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Summary Introduction Infants with myelomeningocele are at risk for chronic kidney disease caused by neurogenic bladder dysfunction. Urodynamic evaluation plays a key role to risk stratify individuals for renal deterioration. Objective To present baseline urodynamic findings from the Urologic Management to Preserve Initial Renal function for young children with spina bifida (UMPIRE) protocol, to present the process that showed inadequacies of our original classification scheme, and to propose a refined definition of bladder hostility and categorization. Study design The UMPIRE protocol follows a cohort of newborns with myelomeningocele at nine children's hospitals in the United States. Infants are started on clean intermittent catheterization shortly after birth. If residual volumes are low and there is no or mild hydronephrosis, catheterization is discontinued. Baseline urodynamics are obtained at or before 3 months of age to determine further management. Based on protocol-specific definitions, urodynamic studies were reviewed by the clinical site in addition to a central review team; and if necessary, by all site urologists to achieve 100% concurrence. Results We reviewed 157 newborn urodynamic studies performed between May 2015 and September 2017. Of these 157 infants, 54.8% were boys (86/157). Myelomeningocele closure was performed in-utero in 18.4% (29/157) and postnatally in 81.5% (128/157) of newborns. After primary review, reviewers agreed on overall bladder categorization in 50% (79/157) of studies. Concurrence ultimately reached 100% with further standardization of interpretation. We found that it was not possible to reliably differentiate a bladder contraction due to detrusor overactivity from a volitional voiding contraction in an infant. We revised our categorization system to group the “normal” and “safe” categories together as “low risk”. Additionally, diagnosis of detrusor sphincter dyssynergia (DSD) with surface patch electrodes could not be supported by other elements of the urodynamics study. We excluded DSD from our revised high risk category. The final categorizations were high risk in 15% (23/157); intermediate risk in 61% (96/157); and low risk in 24% (38/157). Conclusion We found pitfalls with our original categorization for bladder hostility. Notably, DSD could not be reliably measured with surface patch of electrodes. The effect of this change on future renal outcomes remains to be defined. Download : Download high-res image (392KB) Download : Download full-size image summary figure . Change in urodynamic bladder categorization.

The Mirena intrauterine device (IUD) is a hormone-secreting contraceptive device. Pregnancy with the Mirena is rare and effects to the fetus are unknown. Here we present four females with genital virilization after pregnancy with persistent Mirena IUD. All patients had a 46, XX karyotype and normal hormone evaluation. All underwent exam under anesthesia, demonstrating posterior labial fusion and short urogenital sinus with normal bladder, urethra, vagina, and cervix. Three of four patients underwent flap vaginoplasty without complications and good cosmetic outcomes. This series suggests that persistent levonorgestrel-secreting IUD during pregnancy is associated with genital virilization in female fetuses.

Summary Introduction Excision of the prostatic utricle has been a challenging surgical problem due to the location deep in the pelvis between the rectum and bladder. Robotic-assisted laparoscopic surgery allows minimally invasive access to this location. Objective To describe the robotic surgical outcomes and important techniques associated with robotic excision of the prostatic utricle and explain how these techniques apply to similar pediatric pelvic pathology. Study design Retrospective chart evaluation of patients treated at a single institution with robotic excision of a prostatic utricle as well as two other patients in which the similar approach was employed. Results 4 patients underwent robotic excision of a prostatic utricle. A fifth patient underwent excision of urethral diverticulum that was the remnant of an ectopic ureter. The sixth patient had a high urogenital sinus that required laparoscopic assisted vaginoplasty approached in a similar manner to the above listed cases. Mean age at surgery was 35.5 months for the utricle patients. Mean follow-up duration for the utricle patients was 30.75 months. Average hospital stay for the utricle patients was one day. In the utricle patients one patient developed transient urinary retention and one had a postoperative urinary tract infection. Concomitant cystoscopy used in the two non-utricle patients was a key step in defining the proper location of dissection. Discussion Robotic-assisted laparoscopic retrovesical excision of the prostatic utricle is a safe operation that requires a skill set that can be readily applied to other complex pelvic pathology such as the ectopic ureter, urethral diverticulum, and urogenital sinus. Concomitant cystoscopy can aid significantly in defining the location and limits of dissection.

