Home Urodynamic Pressures and Volume Measurement for the Neurogenic Bladder: Initial Validation Study.

PURPOSE: We evaluated the ability of a bladder pressure/volume diary to identify patients at risk for increased intravesical pressures. MATERIALS AND METHODS: Patients dependent on clean intermittent catheterization used ruler based manometry to measure intravesical pressures before leakage or scheduled drainage at home. We prospectively collected clinical, urodynamic and bladder pressure/volume diary data in patients with spina bifida who were optimized on anticholinergic therapy and clean intermittent catheterization. Measurements were taken with patients in the supine position with relaxed abdominal muscles. We defined increased pressure as detrusor pressure greater than 30 cm water as measured by urodynamics. ROCs were plotted to correlate bladder pressure/volume diary variables with abnormal intravesical pressures, and the most sensitive variable in determining abnormal intravesical pressures was sought as the end point. RESULTS: A total of 30 patients with a mean age of 10 years (range 1 to 20) were included. Home pressures measured at maximal clean intermittent catheterization volume and mean bladder pressure/volume diary pressures were most reliable in predicting urodynamic pressures greater than 30 cm water (AUC 0.93 and 0.87, respectively). Home pressures measured at maximal clean intermittent catheterization volumes less than 20 cm water were associated with normal bladder pressures (less than 30 cm water) on urodynamics, with a sensitivity of 100% and a specificity of 80%. CONCLUSIONS: Home manometry less than 20 cm water provides a reliable measurement of safe pressures. A bladder pressure/volume diary is feasible and can aid in monitoring pressures at home without the additional cost and morbidity of urodynamics. A bladder pressure/volume diary may be a useful tool to help identify patients who would benefit from urodynamic testing.

Prospective evaluation of Peristeen® transanal irrigation system with the validated neurogenic bowel dysfunction score sheet in the pediatric population.

AIMS: To determine the ability of Peristeen® transanal irrigation system to reduce symptoms of neurogenic bowel dysfunction (NBD) in patients using the validated neurogenic bowel dysfunction scoring system for the pediatric population. METHODS: Patients 3-21 years with NBD whose current bowel program was unsuccessful were given the Neurogenic Bowel Dysfunction (NBoDS) score sheet before initiating Peristeen®, and at 2 weeks, 2 months, and 6 months after. All patients were started on Peristeen® with tap water (20 ml/kg) per daily irrigation. Mean and paired t-tests were completed. RESULTS: 24 patients were enrolled and had follow-up. Mean age was 10.5 years (range 3-21 years), 25%, 50%, 25% had thoracic, lumbar and sacral level lesions respectively. Mean NBoDS score at initiation of Peristeen® was 20.21 (±5.56), n = 24. The mean score after two weeks of use was 12.75 (±4.40), n = 24. There was a statistically significant decrease of 7.46 (95%CI, 5.07-9.84) points, t(23) = 6.47, P < 0.0005 after two weeks. There was a statistically significant decrease in their scores from initiation to the 2 month time period of 7.00 (95%CI, 2.18-11.82) points, t(9) = 3.29, P = 0.009. By the sixth month of daily use the mean NBoDS score was 9.67 (n = 12). This was an average decrease of 8.83 (95%CI, 5.39-12.28) points from initiation score, t(11) = 5.641, P < 0.005. CONCLUSION: The Peristeen® transanal irrigation system provides a significant reduction in NBoDS scores in pediatric patients with NBD. Peristeen® should be considered when other conservative bowel management options have been unsuccessful. Neurourol. Urodynam. 36:632-635, 2017. © 2016 Wiley Periodicals, Inc.

Kelly procedure for exstrophy or epispadias patients: Anatomical description of the pudendal neurovasculature.

