Screening for Zinner Syndrome in Patients With a Congenitally Solitary Kidney: Lessons Learned.

PURPOSE: We determined if serial screening ultrasounds are beneficial in evaluating for the development of Zinner syndrome in males with a congenital solitary kidney. MATERIALS AND METHODS: All patients included had their congenital solitary kidney diagnosed at <20 years of age and had to be ≥20 at their last visit. Individuals were seen annually, with pelvic ultrasounds to screen for mesonephric duct cysts obtained at birth and every year of age, divisible by 5. RESULTS: At a median follow-up of 38 years of age (range 20-57), 17% (20/121) developed Zinner syndrome, with 60% (12/20) developing clinical symptoms. The yield for screening ultrasound studies was significantly higher in patients ≤20 years of age at 3.5% (12/340), compared to 0.33% (1/296) in patients >20 years of age (P = .004). Serial ultrasounds reveal the onset of lower urinary tract and cyst-related pain symptoms are associated with the growth of the seminal vesicle cyst to ≥5 cm (P = .0198). Of symptomatic patients, 75% (8/12) had abnormal uroflows. Complete urodynamic studies revealed findings consistent with bladder outlet obstruction in 38% (3/8), equivocal for obstruction in 24% (2/8), and detrusor underactivity in 38% (3/8). Cyst excision effectively resolved voiding symptoms that were obstructive in etiology but failed to resolve symptoms in patients with detrusor underactivity. CONCLUSIONS: Serial ultrasound evaluations reveal that cyst growth to ≥5 cm in size is highly related to the onset of clinical symptoms, with the resolution of voiding symptoms by cyst excision directly associated with urodynamic findings.

Erectile dysfunction in patients undergoing multiple attempts at hypospadias repair: Etiologies and concerns.

INTRODUCTION: One-third of adult patients presenting for the repair of persistent penile defects after failing multiple hypospadias repair attempts during childhood will complain of erectile dysfunction (ED). The goal of this paper is to identify possible etiological causes of its onset. MATERIALS AND METHODS: Five selection criteria were used for entrance into the study: 1) Patients had to have failed ≥ three prior hypospadias repair attempts. 2) Present for evaluation between 18 and 40 years of age. 3) No known congenital or medical anomaly could be present that could have predisposed to erectile dysfunction. 4) Sexual history inventory for men (SHIM-5 score) completed. 5) All patients with moderate to severe ED (SHIM scores ≤ 16) underwent psychological screening; individuals with good quality spontaneous or self-stimulated erections, experiencing major life events, or had documented psychological problems were excluded from the study. One hundred consecutive patients meeting these criteria were assessed. We evaluated multiple factors to discern if they were associated with the onset of ED: the initial location of the urethral meatus, if a corporoplasty was performed, the type of corporoplasty used, if the urethral plate was divided or resected, the use of a ventral corporal graft, the total number of open reparative procedures performed before referral, the number of direct visual internal urethrotomies (DVIU) performed, the length of a urethral stricture at the time of the referral and whether lichen sclerosus was present. Statistical evaluations used chi-square analysis, two-tailed t-tests, or a logistic regression model where indicated, p-values < 0.05 were considered significant. RESULTS: 37% (37/100) of our patients complained of moderate to severe ED (SHIM score ≤16). Statistical analysis comparing patients with ED to those without ED (63%:63/100), revealed patients with ED were older, median age 34 yrs (range 20-40) vs 26 yrs (range 18-40) p = 0.0212, had undergone division of the urethral plate 70.3% (26/37) vs 47.6% (30/63), p = 0.0276, had placement of a ventral corporal graft, 24% (8/33) vs 1.5% (1/67), p = 0.0003 or had undergone repetitive DVIU's to manage urethral stricture disease, median number 4 (range 0-15) vs 0 (range 0-6), p < 0.0001, see table. CONCLUSIONS: The early onset of ED in patients that failed multiple attempts at hypospadias repair in childhood is associated with advancing age, division of the urethral plate, and prior ventral corporal grafting. Especially significant is the association of ED to the use of repetitive internal urethrotomy to manage urethral stricture disease.