Care of children with spina bifida has significantly advanced in the last half century, resulting in gains in longevity and quality of life for affected children and caregivers. Bladder dysfunction is the norm in patients with spina bifida and may result in infection, renal scarring and chronic kidney disease. However, the optimal urological management for spina bifida related bladder dysfunction is unknown.In 2012 the Centers for Disease Control and Prevention convened a working group composed of pediatric urologists, nephrologists, epidemiologists, methodologists, community advocates and Centers for Disease Control and Prevention personnel to develop a protocol to optimize urological care of children with spina bifida from the newborn period through age 5 years.An iterative quality improvement protocol was selected. In this model participating institutions agree to prospectively treat all newborns with spina bifida using a single consensus based protocol. During the 5-year study period outcomes will be routinely assessed and the protocol adjusted as needed to optimize patient and process outcomes. Primary study outcomes include urinary tract infections, renal scarring, renal function and bladder characteristics. The protocol specifies the timing and use of testing (eg ultrasonography, urodynamics) and interventions (eg intermittent catheterization, prophylactic antibiotics, antimuscarinic medications). Starting in 2014 the Centers for Disease Control and Prevention began funding 9 study sites to implement and evaluate the protocol.The Centers for Disease Control and Prevention Urologic and Renal Protocol for the Newborn and Young Child with Spina Bifida began accruing patients in 2015. Assessment in the first 5 years will focus on urinary tract infections, renal function, renal scarring and clinical process improvements.

We previously developed a computational model to predict vesicoureteral reflux resolution 1 and 2 years after diagnosis. Previous studies suggest that an abnormal renal scan may be a predictor of the failure of vesicoureteral reflux to resolve. We investigated whether the addition of renal scan data would improve the accuracy of our computational model.Medical records and renal scans were reviewed on 161 children, including 127 girls and 34 boys, with primary reflux between 1988 and 2004. In addition to the 9 input variables from our prior model, we added renal scan data on decreased relative renal function (40% or less in the refluxing kidney) and renal scars. Resolution outcome was evaluated 1 and 2 years after diagnosis. Data sets were prepared for 1 and 2-year outcomes, and randomized into a modeling set of 111 and a cross-validation set of 50. The model was constructed using neUROn++.A logistic regression model had the best fit with an ROC area of 0.945 for predicting reflux resolution in the 2-year model. This was improved compared to our previous model without renal scan data. A prognostic calculator using this model can be deployed for availability on the Internet, allowing input variables to be entered and calculating the odds of resolution.This computational model uses multiple variables, including renal scan data, to improve individualized prediction of early reflux resolution with almost 95% accuracy. The prognostic calculator is a useful tool for predicting individualized vesicoureteral reflux resolution.

Questions regarding the efficacy and safety of long-term prophylactic antibiotics and the option of minimally invasive therapy have increased clinical emphasis on predicting early resolution in patients with vesicoureteral reflux. We reviewed multiple potential prognostic factors in addition to grade to enhance predictive ability regarding early vesicoureteral reflux resolution.Medical records and cystograms from 324 children (257 girls and 67 boys) with primary vesicoureteral reflux were reviewed. Multiple factors were analyzed with respect to outcome and multivariable dependent tables were constructed to enhance the prediction of vesicoureteral reflux resolution.Mean +/- SD age at diagnosis was 2.3 +/- 2.1 years and followup was 1.7 +/- 2.8 years. Reflux grade was significantly associated with resolution (p0.001). Multivariate analysis stratifying by grade demonstrated that a bladder volume at reflux onset on the initial cystogram of greater than 50% of predicted bladder capacity (p0.001), age younger than 2 years at diagnosis (p = 0.003) and history of prenatal hydronephrosis (p0.001) were significant factors predicting resolution within 2 years. Multivariable tables using age, bladder volume at reflux onset and grade demonstrated that children younger than 2 years at diagnosis and volume at onset greater than 50% of predicted bladder capacity had a higher early resolution rate for grades 2 (p = 0.012) and 3 (p0.001) reflux.Initial reflux grade, bladder volume at reflux onset, age at diagnosis and history of prenatal hydronephrosis were shown to be independent factors affecting the resolution rates of vesicoureteral reflux. The multivariable tables permit improved individualized prediction of resolution in patients with grades 2 and 3 reflux.

Minimally invasive treatment options and concern regarding long-term antibiotics have increased emphasis on predicting the chance of early vesicoureteral reflux resolution. Computational models, such as artificial neural networks, have been used to assist decision making in the clinical setting using complex numeric constructs to solve multivariable problems. We investigated various computational models to enhance the prediction of vesicoureteral reflux resolution.We reviewed the records of 205 children with vesicoureteral reflux, including 163 females and 42 males. In addition to reflux grade, several clinical variables were recorded from the diagnostic visit. Outcome was noted as resolved or unresolved at 1 and 2 years after diagnosis. Two separate data sets were prepared for the 1 and 2-year outcomes, sharing the same input features. The data sets were randomized into a modeling set of 155 and a cross-validation set of 50. The model was constructed with several constructs using neUROn++, a set of C++ programs that we developed, to best fit the data.A linear support vector machine was found to have the highest accuracy with a test set ROC curve area of 0.819 and 0.86 for the 1 and 2-year models, respectively. The model was deployed in JavaScript for ready availability on the Internet, allowing all input variables to be entered and calculating the odds of 1 and 2-year resolution.This computational model allowed the use of multiple variables to improve the individualized prediction of early reflux resolution. This is a potentially useful clinical tool regarding treatment decisions for vesicoureteral reflux.