INTRODUCTION: Adequate penile length in males with bladder exstrophy or epispadias is a major challenge. Kelly previously described a surgical technique of a single stage reconstruction for patients with exstrophy or epispadias that potentially achieves significant penile lengthening by completely detaching the insertion of the corpora cavernosa from the ischiopubic rami. However, because of the possibility of damage to the pudendal neurovascular supply that may lead to partial or complete penile loss, this technique has not gained popularity. The aim of this study is to describe the surgical anatomic relationship of the pudendal neurovascular bundle (NVB) to the ischiopubic rami and to determine a safer approach to dissection during the Kelly procedure. METHODS: We performed meticulous dissection in three formalin-fixed and one fresh adult male cadavers to demonstrate the anatomical relationships between the pudendal neurovascular supply of the penis and the cavernosal insertion to the ischiopubic ramus. RESULTS AND DISCUSSION: We demonstrated the relationships and distance between the NVB and the area of separation between the crus and the ischiopubic ramus at the level of the periosteum. The insertion of the crus to the ischiopubic ramus is inferior lateral, whereas the NVB lies at a superior medial position. This anatomical relationship is best visualized when the dissection is carried out starting from the distal portion of the NVB and proceeding proximally. This area of the periosteum is avascular and the NVB can be preserved safely as long as the dissection is conducted at that subperiosteal level. Based on this cadaver dissection study, we suppose that detaching the corporal cavernosa from the pubic bones at the subperiosteal level allows for a safe distance to be maintained from the pudendal nerve at all times. We believe that if a surgeon performs the dissection inferiorly and laterally, the corpora cavernosa can be safely detached from the ischiopubic ramus and injury to the pudendal vessels and nerve can be avoided. However, it must be noted that there are limitations to applying the results from this study of normal, adult cadavers to the anatomy of children and adolescents with exstrophy or epispadias, who form the largest proportion of patients who are candidates for this procedure. CONCLUSION: This anatomical study demonstrates the relationship between the pudendal NVB, the crus, and the ischiopubic ramus. We demonstrated how the separation of the crus from the ischiopubic periosteum might be performed more safely.

Endoscopic correction of vesicoureteral reflux simulator curriculum as an effective teaching tool: Pilot study.

INTRODUCTION: It has been well recognized that simulators are effective tools to teach and evaluate technical skills in laparoscopic surgery. Endoscopic injection for the correction of vesicourteral reflux has a definite learning curve. Surgeon experience has also been demonstrated to have an important role in the outcome of the procedure. Simulated training allows for practice in a realistic setting without the inherent risk of harm to the patient. This stress free environment allows the trainee to focus on the acquisition of surgical skills without worry about surgical outcome. OBJECTIVE: The aim was to validate a porcine bladder simulator curriculum for training and assessment of the surgical skills for the endoscopic correction of vesicoureteral reflux. STUDY DESIGN: We developed a porcine bladder-based dextranomer/hyaluronic acid (Dx/HA) injection simulator consisting of a dissected ex vivo porcine bladder in a polystyrene box with the distal ureters and urethra secured (Figure). We performed content validation by five experienced pediatric urologists. We then organized a simulator curriculum, which included lecture, demonstration, and a 2-h hands-on training on the simulator. Content, discriminant, and concurrent validation of the simulator curriculum were carried out using 11 urology trainees at different levels of expertise. All the trainees were evaluated for each step of the procedure of both their first and last performances on the simulator. RESULTS: Overall, the model demonstrated good content validity by all experts (mean questionnaire score 92%). The simulator curriculum demonstrated a significant improvement in the performance of the trainees between their first and last evaluations (56-92%; p = 0.008). Specific parts of the procedure that showed significant improvement (p < 0.05) were identification of the ureteral orifice, ureteral orifice hydrodistention, first and second injection, and location, size, and depth of the mound after injection. DISCUSSION: The Dx/HA endoscopic injection simulator is an effective training tool to improve the performance of the surgeon carrying out the procedure. This teaching tool may be used to help improve the performance of the surgeon carrying out the procedure. This teaching curriculum may shorten the early learning curve historically associated with the procedure and provide a greater understanding of the technical components of successful endoscopic vesicoureteral reflux correction. Additionally, the implementation of this simulator within the developed curriculum can improve the performance of training urologists in all steps of the challenging technique of Dx/HA needle injection confirming concurrent validity. The next step in evaluation of this surgical skill-training curriculum would be to determine if the improvement in skill performance observed during training translates to improved performance in the clinical realm, or predictive validity. LIMITATIONS: Some small differences exist between the porcine model and human ureteral orifices. In the porcine model the ureteral orifices are located medially and distally in the bladder neck, which make injection more challenging. Participants suggested that after practicing with the simulator endoscopic injection to a human ureteral orifice would be easier. CONCLUSION: The simulator curriculum was able to improve the performance of the surgeon carrying out the procedure during subsequent simulations.

Development, reliability and validation of a neurogenic bowel dysfunction score in pediatric patients with spina bifida.