Cancer screening in the pediatric cancer patient: a focus on genitourinary malignancies, and why does a urologist need to know about this?

INTRODUCTION: The long-term survival of a patient with childhood cancer now exceeds 80%. Unfortunately, as survivorship improves, the long-term consequences of the treatments used have become manifest. Specifically, the finding that development of a subsequent malignant neoplasm (SMN) is the leading cause of late mortality is concerning. In cancer survivors who are at high risk for developing an SMN, cancer screening protocols have well-documented survivorship benefits. Regrettably, 50% of these high-risk patients are non-compliant with these protocols, with studies revealing that inadequate patient compliance is in part because of insufficient knowledge of the physician regarding its need. DISCUSSION: Urologists are in a unique position to correct this deficiency. Characteristically, survivors of childhood cancer present to urologists as an adult with complaints of infertility, erectile dysfunction, androgen deprivation, lower urinary tract symptoms or for follow-up of a urinary diversion. The urologist because of their specialty should be able to treat the patients presenting complaint, identify the high-risk patient, and re-establish them on their surveillance protocol. SCREENING RECOMMENDATIONS FOR HIGH-RISK PATIENTS: The risk for developing an SMN is unequally expressed and is temporally biphasic. A minimal 10-year follow-up time span is recommended for patients who received alkylating agents or topoisomerase inhibitors. These agents can induce hematologic malignancies classically within the first 3-5 years after chemotherapy completion, with minimal risk existing after 10 years. Lifelong follow-up for SMN development is recommended under five distinct circumstances; if a genetic predisposition to tumor formation exists, a persistent post-treatment non-malignant mass is present if chemotherapy was received before 2 years of age, if the initial type of tumor predisposes to SMN, or if the patient received radiation therapy. CONCLUSION: The urologists ability to identify the patient at high risk for developing an SMN and return them to a surveillance protocol is crucial for appropriate patient management.

Systematic review of bladder cancer outcomes in patients with spina bifida.

BACKGROUND: In patients with congenital bladder anomalies, bladder augmentation is used as a last resort to reduce intravesical pressure, but concerns about malignant transformation in augmented patients were first raised in the 1980s. The best evidence to date indicates that augmentation does not appear to increase the risk of bladder cancer in spina bifida patients. To date, oncologic outcomes from patients with spina bifida with and without augmentation have only been available in small case reports. OBJECTIVE: To systematically evaluate factors in myelomeningocele patients with bladder cancer, including bladder augmentation, that contribute to overall survival (OS). STUDY DESIGN: A systematic review using PubMed was conducted by cross referencing terms 'myelomeningocele,' 'cystoplasty,' 'bladder cancer' and respective synonyms according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Inclusion criteria were studies with patients with an underlying diagnosis of myelomeningocele and bladder cancer with data on age, stage, and mortality status. Studies were excluded for spinal cord injury, history of tuberculosis or schistosomiasis, or prior ureterosigmoidostomy. RESULTS: Fifty-two patients were identified from 28 studies with a median age at bladder cancer diagnosis of 41 years (range 13-73); 37 (71%) presented with stage III or IV bladder cancer. Overall survival at 1 year and 2 years was 48.5% and 31.5%, respectively. Overall survival was different between those with and without augmentation (P = 0.009) by log-rank analysis. No between-group differences in OS were seen based on age, management with indwelling catheter, diversion with ileal conduit or being on a surveillance program. Only stage remained a significant predictor of OS on multivariate analysis (HR 2.011, 95% CI 1.063-3.804, P = 0.032). Secondary analysis was performed after removing patients with gastric augmentation (n = 8), and no difference in OS was seen between patients with (n = 8) and without augmentation (n = 36, P = 0.112). Of augmented patients, latency to development of bladder cancer was variable (Summary Figure). DISCUSSION: Bladder cancer is a deadly diagnosis in patients with congenital bladder anomalies like spina bifida, and while overall prevalence of the two conditions occurring together is low, bladder cancer will go on to affect 2-4% of spina bifida patients. The present study examined overall survival, and further characterized outcomes in these patients. Presence of a bladder augment did not appear to worsen overall survival. CONCLUSIONS: Patients with myelomeningocele who developed bladder cancer had aggressive disease. Augmentation did not worsen OS, based on cases reported in the literature. Risk of bladder cancer should be discussed with all myelomeningocele patients.

