Ureteral Duplication, Ureteral Ectopia, and Ureterocele Treatment & Management

Antibiotic suppression is usually warranted in newborns with hydronephrosis or in patients who present with urinary tract infection (UTI) until the diagnosis is made and reflux is ruled out. For vesicoureteral reflux (VUR), antibiotics are generally continued until the reflux spontaneously resolves or is surgically treated or until the patient is toilet-trained and has a considerable infection-free interval. Antibiotic prophylaxis is often continued in patients with obstructed systems and in infants with dilated nonobstructed systems.

Early consultation, even antenatally, with a pediatric urologist is suggested in all cases.

Various surgical options are available to treat ureteral duplication, especially with ureteral ectopia or ureterocele. In a survey of urologists, little consistency was found in the management strategy for ureteroceles, emphasizing that care must be individualized. ^[7]

There has been a trend toward managing ureteroceles more conservatively with expectant or minimally invasive therapies rather than with major reconstructive efforts. ^{[8, 9]}  The decision to treat ureteral duplication surgically requires the consideration of multiple elements (see below).

Factors affecting decision for surgical treatment

Age of patient

Duplicated collecting systems with reflux are expectantly managed with antibiotic suppression until the reflux spontaneously resolves or until the child is older (6-12 months), at which time surgery may be more easily accomplished.

In infants, the small bladder can limit surgical reconstruction with regard to creating a ureteral tunnel that is long enough to prevent recurrent reflux. Ureteral reimplants in small infants with breakthrough infections are possible but can be difficult and are associated with increased complications and failures.

In infants with duplicated systems and a well-functioning but obstructed upper-pole moiety or an obstructed ectopic single-system ureter, urinary diversion may be the treatment of choice until the bladder is bigger and a ureteral reimplant with or without ureteral tailoring is more feasible.

A cutaneous ureterostomy allows decompression of the system and may obviate the need for tailoring of the ureter at subsequent reimplant. However, it does commit the child to a second operation.

If the system is duplicated and no reflux is present in the lower-pole system, a ureteroureterostomy is an attractive approach because it is a single-stage operation with relatively low risk.

Even in infancy, small instrumentation allows ureteroceles that are associated with good function to be endoscopically decompressed with incision. However, this carries the risk of subsequent reflux into that moiety. Incising a ureterocele that is associated with poor function provides little gain. The exception is an infant with urosepsis, but this may be best treated with percutaneous drainage and subsequent reconstruction, depending on the stability of the patient.

Amount of functioning parenchyma

The upper-pole system that serves a duplicated ureterocele typically makes up less than 30% of the unilateral renal function, and preservation of this function is usually not critical. If this poorly functioning moiety is not associated with reflux in other moieties, the best approach is often removal. Conversely, a poorly functioning renal unit that serves a decompressed ureterocele with no reflux has little or no indication for removal.

Intravesical vs extravesical ureterocele

More than 90% of intravesical ureteroceles can be decompressed with endoscopic incision without the need for subsequent surgery for reflux. In endoscopic incision of extravesical ureteroceles, 50% of the cases require secondary surgery. ^[10]

Detrusor backing

A poorly supported ureterocele that everts during voiding and becomes a bladder diverticulum may be more likely to require secondary reconstruction of the trigone than one that is well supported.

Degree of ureteral dilation

If the ureter that is associated with ectopia or the ureterocele is massively dilated, attempts at reimplantation may be associated with a higher complication rate, such as obstruction and persistent reflux.

Vesicoureteral reflux

Associated VUR may be the single most important predictor of the need for open surgery. Reflux is the major factor that leads to the need for subsequent surgery after upper-pole partial nephrectomy to decompress a ureterocele. If high-grade reflux is associated with a ureterocele, primary endoscopic incision decompresses the ureterocele and facilitates subsequent bladder-level surgery. If no reflux occurs, a simplified approach that consists of only an upper-pole partial nephrectomy may be indicated.

Number of renal moieties involved

Each ureter subtends a separate renal moiety. If only one moiety is involved and is poorly functioning, a single-stage nephrectomy or heminephrectomy is usually curative. The likelihood that this upper-tract approach will be curative diminishes as the number of other moieties involved with either reflux or obstruction increases. In this case, a lower-tract approach in which all problematic ureters can be simultaneously treated is a better option.

Surgical approach

Endoscopic decompression

For intravesical ureteroceles associated with good renal function and associated with either a single or a duplex renal unit, a primary endoscopic approach may be used. A small incision low on the ureterocele is made, creating a flap valve to avoid reflux.