OBJECTIVE: To develop a reliable and valid questionnaire to monitor neurogenic bowel symptoms in children. PATIENTS: Thirty-four children aged 6-18 with neurogenic bowel and their caregivers. Eighteen control patients. METHODS: An expert panel generated a domain of observables and formative/reflective content. Response options were scaled following Likert-type items. Key informant interviews revised the measures. A final questionnaire was given to patients twice to calculate intra-rater reliability using Cohen's Kappa Coefficient (k) and paired t-test. Blinded interviews were conducted after physical examination and health assessment and questionnaires completed by a nurse to determine construct validity and inter-rater reliability using k and Spearman's rank-order correlation. Control patients completed the questionnaire once, their results were used to determine discriminate validity and a receiver operating characteristic (ROC) curve. RESULTS: Intra-rater reliability showed 85% of the questionnaires having k >0.6. Paired t-test results of t(33) = 1.997, P = 0.054, d = 0.53, confirmed there was not a significant difference between the scores of the two completed questionnaires. Inter-rater reliability showed 97% of the questionnaires having k >0.6 between the nurse and the patient/caregiver responses. Scores had a strong positive correlation at rs (32) = 0.943, P < 0.0005. Mean score with neurogenic bowel was 15.18(STD ± 5.77) and control group 4.68(STD ± 2.98). ROC analysis showed an area under the curve of 0.9. A score of 8.5 correlated with presence of neurogenic bowel with sensitivity of 94% and specificity of 87%. CONCLUSION: The questionnaire shows positive reliability and validity when used for pediatric neurogenic bowel patients. The questionnaire differentiates between normal and neurogenic patients. Larger studies are necessary to conduct further validation.

Predicting the Risk of Breakthrough Urinary Tract Infections: Primary Vesicoureteral Reflux.

PURPOSE: We constructed a risk prediction instrument stratifying patients with primary vesicoureteral reflux into groups according to their 2-year probability of breakthrough urinary tract infection. MATERIALS AND METHODS: Demographic and clinical information was retrospectively collected in children diagnosed with primary vesicoureteral reflux and followed for 2 years. Bivariate and binary logistic regression analyses were performed to identify factors associated with breakthrough urinary tract infection. The final regression model was used to compute an estimation of the 2-year probability of breakthrough urinary tract infection for each subject. Accuracy of the binary classifier for breakthrough urinary tract infection was evaluated using receiver operator curve analysis. Three distinct risk groups were identified. The model was then validated in a prospective cohort. RESULTS: A total of 252 bivariate analyses showed that high grade (IV or V) vesicoureteral reflux (OR 9.4, 95% CI 3.8-23.5, p <0.001), presentation after urinary tract infection (OR 5.3, 95% CI 1.1-24.7, p = 0.034) and female gender (OR 2.6, 95% CI 0.097-7.11, p <0.054) were important risk factors for breakthrough urinary tract infection. Subgroup analysis revealed bladder and bowel dysfunction was a significant risk factor more pronounced in low grade (I to III) vesicoureteral reflux (OR 2.8, p = 0.018). The estimation model was applied for prospective validation, which demonstrated predicted vs actual 2-year breakthrough urinary tract infection rates of 19% vs 21%. Stratifying the patients into 3 risk groups based on parameters in the risk model showed 2-year risk for breakthrough urinary tract infection was 8.6%, 26.0% and 62.5% in the low, intermediate and high risk groups, respectively. CONCLUSIONS: This proposed risk stratification and probability model allows prediction of 2-year risk of patient breakthrough urinary tract infection to better inform parents of possible outcomes and treatment strategies.

Autologous cell seeded biodegradable scaffold for augmentation cystoplasty: phase II study in children and adolescents with spina bifida.

PURPOSE: Augmentation cystoplasty using gastrointestinal segments in children/adolescents with medically refractory neurogenic bladder is associated with significant complications. We evaluated an autologous cell seeded biodegradable scaffold (Tengion®) for bladder augmentation as an alternative to traditional enterocystoplasty in this population. MATERIALS AND METHODS: A phase II prospective study was performed in children with neurogenic bladder due to spina bifida requiring enterocystoplasty for detrusor pressure 40 cm H2O or greater despite maximum antimuscarinic medication. Following open bladder biopsy, urothelial and smooth muscle cells were grown ex vivo and seeded onto a biodegradable scaffold to form a regenerative augment as the foundation for bladder tissue regeneration. Bladder neck sling was the only concomitant surgical procedure permitted. Bladders were cycled postoperatively to promote regeneration. Primary and secondary outcomes at 12 months included change in bladder compliance, bladder capacity and safety. Long-term assessment was done with similar outcomes at 36 months. RESULTS: Compliance improved in 4 patients at 12 months and in 5 patients at 36 months, although the difference was not clinically or statistically significant. There was no clinical or statistical improvement in bladder capacity at 12 or 36 months in any patient. Adverse events occurred in all patients, and most were easily treated. Two patients had low cell growth following bladder biopsy, of whom 1 withdrew from the study and 1 underwent a second biopsy. Serious adverse events of bowel obstruction and/or bladder rupture occurred in 4 patients. CONCLUSIONS: Our autologous cell seeded biodegradable scaffold did not improve bladder compliance or capacity, and our serious adverse events surpassed an acceptable safety standard.