Mortality following augmentation cystoplasty: A transitional urologist's viewpoint.

INTRODUCTION: Three complications have been hypothesized to increase patient mortality following enterocystoplasty: spontaneous bladder perforation, bladder neoplasia, and chronic renal failure (CRF). The present study examined risk of their occurrence and discussed ways to improve the quality of care. MATERIALS AND METHODS: The present transitional clinic followed 385 patients with a history of bladder augmentation using either ileal, sigmoid, or ascending colon. The median age was 37 years (range 16-71). Median follow-up interval after augmentation was 26 years (range 2-59). DISCUSSION: Spontaneous rupture of the bladder occurred in 3% (13/385), with one associated death (0.25%, 1/385). Spontaneous bladder rupture significantly correlated with substance abuse, non-compliance with catheterization, and mental/physical disabilities that required the use of surrogates to perform and monitor intermittent catheterization (P < 0.01). Of the 203 patients that were followed for ≥10 years, 4% (8/203) developed a bladder tumor. In comparison, 2.5% (5/203) of an age-matched control population, managed by anticholinergics and intermittent catheterization, developed a bladder tumor. Therefore, enterocystoplasty cannot be associated with an increased risk of cancer development (P = 0.397). Chronic renal failure ≥ Stage 3 arose in 15% (58/385), and 1% (4/385) of the patients died as a result of this complication. Obese patients (BMI ≥30) catheterizing per urethra were more likely to be non-compliant with catheterization and develop CRF compared with obese patients with a continent catheterizable stoma (P > 0.001). These findings suggest that compliance with intermittent catheterization and renal preservation are enhanced by the presence of a catheterizable abdominal stoma. CONCLUSION: The individual's intellectual and physical capability to obey medical directives, refrain from high-risk habits, maintain a healthy weight, and comply with long-term follow-up visits were all critical to the enduring success of bladder augmentation.

Development of late, symptomatic abdominopelvic lymphoceles more than 10 years following pediatric reconstructive urologic procedures.

PURPOSE: Rare reports of symptomatic abdominopelvic lymphoceles following pediatric genitourinary reconstruction do exist; however there are no data regarding the development or management of late symptomatic lymphoceles. We report on the clinical presentation of these lymphoceles 10 or more years following initial urologic surgery. MATERIALS AND METHODS: We reviewed 480 patients following major intra-abdominal urologic reconstructive procedures from 1986 to 2009 for development of late, symptomatic abdominopelvic lymphoceles. A minimum of 10 years post-surgical follow up was required for inclusion. RESULTS: Late symptomatic lymphoceles developed in 4/480 (0.8%) patients. Median length of follow up post reconstruction was 13.5 years (range 10-17). Median time to lymphocele development was 12 years (range 8-16). Symptoms at presentation included abdominal distension (4/4, 100%), nausea and vomiting (3/4, 75%), flank pain/progressive hydroureteronephrosis (3/4, 75%), and obstructive pyelonephritis (1/4, 25%). Additional surgical procedures that may have contributed to lymphocele development were present in 100%. 75% (3/4) of the patients underwent open surgical drainage, with one electing observation for intermittent symptoms. Exploration revealed loculated fluid collections between bowel loops and dense adhesions; symptoms resolved although small asymptomatic recurrences developed in all patients. CONCLUSIONS: Late, symptomatic abdominopelvic lymphoceles following major pediatric urinary tract reconstruction or diversion develop in <1% patients. Many undergo subsequent abdominopelvic surgery, which may contribute to development of these late, pathologic lymphoceles. Open surgical drainage is usually required with excellent outcome.

Evaluation of the United States pediatric urology workforce and fellowships: a series of surveys performed in 2006-2010.