Objectives To analyze the utility of assessing degree of constipation by abdominal radiograph (KUB) in relation to symptoms and urodynamic data in children with dysfunctional elimination. Methods A retrospective review of children with concomitant constipation and daytime incontinence was performed. Inclusion required at least two consecutive visits with KUB and noninvasive uroflowmetry. Patients were excluded for anticholinergic medication use or neurogenic or anatomic abnormalities. Rectal fecal quantification and presence of stool throughout the colon was assessed on KUB and categorized as “empty,” “normal amount of stool,” or “fecal distention of rectum (FDR).” Results Twenty-six patients met inclusion requirements (6 boys, 20 girls; average age, 7.7 ± 2.2 years). The average time between the initial and subsequent visit was 12.5 ± 7.8 weeks. Initial KUB revealed FDR in 17. No statistical significance was found between FDR on initial or final KUB and outcome of wetting symptoms, nor could a relationship between FDR uroflow parameters at either visit be demonstrated. Conclusions No correlation between any uroflowmetry parameter and the presence of FDR at the initial or final visits could be demonstrated. Similarly, no statistical significance between FDR on final or initial KUB and outcome of wetting symptoms was established.

OBJECTIVES The bladder pressure at the onset of reflux may reflect the competence of the ureterovesical junction. The predictive value of the bladder pressure at the onset of vesicoureteral reflux, as measured by nuclear cystometrography, was assessed relative to subsequent reflux resolution. METHODS Nuclear cystometrograms of 67 children were reviewed to determine the presence of reflux, bladder pressure at the onset of reflux, and bladder volume at the onset of reflux. The data were analyzed for significant relationships between the nuclear cystometrogram results and patient outcomes using a Cox proportional hazards regression model, controlling for the duration of follow-up and for patients with bilateral reflux. RESULTS Data were gathered for reflux occurring in 85 ureters. As expected, reflux of a greater grade was less likely to resolve spontaneously (P = 0.005). The pressure at the onset of reflux was also highly predictive of spontaneous resolution (P = 0.0005). Reflux occurring at greater pressures was more likely to resolve spontaneously, independent of the reflux grade or bladder volume at the onset of reflux. Patient age (P = 0.45), normalized volume of urine refluxed (P = 0.97), and normalized bladder volume at the onset of reflux (P = 0.18) were not significant predictors of reflux resolution. CONCLUSIONS The findings of this report have demonstrated for the first time, to our knowledge, that the bladder pressure at the onset of vesicoureteral reflux, as determined by nuclear cystometrography, is a significant independent predictor of vesicoureteral reflux resolution in children.

The treatment of vesicoureteral reflux has evolved over the past half-century from primarily surgical to nonoperative treatments. Although the benefits of surgical correction versus medical management are debated, the surgical techniques that have evolved are highly effective in correcting vesicoureteral reflux. Recently, the US Food and Drug Administration has approved an injectable implant of dextranomer/hyaluronic acid copolymer for use in children, making the endoscopic treatment of reflux a potential alternative to open surgical correction. This article reviews the different surgical techniques,postoperative management, and complications, as well as the various implants used in the endoscopic correction of vesicoureteral reflux and their outcomes.

Reflux grade predicts resolution and influences followup and treatment. Bladder pressure at the onset of reflux may reflect the ureterovesical junction competence. By combining simultaneous cystometry with nuclear cystography (nuclear cystometrogram) we determined bladder pressure at the onset of reflux.Nuclear cystometrograms were performed in 40 children to determine bladder pressure at the onset of reflux, reflux volume and bladder capacity at the onset of reflux. The effect of bladder pressure and capacity at the onset of reflux was assessed with a reflux pressure volume index.A weak inverse correlation existed between bladder pressure at the onset of reflux and a normalized volume of reflux (r = -0.32, p = 0.018). A stronger correlation existed between reflux pressure and bladder volume at reflux (r = 0.47, p = 0.0002). An inverse correlation of the reflux pressure-volume index and reflux volume was significant (r = -0.49, p = 0.0002) as was the correlation between bladder volume at reflux and amount of reflux (r = -0.47, p = 0.0003). The reflux pressure-volume index for patients with persistent reflux at followup (median 1.89) was significantly smaller than that for those without reflux (median = 8.71, p = 0.02). A difference existed in normalized bladder volume at reflux between resolved and persistent reflux (p = 0.003). No difference was detected with respect to reflux volume and resolution (p = 0.738).A nuclear cystometrogram is no more invasive than a standard cystogram and permits determination of bladder pressure at the onset of reflux. Factors such as reflux pressure and bladder volume at reflux provide additional characterization of the ureterovesical junction and may help determine the prognosis for reflux resolution.

