Ureterocele Treatment & Management

Observation alone is rarely a good option in symptomatic ureteroceles. Antibiotic prophylaxis is started in newborns with prenatal diagnosis of ureterocele, which decreases the overall incidence of urinary infection. In the setting of urosepsis with ureterocele, the physician must rapidly initiate aggressive antibiotic therapy. Antibiotics should be instituted during the initial diagnostic evaluation and during surgical intervention for both pediatric and adult ureteroceles.

Indications for surgical treatment for both pediatric and adult ureteroceles depend on the site of the ureterocele, the clinical situation, associated renal anomalies, and the size of the ureterocele.

Goals of treatment include the following:

• Control of infection
• Preservation of renal function
• Protection of ipsilateral and contralateral renal units
• Maintenance of urinary continence
• Elimination of obstruction and reflux

Surgical approach is selected based on the following:

• Age of the patient
• Size and location of ureterocele
• Degree of renal function
• Presence and degree of vesicoureteral reflux
• Comorbid conditions (risk of anesthesia)

Surgical therapy for both pediatric and adult ureteroceles may include endoscopic puncture, incision or transurethral unroofing of the ureterocele, upper pole heminephrectomy, excision of ureterocele and ureteral reimplantation, and nephroureterectomy.

Endoscopic puncture

Endoscopic puncture is the least invasive method for ureterocele decompression. This is an ideal method for dealing with a neonate with ureterocele-induced obstructive uropathy and sepsis. It may also be performed safely in adults with a symptomatic ureterocele. Other indications include a single system intravesical ureterocele with obstruction or a duplex system ureterocele with indeterminate function of the affected renal moiety. Performed via the cystoscope, a small puncture is created at the base of the ureterocele. This technique is often done using a 3F Bugbee electrode. The thermal damage to the surrounding tissue subsequently results in an opening larger than 3F. With a thick-walled ureterocele, either a larger puncture or incision, or multiple punctures may be required to establish drainage. Multiple endoscopic procedures may be required to successfully decompress an ectopic ureterocele.^[3]

This procedure also allows palliative decompression in children at high risk (secondary to concurrent medical illness), so that definitive reconstruction can be delayed until an adequate healing period has occurred. Antibiotic prophylaxis should be administered postoperatively in pediatric patients until a VCUG can be performed to assess for vesicoureteral reflux. Ectopic ureterocele and duplicated system are associated with a significantly higher rate of secondary procedures, which is most often related to the presence of reflux. Endoscopic treatment provides definitive therapy in only 10-40% of patients with ectopic ureteroceles, compared to 80-90% of patients with a single system intravesical ureterocele.

Transurethral unroofing

Transurethral unroofing of a ureterocele in adults reliably achieves decompression and allows effective treatment of infection and calculi in symptomatic ureteroceles. Low transverse incision of the ureterocele, as described by Monfort and colleagues^[3] creates a "flap-valve" effect and minimizes the chance of subsequent vesicoureteral reflux compared with transurethral resection of the ureterocele roof. The actual incidence of reflux after endoscopic unroofing in ureteroceles in adults is unknown because a large prospective adult series is lacking. However, several case reports have alluded to the fact that the incidence of reflux appears to be proportional to the type of incision made.

Vesicoureteral reflux in adults is not routinely treated with ureteral reimplantation. Rather, these patients are monitored expectantly, and the need for reimplantation is tailored to the individual. Data on the use of bulking agents for treatment of adult vesicoureteral reflux in these situations are lacking. The potential for vesicoureteral reflux limits the use of endoscopic unroofing in children.

Upper pole heminephrectomy and partial ureterectomy

Upper pole heminephrectomy and partial ureterectomy with ureterocele decompression involves removal of the upper pole of the kidney, as well as the affected proximal ureter to the level of iliac vessels. The remaining distal ureterocele is not excised but rather is decompressed. This is the definitive treatment in patients with an obstructed ectopic ureterocele and a dysplastic upper pole, but without associated vesicoureteral reflux. If reflux is present preoperatively, the distal ureter should be ligated. Upper pole heminephrectomy and partial ureterectomy with ureterocele decompression is a reasonable alternative for adults in whom transurethral ureterocele unroofing has failed due to technical or anatomical difficulties.

Upper pole heminephrectomy and partial ureterectomy with ureterocele decompression has been reported to cause spontaneous resolution of grade I and II vesicoureteral reflux in 60% of cases, while higher grades of reflux necessitated bladder reconstruction in 96% of cases. While upper pole heminephrectomy provides effective decompression, the risk for subsequent bladder surgery may be significant, especially if reflux is present.

Factors that may predict the likelihood of future surgical intervention include the following:

• High-grade reflux (grades III, IV, V)
• Complications resulting from remaining stump of upper ureter (ie, UTI, calculus)
• Poor detrusor backing behind the remaining ureterocele

Upper pole heminephrectomy is an excellent first-line procedure for the relatively rare child with minimally functioning upper pole and no reflux. However, the patient and family should be counseled regarding the potential need for further surgical procedures.