OBJECTIVE: In the US, there has been an evolution in the practice of pediatric urology from a primary academic sub-specialty focused on reconstruction of major congenital genitorurinary abnormalities to a mixed academic and private practice that serves as the primary care giver for all pediatric urologic concerns. The estimated manpower needs were unable to be resolved, due to our inability to determine the impact of sub-specialty certification on referral patterns, along with the failure to embrace the use of physician extenders. Here, we review a series of surveys performed in 2006-2010 regarding the sub-specialty of pediatric urology. MATERIALS AND METHODS: The four surveys focused on workforce needs, appraised the financial impact of educational debt on the pediatric urology community, and evaluated concerns of the current fellows in training. RESULTS: The median financial income for a pediatric urologist, the resident's educational debt load, and a desire of the fellows to have an open dialog with the urologic community regarding the merits of the research year are revealed. CONCLUSION: We have identified that the ability to recruit fellows into our field is dependent upon a combination of factors: interest in the field, job availability in relationship to geographic locations, mentoring, concerns regarding financial/familial hardships encountered during a 2-year fellowship, and the lack of increased financial reimbursement for the extra training required.

Augmentation cystoplasty and risk of neoplasia: fact, fiction and controversy.

PURPOSE: We determined if ileal/colonic bladder augmentation performed in patients with congenital bladder abnormalities is an independent risk factor for bladder malignancy. MATERIALS AND METHODS: We reviewed a registry of patients with bladder dysfunction due to neurological abnormalities, exstrophy and posterior urethral valves. Individuals treated with augmentation cystoplasty were matched (1:1) to a control group treated with intermittent catheterization based on etiology of bladder dysfunction, gender and age (±2 years). RESULTS: We evaluated 153 patients with an ileal/colonic cystoplasty and a matched control population. There was no difference (p=0.54) in the incidence of bladder cancer in patients with augmentation cystoplasty (7 patients [4.6%]) vs controls (4 [2.6%]). In addition, there was no difference between the 2 groups regarding age at diagnosis (51 vs 49.5 years, p>0.7), stage (3.4 vs 3.8, p>0.5), mortality rate (5 of 7 [71%] vs 4 of 4 [100%], p>0.4) or median survival (18 vs 17 months, p>0.8). Irrespective of augmentation status patients with a history of renal transplant on chronic immunosuppression had a significantly higher incidence of bladder cancer (3 of 20 [15%]), compared to patients who were not immunosuppressed (8 of 286 [2.8%], p=0.03). CONCLUSIONS: In patients with congenital bladder dysfunction ileal/colonic bladder augmentation does not appear to increase the risk of bladder malignancy over the inherent cancer risk associated with the underlying congenital abnormality. In addition, immunosuppression irrespective of bladder treatment is an independent risk factor for malignancy in this patient population.

Long-term follow up of enteric bladder augmentations: the risk for malignancy.

OBJECTIVE: To determine the risk of bladder cancer following enteric bladder augmentation. MATERIALS AND METHODS: Patients followed for care after an enteric bladder augmentation have been entered into a registry; individuals followed for a minimum of 10 years were evaluated. RESULTS: The study criteria were met by 153 patients. Indications for bladder augmentation were neurogenic bladder in 97, exstrophy in 38 and posterior urethral valves in 18. There was a median follow-up interval of 27 years (range 10-53). A total of seven cases of malignancy developed. Median time to tumor development following augmentation was 32 years (range 22-52). Two patients with neurogenic bladder developed transitional cell carcinoma; both were heavy smokers (>50 pack per year history). Two patients with a history of posterior urethral valves and renal transplantation developed adenocarcinoma of the enteric augment. Three patients with bladder exstrophy developed multifocal adenocarcinoma of the augmented bladder. Two patients remain alive, 5 and 6 years following radical cystoprostatectomy; five died of cancer-specific causes. CONCLUSIONS: Malignancy following enteric bladder augmentation arose in 4.5% (7/153) of our patients and was associated with coexisting carcinogenic stimuli (prolonged tobacco/chronic immunosuppressive exposure), or alternatively with the inherent risk of malignancy existing with bladder exstrophy.