A duplex kidney associated with a poorly functioning upper-pole segment is commonly associated with incontinence, voiding dysfunction, and urinary tract infections. A standard treatment option for this condition is upper-pole heminephrectomy. With the continued development of minimally invasive urology, this technique can now be safely performed laparoscopically. This report details step by step our technique of laparoscopic upper-pole heminephrectomy. Key points include placement of a catheter in the normal ureter at the start of the case, full mobilization of the upper-pole ureter away from the renal hilum, and precise identification of the vasculature supplying the upper pole. Laparoscopic upper-pole heminephrectomy for ectopic ureter is safe and reproducible and offers the patient the typical postoperative benefits of laparoscopic surgery.

In pre-clinical gene therapy studies of bladder cancer there is tremendous variation in the ability of viral vectors to deliver genetic material to bladder epithelium. Possible explanations for this variability may involve the physical parameters of delivering vectors in these experimental models. We examined the effects of intravesical volume and pressure during instillation as well as chemical modification of the bladder epithelium on subsequent gene expression in the bladder in mice.Female C57B1/6 mice underwent intravesical instillation of the replication restricted canarypox virus (ALVAC) recombinant for the reporter genes luciferase or beta-galactosidase. Similar viral titers were instilled at different volumes and a pressure transducer measured intravesical pressure when the vector was instilled. Also, various agents, including 0.6 N hydrochloric acid, 0.4% oxychlorosene, poly-L-lysine and 0.25 M. ammonium chloride, were used to modify the bladder surface before vector instillation and then assayed for transgene expression.As expected, maximum intravesical pressure measured during instillation was significantly greater in mice instilled with a higher volume (33.1 versus 9.8 mm. Hg). Significantly more gene expression was detected in bladders instilled with a higher volume of viral vectors (p0.05). Likewise, higher instillation pressures resulted in higher transgene expression in distant organs. Modification of the bladder epithelium with agents such as oxychlorosene and poly-L-lysine resulted in elevated gene expression with only minimal increases in systemic activity.Significant differences in gene expression are achieved by varying physical parameters during intravesical instillation. Increased gene expression associated with larger volume instillation may be responsible for some reported variability of gene transfer to the bladder. Alternate manipulations, such as modifying the bladder surface, may be done to enhance gene transfer to the urothelium without increasing systemic distribution.

In children with severe unilateral reflux nephropathy and diminished relative renal function (RRF) a dilemma exists between the choice of treatment with ureteral reimplantation or nephrectomy. Limited followup data are available regarding relative renal function or postoperative complications after ureteral reimplantation in kidneys with significant unilateral reflux nephropathy.We retrospectively reviewed the records of 460 patients who underwent ureteral reimplantation between 1980 and 2002, and identified children with primary vesicoureteral reflux and severe unilateral reflux nephropathy (RRF 30% or less on renal scintigraphy). The postoperative outcomes were assessed for relative renal function and complications including hypertension, pyelonephritis or persistent reflux.A total of 18 girls and 14 boys with a mean preoperative relative renal function of 20.1 +/- 7.8% (range 2% to 30%) met the inclusion criteria. Reflux grade in the poorly functioning kidney was II in 4 children (13%), III in 14 (44%), IV in 11 (34%) and V in 3 (9%). Reflux was unilateral in 15 children (47%) and bilateral in 17 (53%). Mean followup from surgery was 3.7 years (range 0.3 to 12.9). In 28 children with both preoperative and postoperative renal scans, mean preoperative RRF was 20.3 +/- 7.4% and mean postoperative RRF was 20.5 +/- 8.6% with a mean time between renal scans of 2.3 years. No statistically significant change was noted from preoperative to postoperative relative renal function with a mean change of +0.2 +/- 3.7% (range -6.5% to +10%, p=0.82). Postoperative complications occurred in 7 of the 32 children (22%), including hypertension (1), pyelonephritis (3) and persistent reflux (4). Pyelonephritis occurred in 1 child with persistent reflux. No statistically significant difference existed in mean preoperative relative renal function between those with and without complications (24.6 +/- 8.9% vs 18.8 +/- 7.2%, p = 0.09).Children who underwent ureteral reimplantation in association with unilateral reflux nephropathy maintained stable relative renal function.

Purpose: We compared 2 treatment modalities (sling cystourethropexy and periurethral collagen injection) in patients with intrinsic sphincter deficiency alone or with urethral hypermobility (combined stress urinary incontinence).Materials and Methods: We retrospectively reviewed a series of 50 consecutive patients treated surgically for intrinsic sphincter deficiency during a 2-year period. All patients were evaluated by history and physical examination to assess urethral hypermobility and urodynamic testing. Intrinsic sphincter deficiency was assessed by abdominal leak point pressure and video urodynamics. Of the 50 patients 28 underwent a pubovaginal sling operation and 22 received a periurethral injection of collagen.Results: Of the patients studied 40 percent had combined stress urinary incontinence. A pubovaginal sling procedure resulted in a cure rate of 81 percent in this group, compared to 25 percent for periurethral injection of collagen.Conclusions: A subgroup of women exists with combin...

