Ureteroceles represent abnormal congenital dilatation of the distal-most portion of the ureter. The dilated portion of the ureter may herniate into the bladder secondary to the abnormal structure of vesicoureteric junction (VUJ).
A ureterocele occurs in about 1 in 4000 children and most commonly seen in the Caucasian population9. Females are 4-7 times more commonly affected than male. Bilateral ureteroceles are seen in 10% of cases only10.
Most ureteroceles are congenital, usually associated with ectopic insertion of the ureter with associated duplicated collecting system (see below).
In a minority of cases, ureteroceles are an isolated abnormality, and these are usually seen in adults (see below).
The most accepted theory is failure in regression of the Chwalla membrane which is a membrane between the urogenital sinus and the developing ureteral bud11.
Most cases have a sporadic occurrence.
Location and classification
Ureteroceles are classified by location. The most common system of classification is that of the urologic division of American Academy of Pediatrics 6,7. Both types are the result of cystic ectasia of the subepithelial portion of the ureter as it enters the bladder:
- intravesical: occur at the normal VUJ position
- extravesical: occur ectopically low and medial, near bladder neck/urethra
They pose a challenge for diagnosis and treatment because of the wide variety of anatomical abnormalities that may exist and the non-specific symptoms that patients present with.
Intravesical ureterocele (~25%)
Also known as "simple" or "orthotopic" ureterocele 4,6. Considerably less common than the ectopic variety and is almost always confined to the adult population. There is a congenital prolapse of a dilated distal ureter into the bladder lumen. Where they do occur in children, they usually cause symptoms. Bilateral in about 30% of cases 2.
Extravesical ureterocele (~75%)
Also known as "ectopic" ureterocele 4,6. Almost always associated with a duplicated collecting system and the result of abnormal embryogenesis. There is an abnormality in the early development of the intravesicular ureter, the ipsilateral kidney and its collecting system 1. It is significantly more common than the simple type.
Approximately 80% of cases are unilateral and may cause obstruction to the entire renal tract because of prolapse into the bladder neck causing bladder outlet obstruction. Additionally, ureteroceles may contain calculi.
A cecoureterocele is a subtype of extravesical ureterocele which extends inferiorly to involve the urethra. Rarely, it may herniate into the urethra and present as a perineal mass 4,8.
A ureterocele appears as a cystic structure projecting into the bladder, often near the normal location of the vesicoureteric junction (VUJ). This is ectopic in the majority of cases and therefore not at the expected location of the ureteric orifice. The associated ureter is usually noticeably dilated.
Detectable findings include:
- filling defect in the bladder (cobra head sign) which may be distended, collapsed or even everted
- radiolucent halo effect
- an everted ureterocele will appear like a bladder diverticulum
Voiding cystourethrogram may show:
- a round or oval lucency near the trigone
- there may be effacement of the defect with increasing filling of the bladder
- progression to obstructive cystic renal dysplasia
Treatment and prognosis
The prognosis of ureterocoeles are related to the degree of associated reflux or obstruction. Depending on the size and position of a ureterocele, they may prolapse into the ureter causing complete bladder obstruction.
General imaging differential considerations include:
- 1. Ayers, Elizabeth. Incidental Sonographic Finding of Bilateral Ureteroceles Journal of Diagnostic Medical Sonography. 22 (2): 123. doi:10.1177/8756479306286596 -
- 2. Dähnert W. Radiology review manual 7th Edition. Lippincott Williams & Wilkins. ISBN:1609139437. Read it at Google Books - Find it at Amazon
- 3. Weissleder R, Wittenberg J, Harisinghani MG et-al. Primer of diagnostic imaging. Mosby Inc. (2007) ISBN:0323040683. Read it at Google Books - Find it at Amazon
- 4. Berrocal T, López-pereira P, Arjonilla A et-al. Anomalies of the distal ureter, bladder, and urethra in children: embryologic, radiologic, and pathologic features. Radiographics. 22 (5): 1139-64. Radiographics (full text) - Pubmed citation
- 5. Entezami M, Albig M, Knoll U et-al. Ultrasound Diagnosis of Fetal Anomalies. Thieme. (2003) ISBN:1588902129. Read it at Google Books - Find it at Amazon
- 6. Tekgül et al. EAU Guidelines on European Society for Paediatric Urology. European Association of Urology. 2016. European Association of Urology
- 7. Schultza K, Todab LY. Genetic Basis of Ureterocele. (2016) Current genomics. 17 (1): 62-9. doi:10.2174/1389202916666151014222815 - Pubmed
- 8. Florian L, Stefan P, Mark M. A Prolapsed Cecoureterocele in an Adult Treated Conservatively: Highly Rare, but Existent. (2016) Case reports in urology. 2016: 5049072. doi:10.1155/2016/5049072 - Pubmed
- 9.Adesiyun OM, Oyinloye OI, Akande HJ, Atobatele MO, Adeniyi WA, Abdur-Rahman LO. Bilateral giant orthotopic ureterocele appearing as kissing cobra in a Nigerian child. West Afr J Radiol. 2015;22:42-4.
- 10. Shamsa A, Asadpour AA, Abolbashari M, Hariri MK. Bilateral simple orthotopic ureteroceles with bilateral stones in an adult: A case report and review of literature. Urol J. 2010;7:209-11.
- 11. P.W. Jeffrey, Embryogenesis of ureteral anomalies: a unifying theory, Aust. N.Z. J. Surg 1988;58:631–638.
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