Ureteropyelostomy

Ureteropyelostomy is an operation that joins the upper pole ureter to the lower pole renal pelvis. This is preferred in both children and adults if the affected renal unit demonstrates significant function on nuclear renography and there is no associated vesicoureteral reflux. Alternatively, a high ureteroureterostomy may also be performed.

Excision of the ureterocele and ureteral reimplantation

Ureterocele excision with ureteroneocystostomy is indicated as a primary procedure if the patient has significant vesicoureteral reflux in the lower pole moiety and/or significant contralateral vesicoureteral reflux. Both ipsilateral ureters may be reimplanted within a common sheath or via ureteroureterostomy. Note that common sheath reimplantation has the distinct disadvantage of reimplanting a very dilated distal ureter into the small bladder of an infant. The decision whether to taper the ureters must be made on an individual basis. This operation is commonly delayed until the child is older (aged approximately 2 y) following endoscopic puncture as an infant. Ureteral reimplantation is not commonly performed in adults as most patients respond favorably to endoscopic unroofing of the ureterocele.

In the pediatric population, ureterocele excision and ureteral reimplantation is commonly a secondary procedure (after previous heminephrectomy or endoscopic incision of a ureterocele) because of recurrent urinary tract infections, voiding disturbance, persistent vesicoureteral reflux, or obstruction. Significant vesicoureteral reflux on initial VCUG usually indicates that lower-tract reconstruction will be necessary. Of note, if ureteral reimplant is performed as first-line treatment in the appropriately selected patient, the rate of secondary surgery is low.

Nephroureterectomy

Nephroureterectomy is performed in patients with single system ureterocele and a poorly functioning kidney. A recent retrospective review reported a 4% incidence of nephroureterectomy in children with single system ureterocele and nonfunctioning renal unit.^[4] The traditional method of correcting an ectopic ureterocele in a duplex system has been to perform a total reconstruction. This involved a bladder level operation with ureterocele excision and reimplantation of the lower pole ureter, followed by a flank incision and upper pole heminephrectomy. Since most ureteroceles typically present in young children, total reconstruction was technically challenging, and complications were common.

Treatment of the ureterocele is individualized based on the patient age, clinical situation, type of ureterocele, presence of vesicoureteral reflux, renal function and surgeon preference.

In the neonate or infant, transurethral puncture of the ureterocele or an upper tract approach (eg, heminephrectomy) would be the most feasible options, while excision of the ureterocele with bladder reconstruction or total reconstruction, including heminephrectomy, may be added to the therapeutic armamentarium in the older child (>2 y). In the adult, transurethral unroofing of the ureterocele is a reasonable first-line approach, because the development of postoperative vesicoureteral reflux is less problematic than in the child.

Treatment of the ureterocele is individualized based on the patient age, clinical situation, functional characteristics of the ureterocele, and surgeon preference. In the neonate or infant, transurethral puncture of the ureterocele or an upper tract approach (ie, heminephrectomy) are often the most feasible options. For infants and children with an intravesical nonrefluxing ureterocele, endoscopic puncture is usually the first-line therapy because it is minimally invasive and has a high chance of providing definitive treatment. For those with a nonrefluxing, poorly functioning upper pole associated with an ectopic ureterocele, an upper pole heminephrectomy is a reasonable first-line therapy. Opinions and approaches vary the most in those children with ectopic ureteroceles associated with vesicoureteral reflux.

In the adult, transurethral unroofing of the ureterocele is a reasonable first-line approach, because the development of postoperative vesicoureteral reflux is less problematic than in the pediatric population.

The goals of the preoperative evaluation of the ureterocele include:

• Detailed delineation of upper and lower urinary tract anatomy
• Estimation of differential function of all renal moieties
• Determination of the presence of obstruction (anatomic or functional) and/or vesicoureteric reflux

After a preoperative evaluation has been completed, one must obtain a properly informed consent and preoperative laboratory studies. Patients and parents should be counseled regarding the possibility that additional procedures will be required.

When an upper tract approach is planned, preoperative function of the involved moiety and appearance of the renal parenchyma intraoperatively contribute to the decision whether to perform heminephrectomy or renal-sparing surgery (i.e., ureteroureterostomy or ureteropyelostomy).

The principles of successful ureteral reimplantation, with or without ureteral remodeling and bladder reconstruction, are as follows:

• Protection of contralateral ureteral orifice
• Adequate ureteral exposure and mobilization
• Gentle handling of the tissue and meticulous preservation of blood supply
• Creation of a valvular mechanism with a submucosal tunnel with a length to ureteral diameter ratio of 5:1

Endoscopic incision of ureterocele

This is the least invasive technique. The patient is placed in dorsolithotomy position; infants can be placed in the supine position with the lower extremities frog-legged to facilitate urethral exposure. Incision of the ureterocele is performed via the cystoscope. Using a small Bugbee electrode (3F) on cutting current, a small puncture is created at a low point just above the base of the ureterocele.