Vesicoureteral reflux (VUR) grading may be difficult when discrepancies exist between the degree of dilation of the pyelocalyceal system and the ureter. Resolution may be more accurately predicted by the appearance of the distal ureter. We analyzed a novel, objective method of evaluating VUR based on the diameter of the distal ureter.Seventy-nine voiding cystourethrograms were reviewed (18 boys; 61 girls; aged 1 month to 7.5 years). The largest ureteral diameter within the false pelvis was measured and normalized by dividing by the distance from the L1-L3 vertebral body to give the distal ureteral diameter: L1-L3 ratio (UDR). Clinical outcome was defined as spontaneous resolution or surgical correction.A significant association between grade and UDR existed (p0.0001). Mean UDR was significantly greater in those who underwent surgical correction (0.34 ± 0.02 vs 0.18 ± 0.02; p0.0001). Logistic regression analysis demonstrated a significant association of UDR with outcome controlling for grade (p = 0.001). Grade effect on outcome when controlling for UDR was not significant (p = 0.76). Odds ratio for surgical correction corresponding to a 0.1 increase in UDR equaled 2.25 (95% CI: 1.39, 3.64).UDR provides an objective measurement of VUR and appears more predictive of clinical outcome than grade in this series.

There is concern that bladder augmentation with bowel segments predisposes toward the development of carcinoma. Additionally, patients with neurogenic bladder and bladder cancer often present with advanced stage and have poor survival. Cellular hyperproliferation at the urointestinal junction (UIJ) has been implicated in this scenario. We aimed to develop a reproducible murine model of ileocystoplasty (ICP). We also performed preliminary analysis of any early histologic changes with focus on cellular proliferation at the UIJ. Fifteen 6- to 8-week-old female C57BL/6 mice underwent ICP, where a 1-cm ileal segment was used for bladder augmentation. Four sham mice underwent cystotomy and closure, and four mice did not undergo surgery. The mice were euthanized at 12 weeks postsurgery, and paraffin sections were stained for hematoxylin and eosin (H&E). Cellular proliferation was investigated using Ki-67. A novel model of ICP in mice was developed and demonstrated to be technically feasible in approximately 60 min under the operating microscope. Twelve-week postsurgical survival rates were 80% (12 of 15). The surviving mice had a similar weight gain as the sham mice. H&E sections showed thickened urothelium (six to 10 cell layers) at the UIJ, but sparse mitotic figures and no dysplastic changes. Ki-67 staining was rare in the urothelium, and showed no differences between the sham and ICP mice in the bladder or at the UIJ. We here demonstrate the first murine model of ICP. Preliminary studies did not show evidence of early hyperproliferation at the UIJ or in the bladder, but further long-term studies as well as studies with transgenic mice are warranted.

Objective In a group of children diagnosed with vesicoureteral reflux (VUR) we evaluated renal ultrasound findings, associated findings on renal scan, and prognostic impact on VUR resolution. Methods Medical records were reviewed for children with primary reflux and no history of antenatal hydronephrosis who underwent an initial renal ultrasound. Abnormal renal ultrasound was defined as hydronephrosis or relative difference in renal size ≥1 cm. Reflux resolution was evaluated at 2 years post diagnosis. Results In 129 children with VUR (111 girls, 18 boys), 39 (30%) had an abnormal renal ultrasound. Two-year VUR resolution in the abnormal renal ultrasound group was 21% versus 46% in the group with normal renal ultrasound (P = 0.01). Combining grade II and III reflux, an abnormal ultrasound was associated with a statistically significantly lower resolution rate (grade II–III 23% vs 47%, P = 0.049). For children with moderate hydronephrosis, 8/9 (89%) had abnormal initial renal scans and all failed to achieve resolution of reflux at 2 years. Conclusions In this cohort of children with VUR, 30% had abnormalities on renal ultrasound. The presence of moderate hydronephrosis on ultrasound may indicate a high likelihood of abnormality on renal scan and failure to achieve early resolution of VUR.

Limited studies suggest a relationship between scarring on renal scan and failure to resolve vesicoureteral reflux. We evaluated the impact of abnormal renal scans on early vesicoureteral reflux resolution.The medical records and renal scans were reviewed of children diagnosed with primary reflux between 1988 and 2004. We defined an abnormal renal scan as renal scarring or relative renal function 40% or less. Reflux resolution was noted 1 and 2 years after diagnosis.Renal scan data were available on 161 children with vesicoureteral reflux, including 127 girls and 34 boys. Relative renal function was 15% or less in 7 children, 16% to 35% in 14, 36% to 40% in 18 and greater than 40% in 122. Of the 161 patients 79 (43%) had an abnormal renal scan, including 37% with grades 1 to 3 reflux. The rate of 2-year reflux resolution in the abnormal and normal renal scan groups was 13% vs 53%. Of children with grades II and III reflux those with an abnormal renal scan were less likely to have reflux resolution compared to those with normal renal scans (23% vs 55% and 4% vs 41, respectively, p0.05). The same relationship was present at 1 year for grades 2 and 3 (18% vs 49% and 4% vs 30, respectively, p0.05).Abnormal renal scans are an important independent predictor of early failure to resolve vesicoureteral reflux. An abnormal renal scan should be considered when counseling families about the likelihood of early reflux resolution. Performing a renal scan may be indicated in select patients.