Klippel-Trenaunay syndrome: incidence and treatment of genitourinary sequelae.

PURPOSE: Prior reviews regarding genitourinary manifestations of Klippel-Trenaunay syndrome used data acquired from diverse specialty specific articles to define the incidence and sequelae of its genitourinary manifestations. We believe that this resulted in erroneous conclusions regarding the urological complications of Klippel-Trenaunay syndrome. MATERIALS AND METHODS: Data on genitourinary manifestations in patients with Klippel-Trenaunay syndrome treated at 1 institution from 1970 through 2005 were acquired. RESULTS: Of 218 patients with Klippel-Trenaunay syndrome 30% (66 of 218) had genitourinary involvement, including 7% (15) with cutaneous genital abnormalities, 7% (15) with visceral genitourinary involvement and 16% (36) with each type. Intermittent bleeding from cutaneous genital abnormalities developed in 65% of patients (33 of 51). Conservative treatment with compression and/or cauterization was attempted in all 33 patients and it was successful in 64% (21 of 33). Intractable hemorrhage resulted in excision of the cutaneous bleeding site in 36% of cases (12 of 33). A total of 39 hospitalizations for gross hematuria occurred in 9% of the patients (19 of 218). Hematuria developed from the bladder in 11 cases, the urethra in 4 and the kidney in 4. Conservative therapy resolved gross hematuria in 21% of the patients (4 of 19). Refractory hematuria was successfully treated with cauterization in 37% of the patients (7 of 19) and by angiographic embolization in 10% (2 of 19). Intractable gross hematuria resulted in open surgical excision of the bleeding site in 32% of the patients (6 of 19). CONCLUSIONS: The incidence of genitourinary manifestations of Klippel-Trenaunay syndrome is 30%, which is triple the previously reported incidence of 9%. Unlike prior reports stating that the genitourinary abnormalities rarely caused problems, 52% of the patients (34 of 66) with Klippel-Trenaunay syndrome who had urological manifestations eventually required interventional therapy for genitourinary complications.

Long-term followup of visual internal urethrotomy for management of short (less than 1 cm) penile urethral strictures following hypospadias repair.

PURPOSE: We reviewed the results of direct vision urethrotomy for short (less than 1 cm) penile urethral strictures following hypospadias surgery. MATERIALS AND METHODS: Patients with less than 1 cm anterior penile urethral strictures located proximal to the meatus underwent direct vision urethrotomy. Based on the type of initial urethroplasty patients were randomly divided into treatment with direct vision urethrotomy vs direct vision urethrotomy plus clean intermittent catheterization for 3 months. Success was defined as absent obstructive voiding symptoms and a normal urine flow 2 years following the last patient instrumentation. RESULTS: Of patients with urethral strictures following hypospadias repair 44% (32) had previously undergone tubularized graft urethroplasty and 56% (40) had previously undergone flap urethroplasty, including a tubularized island flap in 18, an onlay flap in 11 and urethral plate urethroplasty in 11. Direct vision urethrotomy alone was performed in 51% of patients (37), and direct vision urethrotomy and clean intermittent catheterization were performed in 49% (35). Success with the 2 methods was similar, that is 24% (9 of 37 patients) vs 22% (8 of 35). Following direct vision urethrotomy all patients with tubularized graft urethroplasty showed failure (0 of 32). Success was noted in 11% of patients (2 of 18) with tubularized island flap urethroplasty compared to 72% (8 of 11) with onlay urethroplasty and 63% (7 of 11) with urethral plate urethroplasty (each p <0.05). CONCLUSIONS: The addition of clean intermittent catheterization to direct vision urethrotomy does not improve the likelihood of success. Direct vision urethrotomy for short (less than 1 cm) urethral stricture usually fails following any type of tubularized graft or flap urethroplasty but it had moderate success following onlay flap and urethral plate urethroplasties.

Double-blind placebo controlled study of alpha-adrenergic receptor antagonists (doxazosin) for treatment of voiding dysfunction in the pediatric population.