Some series have reported that as many as 90% of patients are adequately treated with endoscopic incision alone. ^[11] Single-system orthotopic ureteroceles appear to respond most definitively with this approach. A duplex renal system, ectopic ureterocele location, or preoperative reflux suggests trigonal anatomic distortion, increasing the likelihood of a secondary operation after puncture. ^[12]  

A study by Haddad et al found that the "watering can" endoscopic ureteral puncture approach, in which a laser fiber was used to make 10-20 punctures in the ureterocele, yielded durable successful results at a median follow-up of 2.8 years. ^[13]

Older age, female sex, and complete ureteral duplication have been associated with a better outcome after dextranomer–hyaluronic acid (DHA) injection. ^[14] At present, however, DHA is contraindicated by its manufacturer for use in the duplicated collecting system.

Some authors have suggested cystoscopic drainage of an upper-pole obstructed segment in the absence of ureterocele with either stenting or creation of a new orifice into the bladder using a holmium laser to bypass the obstruction as a definitive procedure. ^[15]

Open reconstruction at bladder level (lower-tract approach)

The ureter is reimplanted into the bladder with an adequate tunnel length to prevent reflux. Any obstructive elements can be excised; if the bladder base is attenuated, it can be reconstructed to provide the ureteral tunnel with good detrusor backing. After tailoring, duplicated ureters can be tunneled in a common sheath or side by side, if indicated, or a ureteroureterostomy can be performed cephalad to the intramural tunnel.

Although this approach may leave a poorly functioning or dysplastic renal moiety in place, this rarely causes a problem that necessitates reoperation in the absence of reflux or obstruction.

Open reconstruction at renal level (upper-tract approach)

When a ureterocele is associated with the upper pole of a duplex kidney, the upper pole often demonstrates poor function. Partial nephrectomy and partial ureterectomy may be performed. This approach is favorable if no reflux occurs because it potentially avoids bladder-level surgery. This procedure is usually performed laparoscopically. This is also the procedure of choice for treating an incontinent girl with poorly functioning renal moiety that drains to an ectopic ureter.

Open reconstruction at renal and bladder level (combined approach)

Some authors still champion the combined approach to open reconstruction, but the procedures require separate incisions and are associated with increased operating time and morbidity. When patients are carefully selected, an upper- or lower-end approach usually suffices and carries minimal risk of necessitating further intervention.

Laparoscopic reconstruction

Laparoscopy is becoming more frequently used to perform ureteroureterostomy and ureteropyelostomy, in addition to more traditional uses such as partial nephrectomy where indicated. ^{[16, 17, 18, 19]} Some have argued that in the setting of a poorly functioning upper-pole renal moiety, with other moieties unaffected, a laparoscopic upper-pole heminephrectomy is the procedure of choice. ^[20]

Robotic assistance has also been successfully applied to the laparoscopic approach. ^{[21, 22, 23, 24]}

A single-institution retrospective review by Bansal et al found laparoendoscopic single-site partial nephrectomy to be feasible, safe, and effective for treatment of upper urinary tract duplication anomalies in infants and small children. ^[25]

A retrospective review (N = 59) that compared partial nephrectomy (OPN; n = 24), laparoscopic partial nephrectomy (LPN; n = 7), robotic partial nephrectomy (RPN; n = 18), and laparoendoscopic single-site partial nephrectomy (LESS-PN; n = 10) for treatment of a nonfunctioning moiety in a duplicated collecting system found that the minimally invasive approaches to partial nephrectomy (ie, LPN, RPN, and LESS-PN) were effective and safe and, as compared with the open approach, were associated with decreased postoperative analgesia requirements, shorter hospital stay, reduced blood loss, and less use of drains. ^[26]

Short-term complications include UTI, hematuria, and, rarely, significant blood loss. Long-term complications include recurrence of reflux and, more rarely, ureteral obstruction.

Upper- to lower-pole ureteroureterostomy carries a small risk of UTI related to the residual upper-pole ureteral stump. This risk is greater when the diameter of the upper-pole ureter is larger and when reflux into the stump is present. ^[27] In these situations, an effort to excise the distal stump completely may be warranted.

Heminephrectomy carries some risk of devascularization of the adjacent lower-pole moiety, with loss of some or all of its function. Fortunately, this is uncommon.

Reflux after ureterocele puncture can be seen in the ipsilateral upper or lower moiety or in the contralateral system in nearly half of patients. More than 50% of these cases of reflux may resolve spontaneously; accordingly, conservative management with surveillance is warranted if possible. ^[28]

Postoperative radiographic imaging with ultrasonography (US) and voiding cystourethrography (VCUG) is warranted to ensure the successful treatment of reflux and the absence of any obstructive element.

Postoperative residual hydronephrosis that may persist for years, even in the absence of obstruction, is common. Imaging is usually repeated serially until the hydronephrosis resolves or stabilizes.

Patients with renal scarring or dysplasia should be observed by their primary care physician with annual blood pressure assessment and urinalysis to rule out proteinuria.