Upper pole heminephrectomy and partial ureterectomy with ureterocele decompression

The patient should be placed in the flank or lateral decubitus position. A Foley catheter is placed to straight drainage. A flank incision is made off the 12th rib. The kidney and proximal ureter are mobilized. The non-functioning upper pole moeity is excised. There is no need to dissect out the renal hilum. Manual compression of the kidney provides adequate vascular control. Next, excise the upper pole ureter to the level of the iliac vessels. Dissect on the upper pole ureter to avoid injury to the lower pole ureter. Use a catheter to decompress the distal ureterocele. If there is no reflux into the ureterocele, the stump is left open. If reflux is present, the ureter should be ligated. Irrigate the wound and leave a small drain in the pelvis.

Ureteropyelostomy

The patient is positioned supine and a Foley catheter inserted. An anterior subcostal incision is created. Staying extraperitoneal, both ureters are dissected. Incise the upper pole ureter laterally and incise the lower pole renal pelvis medially. Anastomose the upper pole ureter to lower pole renal pelvis. Insertion of a double J stent or a feeding tube is optional.

Excision of ureterocele with ureteral reimplantation

The patient is placed in a supine position. A Pfannenstiel incision is made. The bladder is opened and the ureteral orifice and the ureterocele are identified. Both ureteral orifices are cannulated with infant feeding tubes. Circumscribe and remove the root of the distal ureterocele followed by the floor of the ureterocele. Close the defect in the detrusor. Ureters may be reimplanted within the common sheath or via ureteroureterostomy, with or without tapering of the ureter.

Nephroureterectomy

Nephroureterectomy involves the excision of the ureterocele, ureter, and the ipsilateral renal moiety. This is followed by reimplantation of the lower pole ureter. This approach requires two separate incisions (flank and lower abdominal) and is associated with fairly high morbidity. This type of total reconstruction is not commonly used today; however, with the advent of laparoscopic techniques, the morbidity of this approach has been reduced.

Intravenous antibiotics are continued until the patient is discharged from the hospital. Pediatric patients should be placed on prophylactic antibiotics until VCUG demonstrates resolution of reflux. Urethral catheters are removed when the urine has cleared. Depending on the operation, hospital stay ranges 1-4 days. Patients undergoing endoscopic procedures are often discharged on the same day. If an internal stent is placed, it is removed 3-6 weeks after surgery. Postoperative imaging studies such as renal ultrasonography and VCUG are usually obtained 6-8 weeks postoperatively as dictated by individual clinical scenario or postoperative complications.

Follow-up care consists of serial monitoring of renal function, periodic evaluation of voiding symptoms and bladder function, and interval radiologic studies to assess renal growth, hydroureteronephrosis, and vesicoureteral reflux.

For excellent patient education resources, visit eMedicine's Kidneys and Urinary System Center. Also, see eMedicine's patient education article Intravenous Pyelogram.

• Endoscopic incision of ureterocele

  • Iatrogenic VUR, especially in extravesical ureteroceles.
  • This is infrequently definitive therapy, except in cases of a single system intravesical ureterocele.
• Upper pole heminephrectomy

  • Highest risk of -intraoperative blood loss
  • Vascular compromise of lower pole with potential loss of renal function
  • Need for further lower tract reconstruction due to persistent reflux, infection, or failure to decompress the ureterocele
• Excision of ureterocele and ureteral reimplantation

  • Problematic hematuria and/or bladder spasms
  • Damage to bladder neck or continence mechanism
  • Injury to the contralateral ureteral orifice
  • Compromise of blood supply to the lower pole ureter

No single approach is appropriate for all patients with ureteroceles; therefore, each case must be tailored to the individual. An experienced surgeon must be armed with various surgical techniques (as discussed above) that can be tailored to effectively treat different types of ureterocele malformations. When an appropriate operation is used to correct a specific abnormality, the outcomes remain excellent in both pediatric and adult patients.

Management of ureterocele remains both challenging and controversial, with a vast clinical spectrum, making development of a standardized approach difficult. Robotic-assisted ureteral reimplantation and heminephrectomy are gaining popularity and will continue to evolve. Open ureteral reimplantation, ureteropyelostomy and heminephrectomy currently remain the criterion standard for surgical management of symptomatic ureteroceles that are not successfully managed endoscopically.

Although different surgical philosophies exist in managing adult and pediatric ureteroceles, the following principles may apply:

• Endoscopic puncture of ureteroceles should be used as a primary treatment modality in the setting of any patient with urosepsis or concurrent medical conditions that pose significant anesthesia-related risk.
• Upper pole heminephrectomy with partial ureterectomy is reasonable in the setting of a nonfunctioning upper pole renal moiety without associated vesicoureteral reflux.
• Ureterocelectomy and bladder reconstruction are acceptable in the setting of a ureterocele associated with significant vesicoureteral reflux in either kidney.