PURPOSE: We sought to determine whether the alpha-adrenergic antagonist doxazosin could be used as primary therapy in children with voiding dysfunction. MATERIALS AND METHODS: Children were assigned to maintain a voiding diary and then randomly divided into a double-blind placebo controlled protocol (0.5 mg doxazosin or placebo). Duplicate uroflow studies with post-void residual evaluations and assessment of dysfunctional voiding scores were performed on initiation and completion of the study. At the conclusion parents were asked to rank the perceived improvement of the urinary incontinence (ie parental subjective perception of improvement). RESULTS: No significant differences between doxazosin (18) and placebo (20) treated patients were found in the number of incontinent days per week, severity of incontinent episodes or alterations in uroflow patterns. Although not significant, 2 findings suggested a beneficial effect of doxazosin over placebo. Specifically, doxazosin decreased the number of incontinent episodes weekly from a median of 18 to 4, while the number of incontinent episodes weekly in the placebo group remained essentially unchanged, decreasing from 15 to 14 (p = 0.13). Doxazosin also improved the dysfunctional voiding scores over placebo, for an improvement of -3 vs 0 points. Further substantiating a doxazosin effect over placebo was the subjective perception of the parents that doxazosin significantly improved urinary continence (p <0.02). CONCLUSIONS: Compared to placebo, doxazosin did not demonstrate a significant objective benefit, but produced a significant subjective benefit in the treatment of urinary incontinence secondary to voiding dysfunction.

Loss of the penile glans and/or corpora following primary repair of bladder exstrophy using the complete penile disassembly technique.

PURPOSE: The technique of penile disassembly is increasingly being used for the surgical repair of classical bladder exstrophy. We describe the complications and discuss the possible etiologies of genital injuries we have seen following this operation. MATERIALS AND METHODS: A review of the records of patients evaluated for management of genital complications following complete repair of bladder exstrophy from 1996 to 2003 was performed. RESULTS: Nine patients were evaluated for genital injuries following complete repair of bladder exstrophy using the penile disassembly technique. Injuries included the loss of 1 hemiglans and penile urethra in 2 cases; loss of 1 hemiglans and distal corpora in 2; loss of bilateral glans, distal corpora and penile urethra in 2; loss of 1 hemiglans, 1 corporal body, urethral plate and penile shaft skin in 1; loss of 1 hemiglans, distal corporal body and a portion of the urethra in 1; and loss of 1 hemiglans in 1. The exact etiology of these complications is unknown, and possibilities include, a technical mishap, induction of venous congestion/arterial spasm or disruption of a congenitally abnormal blood supply. CONCLUSIONS: The finding that primary repair of bladder exstrophy using the penile disassembly technique is associated with the risk of partial or complete penile loss dampens our enthusiasm for this procedure. The association of genital injuries with penile disassembly has resulted in a modification of this surgical technique that would hopefully lessen its risk.

The androgen insensitive micropenis: long-term follow-up into adulthood.

AIM: To assess the adult stretched penile length (SPL) and sexuality in patients with micropenis who exhibited an inadequate response to exogenous testosterone therapy and were raised as males. PATIENTS AND METHODS: Patients with micropenis who had an equivocal response to exogenous testosterone therapy and were raised as males were evaluated at adulthood (>17 yr). RESULTS AND CONCLUSIONS: Twenty patients with micropenis, initial median SPL -3.3 SD below the mean (range -5.5 to -2.6) had a suboptimal response to initial testosterone therapy, median SPL post-treatment -2.7 SD (-3.3 to -2.2), and were raised as males. At adulthood, 90% (18/20) had a micropenis, median SPL -3.4 (-5.9 to -2.2). All have a male gender identity, five are undergoing psychiatric counseling (fear of sexual rejection--five patients, one of whom also has suicidal ideation). Eight have not pursued a sexual relationship; 12 are sexually active, one of whom is bisexual.

Ureterocystoplasty: indications for a successful augmentation.

