Bilateral orthotopic ureterocele

A ureterocele is a congenital saccular dilatation of the terminal aspect of the ureter, arising from abnormal embryogenesis and affecting the early development of the intravesicular ureter, the ipsilateral kidney, and its collecting system (1).
There is no characteristic clinical picture caused by this condition, but fever, pyuria and recurrent abdominal pain are often present in children (2).
The following are the different types of ureteroceles classified by their association with the renal unit:
* Single-system ureteroceles are those associated with a single kidney, a single collecting system, and a solitary ureter.
* Duplex-system ureteroceles are associated with kidneys that have a completely duplicated collecting system and two ureters.
* Orthotopic ureterocele is the term used for a ureterocele whose orifice is located in a normal anatomic (orthotopic) position within the bladder. The orthotopic ureterocele usually arises from a single renal unit with one collecting system and is more common in adults.
* Ectopic ureterocele refers to those ureteroceles whose orifices are located in an ectopic position, such as the bladder neck or urethra.
In a duplicated system, the ureterocele typically arises from the upper pole moiety and is discovered more frequently in the pediatric population. Ureteroceles occur in approximately 1 in every 4000 children. They are more common in Caucasian individuals, females are affected 4-7 times more often than males, and there is a slight left-sided preponderance. In the adult population, ureteroceles also occur more frequently in females, and 17-35% of ureteroceles occur in an orthotopic location (3).
Approximately 10% of ureteroceles occur bilaterally, and bilateral orthotopic ureterocele is an uncommon finding (4).
Renal and/or bladder ultrasonography should be the initial study for the evaluation of a neonate with fetal hydronephrosis, with or without a suspected ureterocele.
Every postnatal ultrasonographic evaluation of antenatal hydronephrosis should include examination of the bladder. The sonographic finding of a well-defined cystic intravesical mass within the posterior bladder wall suggests ureterocele
Voiding cystourethrography should be performed in all newborns with fetal hydronephrosis. Early imaging prior to complete opacification of the bladder with contrast material may show the ureterocele as a filling defect.
When ureterocele is symptomatic, treatment can generally be accomplished by cystoscopic transurethral incision at the base of the ureterocele. Unroofing of the ureterocele, also accomplished via a transurethral approach, is sometimes necessary to relieve obstruction. A more radical approach may be necessary in cases of ectopic ureterocele associated with non-functioning renal moiety; partial nephroureterectomy may be necessary.