. Most children present with an abnormal finding on routine prenatal ultrasound before associated symptoms or infection develops. Some patients present with UTI, abdominal mass or hematuria. Children with primary megaureters may also present with cyclic abdominal pain/flank pain, or, less commonly, in acute pain crisis.[1] Patients may present with a cystic mass at the urethral meatus representing a prolapsed ureterocele. In other patients, the diagnosis is incidental after imaging studies for unrelated symptomatology.

Ureteral anomalies may be discovered during the evaluation of a patient with hypertension, proteinuria, or even renal insufficiency (in rare cases of severe bilateral anomalies).

Approximately 50% of females with ectopic ureters present with constant urinary incontinence or vaginal discharge. Consideration of an ectopic ureteral insertion should be given in prepubertal boys with recurrent epididymitis. Postpubertal males with ectopic ureters most commonly present with complaints of chronic prostatitis and painful intercourse and ejaculation. In males, incontinence is never due to an ectopic ureter because the ectopic ureter never inserts distal to the external urethral sphincter.

Previous
Next

Indications

Megaureter

See the list below:

  • Increasing hydroureteronephrosis
  • Decrease in renal function of involved kidney
  • Development of UTI or recurrent pain

Ureterocele

See the list below:

  • Obstruction
  • Urosepsis or compromised renal function may necessitate urgent decompression prior to definitive surgical reconstruction.

Vesicoureteral reflux (absolute indications)

See the list below:

  • Progressive renal injury
  • Documented failure of renal growth
  • Breakthrough pyelonephritis
  • Intolerance or noncompliance with antibiotic suppression
  • Parental preference

Vesicoureteral reflux (relative indications)

See the list below:

  • Pubertal age
  • High-grade (IV or V) VUR
  • Failure to resolve
Previous
Next

Relevant Anatomy

Ureters are paired muscular tubes that run from the renal pelvis to the bladder and travel through retroperitoneal connective tissue in a serpentine fashion. The ureters run through 3 natural areas of narrowing: the ureteropelvic junction, the crossing of the iliac vessels, and the UVJ. From the renal pelvis to the iliac vessels, the ureter is referred to as the abdominal ureter. From the iliac vessels to the bladder, the ureter is called the pelvic ureter.

The UVJ may be divided into 3 sections: the terminal portion (juxtavesical ureter), the intramural portion, and the submucosal portion (under the bladder mucosa).

Previous
Next

Contraindications

The presence of an acute UTI, especially with bullous edema of the bladder mucosa, may be a contraindication to definitive reconstruction. Urinary diversion (eg, ureterostomy, vesicostomy) or drainage may be necessary.

Previous
 
 
Contributor Information and Disclosures
Author

Christopher S Cooper, MD, FACS, FAAP Professor with Tenure and Vice Chair, Department of Urology, Professor, Department of Pediatrics, Associate Dean for Student Affairs and Curriculum, Children's Hospital of Iowa and University of Iowa, Roy J and Lucille A Carver College of Medicine

Christopher S Cooper, MD, FACS, FAAP is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Medical Association, Phi Beta Kappa, Society for Pediatric Urology, Society for Fetal Urology, International Children's Continence Society, American College of Surgeons, American Urological Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Mark Jeffrey Noble, MD Consulting Staff, Urologic Institute, Cleveland Clinic Foundation

Mark Jeffrey Noble, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, American Urological Association, Kansas Medical Society, Sigma Xi, Society of University Urologists, SWOG

Disclosure: Nothing to disclose.

Chief Editor

Edward David Kim, MD, FACS Professor of Surgery, Division of Urology, University of Tennessee Graduate School of Medicine; Consulting Staff, University of Tennessee Medical Center

Edward David Kim, MD, FACS is a member of the following medical societies: American College of Surgeons, Tennessee Medical Association, Sexual Medicine Society of North America, American Society for Reproductive Medicine, American Society of Andrology, American Urological Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Repros.