PURPOSE: We evaluate the outcome of ureterocystoplasty based on preoperative evaluations. MATERIALS AND METHODS: We reviewed preoperative ultrasound, voiding cystourethrography and preoperative/postoperative urodynamic studies (UDS) in 64 patients undergoing ureterocystoplasty. RESULTS: Augmentation was performed with the distal 5 to 8 cm of a single megaureter in 8 patients without and 16 with grade 4 to 5/5 reflux. Median gain or loss in capacity and compliance was +0.14-fold and -0.11-fold, respectively. Re-augmentation has occurred or is pending in 23 cases (92%). Augmentation was performed in 40 patients with either a complete single or double collecting system. In 9 patients without reflux the diameter of the augmenting system was directly related to success. None of 6 with a ureteral diameter of greater than 1.5 cm required re-augmentation (median increase in bladder capacity and compliance 6 and 50-fold, respectively). Ureterocystoplasty was inadequate in 3 patients with a ureteral diameter of less than 1.5 cm and re-augmentation was required. In 31 patients with reflux, preoperative UDS of the entire system was beneficial. If the system had either normal or mild noncompliance (greater than 20 ml/cm H2O) ureterocystoplasty improved compliance 1-fold (6 cases) and re-augmentation not required. If UDS showed moderately or severely noncompliant system (less than 20 ml/cm H2O, 26 cases) ureterocystoplasty increased capacity and compliance by 0.4-fold (40%) and 0.25-fold (25%), respectively. Re-augmentation has occurred or is pending in 21 of 26 cases (81%). CONCLUSIONS: Ureterocystoplasty with any single or double collecting system is warranted in patients without reflux and a ureteral width greater than 1.5 cm, and in patients with reflux and mild noncompliance (greater than 20 ml/cm H2O) on UDS.

Management of urinary incontinence and nocturnal enuresis in attention-deficit hyperactivity disorder.

PURPOSE: We sought to determine whether attention-deficit hyperactivity disorder (ADHD) influences the resolution of urinary incontinence (UI, or diurnal and nocturnal wetness) and monosymptomatic nocturnal enuresis (NE). MATERIALS AND METHODS: We performed a retrospective review of patients with ADHD, UI and NE. Individuals with UI were treated with timed voiding, and anticholinergics were added only after timed voiding failed. Patients with NE were treated with either an enuretic alarm, desmopressin or imipramine. Statistical comparisons used a control population matched for age, sex, IQ, and urinary and gastrointestinal symptoms. RESULTS: The presence of ADHD had a negative effect on the resolution of incontinence, with 68% of the patients with ADHD becoming continent compared to 91% of controls (p <0.01). Two factors impact the resolution of wetness in patients with ADHD-treatment noncompliance and IQ. Treatment noncompliance was found in 48% of the patients with ADHD compared to 14% of controls (p <0.01). The IQ of patients with ADHD affected success, with 32% of children with an IQ of less than 84 achieving continence compared to 80% of those with an IQ of 84 or greater (p <0.01). Patients with ADHD and NE responded similarly to controls when using desmopressin and imipramine. However, they were less likely to exhibit a durable response following management with an enuretic alarm (19% vs 66%, p <0.01). CONCLUSIONS: Treatment of urinary incontinence in children with ADHD is impaired compared to those without ADHD, and is directly affected by compliance and IQ.

p53 immunopositivity in histologically favorable Wilms tumor is not related to stage at presentation or to biological aggression.

PURPOSE: Previous studies have suggested that increased p53 expression is associated with advanced stage and biologically aggressive (chemotherapy resistant) Wilms tumors. We decided to test the hypothesis that increased immunopositivity of p53 is associated with biological aggressiveness in patients with histologically favorable Wilms tumors. MATERIALS AND METHODS: We reviewed the charts of all patients with unilateral Wilms tumor treated at our institution between 1976 and 2001. Histological characteristics, tumor stage, clinical course and p53 expression as determined by immunohistochemical analysis were determined. All immunohistological evaluations were performed on tissue obtained before administration of chemotherapy. RESULTS: A total of 63 cases of unilateral histologically favorable Wilms tumor were assessed. Five cases (8%) were p53 positive. No significant relationship to p53 expression or stage at presentation was noted in 1 of 21 (5%) stage 1, 3 of 21 (14%) stage 2, 1 of 11 (9%) stage 3 and 0 of 10 stage 4 tumors positive for up-regulation of p53. Of the 5 patients with up-regulated p53 expression 1 (20%) had documented disease progression or relapse while on standard National Wilms Tumor Study chemotherapy. Of the 58 patients who were p53 negative 10 (17%) had disease progression or relapse while on standard National Wilms Tumor Study chemotherapy (p >0.3). CONCLUSION: In contrast to previously published studies, we found no correlation of p53 expression to either tumor stage at presentation (p >0.3) or prognosis (p >0.3) in individuals with histologically favorable Wilms tumor assessed for immunopositivity before administration of chemotherapy.