Changing stone composition profile of children with nephrolithiasis.

OBJECTIVE: To determine if this trend toward calcium phosphate stone formation exists in children. METHODS: This is a retrospective study of medical records of 179 children managed at our medical center from 1992-2010 for whom stone analysis and other pertinent laboratory data were available. A comparison of patients managed from 1992-2000 (P1) and 2001-2010 (P2) was undertaken. Statistical analysis included nonparametric tests. RESULTS: There were no significant differences in the mean age of the 2 cohorts. During both periods, boys comprised a significantly higher proportion during the first decade of life, whereas girls comprised a significantly higher proportion during the second decade. A higher percentage of patients had calcium oxalate (CaOx) stones in P1 compared to P2 (60% vs 47%, P = .0019). There was a significant increase in the percentage of patients having calcium phosphate stones in P2 compared to P1 (27% vs 18.5%, P = .008). Twenty-seven patients had recurrent stones. A comparison of the compositions of the first and last stones of patients within this group demonstrated an increasing proportion of brushite stones (3.7% vs 11.1%, P = .04). Twenty-four hour urine testing results were similar for those with CaOx and calcium phosphate stones. CONCLUSION: An increasing proportion of children have calcium phosphate calculi. Brushite stones are more prevalent in children with recurrent stone events. The impetus of these shifts is not readily apparent.

Split ureteral stent after ureteroureterostomy.

Ipsilateral ureteroureterostomy is a well documented surgical approach to duplicated collecting systems associated with obstruction or Vesicoureteral reflux. Indwelling stents are employed in these procedures, to facilitate unobstructed flow across the anastomosis, to minimize anastomotic leak, and to assist in aligning the repair. Positioning of the ureteral stent is controversial. Some advocate placement of the stent to the recipient ureter across the suture line to prevent anastomotic stricture. Others prefer placing the stent along the donor ureter in order to facilitate drainage of the moiety that was originally obstructed. We describe a technique that allows drainage both ureters by using a single stent that has been split at its proximal end just across the U-U anastomosis.

Megaureter.

Almost one-quarter of the children referred to a pediatric urologist for obstructive uropathy suffer from an obstructive megaureter. However, not all megaureters are due to obstruction, as some may be the result of reflux and many simply represent a slightly skewed stage of development that can result in a normal urinary tract if observed. As the use of fetal ultrasonography has expanded, the majority of children with megaureters are now diagnosed early in their development, and physicians are faced with the complex task of distinguishing which children need medical intervention and which do not. The surgical treatments of megaureter are well established, relatively simple, and effective if performed in the correct candidates. Therefore, research efforts in this field have recently focused on improving our ability to diagnose clinically relevant obstructive uropathy and examining the developmental causes of megaureter, and how this disorder may be prevented.

Posterior urethral valves.

The most common cause of lower urinary tract obstruction in male infants is posterior urethral valves. Although the incidence has remained stable, the neonatal mortality for this disorder has improved due to early diagnosis and intensive neonatal care, thanks in part to the widespread use of prenatal ultrasound evaluations. In fact, the most common reason for the diagnosis of posterior urethral valves presently is the evaluation of infants for prenatal hydronephrosis. Since these children are often diagnosed early, the urethral obstruction can be alleviated rapidly through catheter insertion and eventual surgery, and their metabolic derangements can be normalized without delay, avoiding preventable infant mortality. Of the children that survive, however, early diagnosis has not had much effect on their long-term prognosis, as 30% still develop renal insufficiency before adolescence. A better understanding of the exact cause of the congenital obstruction of the male posterior urethra, prevention of postnatal bladder and renal injury, and the development of safe methods to treat urethral obstruction prenatally (and thereby avoiding the bladder and renal damage due to obstructive uropathy) are the goals for the care of children with posterior urethral valves[1].

Cold knife valvulotomy for posterior urethral valves using novel optical urethrotome.