Acknowledgements

Angela M Arlen, MD Chief Resident, Department of Urology, University of Iowa, Roy J and Lucille A Carver College of Medicine

Angela M Arlen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Urological Association, and Society of Women in Urology

Disclosure: Nothing to disclose.

Leslie Tackett McQuiston, MD, FAAP Assistant Professor of Surgery (Urology) Dartmouth Medical School; Staff Pediatric Urologist, Dartmouth-Hitchcock Hospital

Disclosure: Nothing to disclose.

Eugene Minevich, MD Assistant Professor, Department of Surgery, Division of Pediatric Urology, University of Cincinnati College of Medicine

Disclosure: Nothing to disclose.

References
  1. Anderson CB, Tanaka ST, Pope JC 4th, Adams MC, Brock JW 3rd, Thomas JC. Acute pain crisis as a presentation of primary megaureter in children. J Pediatr Urol. 2011 Jun 21. [Medline].

  2. Renjen P, Bellah R, Hellinger JC, Darge K. Pediatric urologic advanced imaging: techniques and applications. Urol Clin North Am. 2010 May. 37(2):307-18. [Medline].

  3. [Guideline] Peters CA, Skoog SJ, Arant BS Jr, Copp HL, Elder JS, Hudson RG. Summary of the AUA Guideline on Management of Primary Vesicoureteral Reflux in Children. J Urol. 2010 Sep. 184(3):1134-44. [Medline].

  4. Hendren WH. Operative repair of megaureter in children. J Urol. 1969 Apr. 101(4):491-507. [Medline].

  5. Fretz PC, Austin JC, Cooper CS, Hawtrey CE. Long-term outcome analysis of Starr plication for primary obstructive megaureters. J Urol. 2004 Aug. 172(2):703-5. [Medline].

  6. Cooper CS. Diagnosis and management of vesicoureteral reflux in children. Nat Rev Urol. 2009 Sep. 6(9):481-9. [Medline].

  7. Marchini GS, Hong YK, Minnillo BJ, Diamond DA, Houck CS, Meier PM. Robotic assisted laparoscopic ureteral reimplantation in children: case matched comparative study with open surgical approach. J Urol. 2011 May. 185(5):1870-5. [Medline].

  8. Gimpel C, Masioniene L, Djakovic N, Schenk JP, Haberkorn U, Tönshoff B. Complications and long-term outcome of primary obstructive megaureter in childhood. Pediatr Nephrol. 2010 Sep. 25(9):1679-86. [Medline].

  9. Atala A, Keating MA. Vesicoureteral Reflux and Megaureter. Walsh PC, Retik AB, Vaughan ED, Wein AJ, eds. Campbell's Urology. 7th ed. Philadelphia, Pa: WB Saunders Co; 1998. 1859.

  10. Baskin LS, Zderic SA, Snyder HM, Duckett JW. Primary dilated megaureter: long-term followup. J Urol. 1994 Aug. 152(2 Pt 2):618-21. [Medline].

  11. Churchill BM, Abara EO, McLorie GA. Ureteral duplication, ectopy and ureteroceles. Pediatr Clin North Am. 1987 Oct. 34(5):1273-89. [Medline].

  12. Cooper CS, Passerini-Glazel G, Hutcheson JC, et al. Long-term followup of endoscopic incision of ureteroceles: intravesical versus extravesical. J Urol. 2000 Sep. 164(3 Pt 2):1097-9; discussion 1099-100. [Medline].

  13. DeFoor W, Minevich E, Reddy P, Polsky E, McGregor A, Wacksman J. Results of tapered ureteral reimplantation for primary megaureter: extravesical versus intravesical approach. J Urol. 2004 Oct. 172(4 Pt 2):1640-3; discussion 1643. [Medline].

  14. [Guideline] Elder JS, Peters CA, Arant BS Jr, Ewalt DH, Hawtrey CE, Hurwitz RS, et al. Pediatric Vesicoureteral Reflux Guidelines Panel summary report on the management of primary vesicoureteral reflux in children. J Urol. 1997 May. 157(5):1846-51. [Medline].

