TABLE 94.1 URETEROCELE THERAPEUTIC OPTIONS
RICHARD N. YU
CHESTER J. KOH
DAVID A. DIAMOND
Ureteroceles are congenital cystic dilations of the intravesical submucosal ureter. They are more commonly found in female children and are almost exclusively diagnosed in Caucasians. Approximately 10% of these children have bilateral ureteroceles. Ureteroceles may be orthotopic (contained entirely within the bladder) or ectopic (partially situated at the bladder neck or urethra). An orthotopic ureterocele is typically associated with a single collecting system, whereas an ectopic ureterocele is usually associated with the upper pole moiety of a kidney with complete ureteral duplication.
Increasingly, ureteroceles are diagnosed by prenatal ultrasonography (Fig. 94.1A and C), which can demonstrate both the intravesical cystic dilation as well as the corresponding hydronephrosis. These patients should undergo a comprehensive postnatal urologic evaluation and be placed on prophylactic antibiotics, which may help to prevent future urinary tract infections (UTIs) (1).
However, for many children, the diagnosis of ureterocele is only made after a UTI or urosepsis. Ureteroceles can also present as a palpable abdominal mass usually representing a hydronephrotic kidney or as a vaginal mass, which represents prolapse of an ectopic ureterocele. Large ureteroceles may even lead to obstruction of the bladder neck or of the contralateral ureteral orifice, which may result in bilateral hydronephrosis.
In general, ultrasonography is the first radiologic study obtained in diagnosing ureteroceles. In addition to the intravesical cystic dilation, ureteroceles are usually seen with duplex collecting systems, with the ureterocele being associated with hydronephrosis of the upper pole moiety.
A voiding cystourethrogram (VCUG) is essential in the evaluation of the patient with a ureterocele because of the high incidence of concomitant ipsilateral and contralateral vesicoureteral reflux. In addition to the reflux, VCUG can demonstrate the size and location of the ureterocele (Fig. 94.1B).
Currently, the dimercaptosuccinic acid (DMSA) renal scan provides the most precise estimate of the differential renal function between each kidney as well as of the associated upper pole contribution to the overall renal function. DMSA scans may even detect lower moiety abnormalities that may not have been demonstrated by ultrasonography (2).
Intravenous urography (IVU) is less commonly used in the modern evaluation of ureteroceles. However, in cases of unusual urinary tract anatomy, IVU, or increasingly magnetic resonance urography (MRU), may be helpful in the delineation of previously undefined anatomy. Severe hydroureteronephrosis associated with a ureterocele may lead to lateral deviation of the upper pole away from the spine.
INDICATIONS FOR SURGERY
The goals of surgical treatment should be the preservation of renal function, elimination of obstruction and reflux, prevention or elimination of infection, and maintenance of urinary continence while minimizing surgical morbidity (3). The main factors to consider in developing an individual treatment plan should be patient age, patient’s clinical presentation, ureterocele size and anatomy, presence of reflux and UTI, and function of the involved renal segment.
The anatomy and clinical presentations of children with ureteroceles vary widely. Therefore, each child should have an individualized treatment plan, as no single method of surgical repair is appropriate for all cases. Table 94.1 lists some therapeutic options for patients with ureteroceles.
Techniques that preserve functional upper pole moieties are listed in the following sections. However, in many instances, the upper pole has little to no contribution to the overall renal function and upper pole ablative techniques, also described in the following sections, may be indicated.
Upper Pole Preservation
Endoscopic Incision of the Ureterocele
The goal of endoscopic incision of ureteroceles is to decompress the ureterocele in a minimally invasive manner while minimizing the risk of postincision vesicoureteral reflux and the need for further urinary tract reconstruction (4). This technique can be used in infants, if infant-sized endoscopic equipment is available, and should be used to drain obstructive urinary systems in any ureterocele patient with urosepsis. Blyth and coauthors (5) recommended the use of a 3Fr Bugbee wire electrode (using the cutting current) to incise the roof of the ureterocele through its full thickness near its base and proximal to the bladder neck. A new unobstructed intravesical ureteral orifice will be created, and the roof of the collapsed ureterocele can act as a flap-valve mechanism to prevent reflux. Although the Bugbee electrode has been a widely used instrument for ureterocele puncture, it has limitations, primarily that following initial decompression enlargement of the puncture site is difficult. Therefore, we prefer to use the pediatric resectoscope and the right-angle hook electrode with the cutting current, which allows one to make a clean transverse incision and enlarge it by placing the hook into the original incision and withdrawing under vision (Fig. 94.2). Magnification with the use of video projection helps improve the accuracy of the incision. Making the incision as distal and as close to the bladder neck as possible should reduce the risk of postoperative reflux into the corresponding ureter. The adequacy of the incision can be confirmed by the presence of a jet of urine from the ureterocele or by visualization of the urothelium inside the ureterocele. The major advantage of the endoscopic incision is that it can be done on an outpatient basis without the need for hospitalization. Other energy sources, such as a laser, have also been described as an alternative method of endoscopic incision (6).