Patients with neurogenic bladder dysfunction due to spina bifida have been reported to be at increased risk for bladder cancer. Recent publications suggest that bladder augmentation is also a significant risk factor. We reviewed our experience with treating patients with spina bifida and bladder cancer.Patients with spina bifida treated for bladder cancer between 1995 and 2005 were identified. Patient demographics, mode of bladder management, risk factors and presenting symptoms were recorded along with therapy, pathological findings and outcome. This patient cohort was combined with all prior known published studies for analysis.Eight patients with a median age of 41 years were treated. Only 1 patient (13%) had undergone bladder augmentation. Locally advanced stage (T3 or greater) or lymph node metastases were present in 88% of cases. Median survival was 6 months with only 1 patient alive with no evidence of recurrence at 20 months. A total of 11 prior published cases were identified and combined with this series. Transitional cell carcinoma was present in 58% of patients. Median survival was 6 months. Only 37% of patients had undergone bladder augmentation.Patients with spina bifida and bladder cancer present at a young age with variable tumor histology and advanced stage, and they have poor survival. Presenting symptoms are often atypical and bladder cancer should be a consideration in this patient population, even in young adults. Due to poor survival further study is warranted in this population to determine whether screening would be beneficial for earlier detection and improved outcomes.

Objective To describe a unique vaginal outlet obstruction and its ultrasound-assisted surgical correction. Design Case report. Setting An academic medical center. Patient(s) A 12-year-old girl was seen with cyclic abdominal pain, nausea, and an abdominal mass. External genital examination revealed no vaginal opening or dimple. Transabdominal ultrasound revealed a large hematometrocolpos 5 cm proximal to the perineum, with an otherwise normal-appearing uterus and ovaries. Intervention(s) Surgical treatment included perineal incision, creation of a 5-cm passage through connective tissue, drainage of the hematometrocolpos, and mobilization of the proximal vagina, allowing for pull-through vaginoplasty. Ultrasound guidance was used throughout the surgery and allowed for a safe transperineal approach. A vaginal form was placed in the neovagina. Main Outcome Measure(s) Follow-up clinical evaluation and pelvic ultrasonography. Result(s) A well-healed perineum and patent 5-cm–long vagina were observed at 4-month follow-up. The patient reported three regular menses. Postoperative pelvic ultrasound scan was normal. Conclusion(s) We describe a unique case of isolated distal vaginal agenesis of significant length that was successfully treated via a perineal approach with the intra-operative assistance of ultrasound guidance.

We report the incidence of calcifications developing at the bladder neck/urethra in pediatric patients treated with glutaraldehyde cross-linked collagen for urinary incontinence.We reviewed charts of patients treated with glutaraldehyde cross-linked collagen injections for urinary incontinence between 1994 and 1999. Etiology of incontinence, pertinent medical history, operative details and postoperative imaging were examined.Of 31 patients 4 (13%) had development of submucosal calcifications in the bladder neck/urethra. All 4 patients had received multiple injections of glutaraldehyde cross-linked collagen for incontinence secondary to neurogenic bladder. The calcifications were confirmed surgically. Pathology reports available for 2 of 4 patients showed chronic inflammation without dysplasia or malignant changes. Mean followup was significantly different between calcified and noncalcified cases (10.3 vs 7.2 years, p = 0.009), as was total volume of collagen injected (21 vs 12 cc, p = 0.012). Mean time to diagnosis of calcifications was 8.8 years (range 7 to 11) after first injection. A total of 24 patients without calcification underwent bladder imaging at a mean of 6.8 years (+/-2.2) after glutaraldehyde cross-linked collagen injection, which was not significantly different than the time to diagnosis (p = 0.089). The number of injections was not significantly different between the 2 groups (p = 0.426).Of our patients 13% had development of calcifications at the site of prior glutaraldehyde cross-linked collagen injections for incontinence. These calcifications were surrounded by chronic inflammation. Patients who have undergone glutaraldehyde cross-linked collagen injections may benefit from long-term followup with bladder imaging to detect and follow calcifications at prior injection sites.