Is endoscopic decompression of the neonatal extravesical upper pole ureterocele necessary for prevention of urinary tract infections or bladder neck obstruction?

PURPOSE: It has been hypothesized that endoscopic decompression of the duplex extravesical ureterocele is necessary to prevent the complications of urinary tract infections and progressive hydronephrosis. This study was performed to test this premise. MATERIALS AND METHODS: Infants younger than 2 weeks with an extravesical ureterocele associated with a duplex upper pole moiety were assigned to immediate endoscopic puncture of the ureterocele followed by antibiotic prophylaxis or antibiotic prophylaxis with plans for delayed surgical intervention. Radiographic studies and catheterized urine cultures were obtained at ages 3 and 6 months and for fever greater than 38.5C. All patients included in this study were followed through 6 months of life. RESULTS: Of the patients 32 underwent endoscopic puncture of the ureterocele. Median patient age at endoscopy was 5 days (range 3 to 13). During the first 6 months of life complications developed in 4 (12%), including febrile urinary tract infections in 3 (9%) and with progressive hydronephrosis due to incomplete puncture of the ureterocele in 1 (3%). The remaining 40 patients were treated with antibiotic prophylaxis and delayed open surgery. Median time to open surgery was 3 months (range 2 to 6). During the first 6 months of life complications developed in 5 (13%), including 3 (8%) febrile urinary tract infections and progressive hydronephrosis in 2 (5%). No statistical difference was noted between the 2 treatment groups. CONCLUSIONS: In patients with extravesical duplex ureteroceles neonatal complications of urinary tract infection and progressive hydronephrosis are not significantly different between those treated with immediate endoscopic decompression versus delayed open surgical intervention.

Electron beam computerized tomography assessment of in vivo single kidney glomerular filtration rate and tubular dynamics during chronic partial unilateral ureteral obstruction in the pig.

PURPOSE: The assessment of hydronephrosis due to chronic partial ureteral obstruction is controversial. We determined whether a new radiographic technique for assessing kidney function, electron beam computerized tomography (CT), can detect altered renal physiology due to chronic partial ureteral obstruction. We also compared and contrasted electron beam CT with standard well tempered diuretic mercaptoacetyltriglycine (MAG-3) urography. MATERIALS ANDS METHODS: Six pigs underwent creation of unilateral partial ureteral occlusion or sham operation. Three weeks after surgery diuretic enhanced MAG-3 renal scan was done and 48 hours later contrast enhanced electron beam CT was performed. RESULTS: Mean differential function plus or minus standard error of mean of the obstructed kidney was 5.6% +/- 2.4% on MAG-3 renography. In contrast, electron beam CT revealed significantly preserved mean renal function at 24.5% +/- 2.7% (p <0.01). Electron beam CT analysis of tubular function revealed persistent glomerular filtration and filtrate flow through the proximal tubules and loop of Henle with a selective decrease in distal tubular flow, which were findings suggestive of proximal tubular sparing that were not demonstrated by nuclear renography. CONCLUSIONS: Renal function on MAG-3 renography is primarily determined by measuring kidney perfusion and tubular secretion of the isotope. In contrast, electron beam CT determines renal function via quantifying the in vivo single kidney glomerular filtration rate and by assessing renal tubular function. This study documents that electron beam CT of differential renal function is significantly different from that of MAG-3 renography. To our knowledge which of these 2 radiographic studies is most clinically applicable is unknown to date.