  15. Elder JS, Snyder HM, Peters C, et al. Variations in practice among urologists and nephrologists treating children with vesicoureteral reflux. J Urol. 1992 Aug. 148(2 Pt 2):714-7. [Medline].

  16. Glassberg KI, Braren V, Duckett JW, et al. Suggested terminology for duplex systems, ectopic ureters and ureteroceles. J Urol. 1984 Dec. 132(6):1153-4. [Medline].

  17. Husmann DA. Ureteral ectopy, ureteroceles, and other anomalies of the distal ureter. Gonzales ET, Bauer SB, eds. Pediatric Urology Practice. Philadelphia, Pa: Lippincott Williams & Wilkins; 1999. 295.

  18. Keating MA, Escala J, Snyder HM 3rd, et al. Changing concepts in management of primary obstructive megaureter. J Urol. 1989 Aug. 142(2 Pt 2):636-40; discussion 667-8. [Medline].

  19. Koff SA, Wagner TT, Jayanthi VR. The relationship among dysfunctional elimination syndromes, primary vesicoureteral reflux and urinary tract infections in children. J Urol. 1998 Sep. 160(3 Pt 2):1019-22. [Medline].

  20. Lebowitz RL. The detection and characterization of vesicoureteral reflux in the child. J Urol. 1992 Nov. 148(5 Pt 2):1640-2. [Medline].

  21. Lee SD, Akbal C, Kaefer M. Refluxing ureteral reimplant as temporary treatment of obstructive megaureter in neonate and infant. J Urol. 2005 Apr. 173(4):1357-60; discussion 1360. [Medline].

  22. Mackie GG, Stephens FD. Duplex kidneys: a correlation of renal dysplasia with position of the ureteral orifice. J Urol. 1975 Aug. 114(2):274-80. [Medline].

  23. Massad C, Smith E. Megaureter. Gonzales ET, Bauer SB, eds. Pediatric Urology Practice. Philadelphia, Pa: Lippincott Williams & Wilkins; 1999. 205.

  24. Noe HN. The current status of screening for vesicoureteral reflux. Pediatr Nephrol. 1995 Oct. 9(5):638-41. [Medline].

  25. Shapiro E. The Ectopic Ureter. AUA Update Series. 1990. 31:

  26. Shokeir AA, Nijman RJ. Primary megaureter: current trends in diagnosis and treatment. BJU Int. 2000 Nov. 86(7):861-8. [Medline].

  27. Shukla AR, Cooper J, Patel RP, et al. Prenatally detected primary megaureter: a role for extended followup. J Urol. 2005 Apr. 173(4):1353-6. [Medline].

  28. Wacksman J, Gilbert A, Sheldon CA. Results of the renewed extravesical reimplant for surgical correction of vesicoureteral reflux. J Urol. 1992 Aug. 148(2 Pt 1):359-61. [Medline].

 
Previous
Next
 
Intravenous urogram demonstrating left primary megaureter in comparison to normal right collecting system.
Ultrasound image of a normal right kidney in a child with a febrile urinary tract infection. Image courtesy of Steven Kraus, MD, Cincinnati, Ohio.
Ultrasound image of the same patient (in Picture 2), demonstrating that the left kidney has a duplex collecting system. Note the lower-pole hydronephrosis. Image courtesy of Steven Kraus, MD, Cincinnati, Ohio.
Voiding cystourethrogram from the same patient (in Picture 2), demonstrating right vesicoureteral reflux into a single system and left vesicoureteral system into the lower pole of a duplicated system. Note the deficiency of upper-pole calyces on the left side and the "drooping lily" appearance of the left lower-pole system, which suggest the duplication anomaly in this case. Image courtesy of Steven Kraus, MD, Cincinnati, Ohio.
Voiding cystourethrogram that illustrates a right ureterocele characterized by the round filling defect at the right bladder base. Image courtesy of Steven Kraus, MD, Cincinnati, Ohio.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.