Despite the acute onset, partial bladder outlet obstruction in the rabbit induces detrusor remodeling similar to that in men with benign prostatic hyperplasia in terms of its impact on structural and functional alterations in smooth muscle. We determined if partial bladder outlet obstruction induced remodeling alters the protein kinase C signaling pathway that leads to contraction.Smooth muscle from control animals and those subjected to 2 weeks of partial bladder outlet obstruction were mounted for isometric force recording, measurement of myosin light chain phosphorylation and levels of adducin phosphorylation. Bladder muscle strips were stimulated by phorbol dibutyrate or carbachol in the presence and absence of bisindolylmaleimide-1.Smooth muscle strips from animals subjected to partial bladder outlet obstruction showed little to no increase in stress in response to phorbol dibutyrate and no increase in myosin light chain phosphorylation levels. Muscle strips from control animals produced a robust contraction with concomitant increases in myosin light chain phosphorylation. Inhibition of protein kinase C by bisindolylmaleimide-1 significantly depressed carbachol induced contractions of muscle strips from control animals but it had no effect on carbachol induced contractions of muscle strips from outlet obstructed animals. Phorbol dibutyrate increased phospho-adducin levels in muscle strips from the 2 animal sources, suggesting that protein kinase C could be activated.We propose that partial bladder outlet obstruction does not alter protein kinase C activation, but rather abolishes or uncouples the pathway(s) downstream of protein kinase C, leading to contraction. Loss of this pathway may contribute to the loss of normal voiding behavior and the resultant decompensated state.

Reflux grade is the factor most commonly used to predict spontaneous reflux resolution. We evaluated other potential predictive factors aside from reflux grade relative to spontaneous resolution.We reviewed the records of 20 males and 98 females who were diagnosed with primary vesicoureteral reflux between ages 0 and 7 years between 1990 and 2000. Age, sex, height, weight, reflux grade, bladder volume at onset of reflux and laterality were recorded for the first, second and most recent voiding cystourethrogram or nuclear cystogram before spontaneous resolution or operative intervention.Of 118 patients 75 (64%) had spontaneous resolution, 27 (23%) underwent corrective surgery and 16 (13%) are still being followed. Average age at diagnosis was 2.3 years (range 1 day to 7.7 years) and average followup was 4.3 years (range 0.2 to 14). Average time to spontaneous resolution was 2.2 years (range 0.5 to 10.3) vs a time to operative treatment of 3.6 years (range 0.2 to 11.2). There was a significantly higher spontaneous resolution rate for lower reflux grades (p = 0.0004). Reflux occurring at greater than 75% of predicted bladder capacity had a significantly higher resolution rate (p = 0.0005). The initial height and weight percentile was not significant for predicting spontaneous resolution. Breakthrough urinary tract infections were negative predictors of spontaneous resolution (p0.0001).In addition to grade, bladder volume relative to predicted bladder capacity at the onset of reflux appears to provide additional prognostic information regarding the likelihood of spontaneous resolution of primary vesicoureteral reflux.

We hypothesized that the calcineurin pathway mediated some of the complex remodeling process that allows a bladder subjected to partial outlet obstruction to adapt to its new workload. Atrial natriuretic factor mRNA expression served as a marker of calcineurin activation.A total of 16 New Zealand White rabbits underwent surgical creation of partial outlet obstruction, followed by randomization to receive cyclosporin A (20 mg/kg intramuscularly twice daily) or no additional treatment for 14 days. Three animals underwent 2 weeks of partial bladder outlet obstruction followed by bladder biopsy and the reversal of obstruction.Atrial natriuretic factor expression was seen only in bladders with severe hypertrophy and it disappeared with the reversal of outlet obstruction. Cyclosporin A treatment resulted in a decrease in atrial natriuretic factor mRNA expression (p0.05) and a marked shift in myosin heavy chain A-to-B ratios toward normal (p0.01) and an increase in smooth muscle cross sectional area (p0.05). Bladder mass decreased 40% but did not attain statistical significance (p = 0.08).The calcineurin pathway has a significant role in bladder wall hypertrophy following partial outlet obstruction. Bladder hypertrophy could not be fully prevented by cyclosporin A, suggesting that multiple signaling pathways are involved in this pathophysiology. The expression of myosin heavy chain AB isoforms is regulated in part by the calcineurin pathway.

Mice with gene deletion or targeted over expression are important for understanding the remodeling that follows partial bladder outlet obstruction (PBOO). This condition predominates in males. We produced PBOO in male mice and now report the physiological, histological and molecular consequences.Male C57bl/6 mice were surgically obstructed or subjected to sham surgery and unoperated mice served as controls. Four weeks later the bladders were excised and their function was assessed with in vitro whole bladder cystometry. The optimum volume for pressure generation was determined and isometric pressures were measured for field stimulation and depolarization with KCl. Bladder hypertrophy was classified as severe-bladder mass greater than 50 mg or mild-bladder mass less than 50 mg. The percent muscle fraction was determined by histological analyses. The expression of C-terminal (SM1 and SM2) and N-terminal (SM-B and SM-A) isoforms of myosin heavy chain was analyzed by reverse transcriptase-polymerase chain reaction.Severely hypertrophied bladders had larger optimum volume (p0.001) and generated less pressure in response to field stimulation (p0.001) and KCl (p0.01) with a slower rate of pressure generation than controls or sham operated mice. Increased SM1-to-SM2 and SM-A-to-SM-B ratios were noted in severely obstructed bladders relative to controls or sham operated mice (p0.05). The muscle fraction decreased slightly in the severely hypertrophied group (p not significant).Our male mouse model of PBOO demonstrates an increase in bladder mass, larger capacity and significantly decreased pressure generation in the in vitro whole bladder model. Obstruction induced increases in the expression of C-terminal (SM1) and N-terminal (SM-A) myosin heavy chain isoforms.

The Starr technique of tapering megaureters was first reported in 1979. Although this method of ureteral plication is well known, to our knowledge there are no clinical studies regarding outcomes. We report the first long-term outcome results with the Starr technique for primary obstructive megaureters.Three girls and 10 boys with a total of 16 primary obstructive megaureters (3 bilateral) underwent reimplantation with Starr plication between 1988 and 2000. Baseline and followup renal function (average followup 6.2 years) was determined with renal scan and/or 24-hour urinary creatinine clearance. Average age at operation was 5.6 years (range 2 months to 13 years). All ureters were plicated using the Starr technique with interrupted polydioxanone sutures performed by a single surgeon (CEH). Reimplantation methods were the Hendren technique in 7 and Politano-Leadbetter in 9.No ureter demonstrated obstruction postoperatively. One ureter refluxed, which resolved with subureteral collagen injection. Seven patients had mean preoperative and postoperative creatinine clearance +/- SD 72.9 +/- 14.8 and 102.1 +/- 10.9 cc per minute, respectively (p0.05). Six patients underwent preoperative and postoperative renal scans. Average preoperative relative renal function on renal scan in the kidney with a megaureter was 53.0% +/- 6.7% (range 37% to 84%). Long-term followup (average 4.1 years) renal scan revealed a relative function of 53.3% +/- 9.2% (range 37% to 100%).Our data demonstrate that Starr plication is a safe procedure that provides long-term stabilization of renal function in the management of primary obstructive megaureter.

Cystic dysplasia is a benign congenital lesion of the rete testis often associated with ipsilateral wolffian duct and ureteral abnormalities. We present a case of cystic dysplasia and associated anomalies in a 12-year-old boy. The workup revealed right testicular cystic dysplasia, ipsilateral renal agenesis, contralateral crossed renal ectopia, and vesicoureteral reflux. We performed right testicular-preserving cyst enucleation and ureteroneocystostomy. No testicular atrophy or new cyst formation was evident at 1 year of follow-up. This is the first reported case of cystic dysplasia associated with ipsilateral renal agenesis and contralateral crossed renal ectopia.

A 24-year-old female underwent prenatal ultrasonography at 19 weeks of gestation, which demonstrated oligohydramnios, bilateral hydronephrosis and a 9 8 5 cm. fluid-filled structure filling the abdomen. Karyotype was 46XX. Percutaneous aspiration drained 420 ml. fluid. Serial aspirations of the fetal urine revealed sodium 88 mg./dl., cloride 78 mg./dl., osmolarity 163 mOsm./l., calcium 4.2 mg./dl., 2-microglobulin 4.3 mg./l., and total protein 30 mg./dl. The mother was referred for possible fetal intervention. Evaluation confirmed bilateral hydronephrosis, a large fluid-filled structure filling the pelvis and abdomen, female external genitalia and clubbed feet. The fetus was thought to have a persistent cloaca with massive hydrocolpos. At 22 weeks of gestation a vesicoamniotic shunt was placed. Followup ultrasound showed decompression of the structure and a normal amniotic fluid volume. Serial studies documented continued decompression of this structure and normal amniotic fluid volume for the next 10 weeks. Then fluid reaccumulated in the dilated mass and amniotic fluid volumes decreased. The fetus was delivered by cesarean section at 39 weeks of gestation and was intubated for respiratory distress. Examination of the abdomen revealed a prune belly appearance. The labia majora were widely separated with a small orifice near the right labia (figs. 1 and 2). The shunt was clogged with mucus. Also noted were bilateral clubbed feet with absence of the fourth and fifth digits on the left foot. Ultrasound revealed a large, fluid-filled structure filling the pelvis, right hydronephrosis and left renal agenesis. Laparotomy demonstrated the distal colon entering the enlarged vagina. There were hemi-uteri and normal appearing fallopian tubes and ovaries. The vagina was septated and communicated with the orifice on the perineum through an atretic channel. End colostomy and vaginostomy were performed. Serum creatine reached a nadir of 0.3 ng./dl. (normal 0.1 to 0.6). The patient required prolonged mechanical ventilation and a tracheostomy was performed at age 1 month. Computerized tomography and a vaginogram confirmed a solitary right kidney, ureteral drainage into the vagina, ureteral reflux and absence of the bladder.