OBJECTIVES: To present our results after valve ablation using a novel cold knife urethrotome. METHODS: Eleven consecutive male patients with posterior urethral valves underwent cold knife valvulotomy using a modified optical pediatric urethrotome. Patients were assessed both pre- and postoperatively using serum creatinine, voiding cystourethrography, and renal/bladder ultrasonography. RESULTS: From August 2003 to August 2005, 11 patients underwent cold knife valvulotomy, of whom 7 returned for postoperative follow-up (mean follow-up 17.4 months). At surgery, the patients ranged in age from 5 days to 9 years. At presentation, 5 of the 7 patients had an elevated serum creatinine (mean 2.5 mg/dL, range 0.3-6.5), all had bilateral hydronephrosis of at least grade 3, and 6 of 7 had at least grade 3 reflux on 1 side. Intraoperatively, 1 complication (minor urethral laceration) occurred. Postoperatively, all 6 patients with serum creatinine levels measured showed improvement in renal function (mean creatinine 0.47 mg/dL, range 0.2-0.9). For the 6 patients who underwent postoperative ultrasonography, 4 had either complete resolution or significant improvement in their hydronephrosis, and none showed worsening. Six patients underwent postoperative voiding cystourethrography, with 5 showing either marked improvement or complete resolution of their reflux and 1 showing stable, unilateral reflux. CONCLUSIONS: Valvulotomy using our modified urethrotome is a safe and effective technique for valve ablation.

Vesicoureteral reflux: where have we been, where are we now, and where are we going?

We present a retrospective review of the scientific and clinical advances, extending over four decades, which have linked vesicoureteral reflux, with renal injury, and urinary tract infection. We have traced the original studies, coupled with advances in technology which led to the awareness, and ability to detect and diagnose the problems early in childhood. These advances progressed through clinical studies which defined the epidemiology of both reflux and urinary tract infection. Along with these diagnostic advances, there were numerous surgical developments, which allowed progressive improvements in the outcomes and effectiveness of a variety of treatment modalities. All of this literature leads us to the current era, when several clinical trials are currently underway in an effort to more fully define the most efficacious and safe methods to treat vesicoureteral reflux and associated urinary tract infection.

Long-term durability, tissue regeneration and neo-organ growth during skeletal maturation with a neo-bladder augmentation construct.

AIMS: To comparatively evaluate bladder regeneration following 80% cystectomy and augmentation using a synthetic biopolymer with autologous urothelial and smooth muscle cells (autologous neo-bladder augmentation construct [construct]) or autotransplantation of native bladder (reimplanted native urinary bladder [reimplant]) in canines. MATERIALS & METHODS: Voiding function, urodynamic assessment and neo-organ capacity-to-body-weight ratio (C:BW) were assessed longitudinally for a total of 24 months following trigone-sparing augmentation cystoplasty in juvenile canines. RESULTS: Within 30 days postimplantation, hematology and urinalysis returned to baseline. Constructs and reimplants yielded neo-organs with statistically equivalent urodynamics and histology. Linear regression analysis of C:BW showed that constructs regained baseline slope and continued to adapt with animal growth. CONCLUSIONS: Constructs and reimplants regained and maintained native bladder histology by 3 months, capacity at 3-6 months and compliance by 12-24 months. Furthermore, construct C:BW demonstrated the ability of regenerated bladder to respond to growth regulation.

The bear paw: a radiologic sign for congenital ureteropelvic junction obstruction.

This article defines a new radiographic descriptor of high-grade hydronephrosis suggestive of ureteropelvic junction obstruction that is easily recognized by urologists and non-urologists alike. This is useful information for medical personnel participating in the management of children with hydronephrosis.

Comparison of traditional enterocystoplasty and seromuscular colocystoplasty lined with urothelium.

OBJECTIVE: To evaluate long-term outcomes between various methods of augmentation cystoplasty. METHODS: A retrospective analysis was performed of patients undergoing seromuscular colocystoplasty lined with urothelium (SCLU, n=26), and their outcomes compared to a similar population of patients in the same institution who had received traditional forms of bladder augmentation (colocystoplasty and ileocystoplasty, n=32). Measurements included efficacy of the procedure in increasing bladder capacity and achieving urinary continence, and the need of subsequent surgery for complications. RESULTS: There was no statistically significant difference in achieved bladder capacity, subjective urinary continence and the rates of subsequent surgery for stones, vesicoureteral reflux, augment failure, bladder neck continence and catheterizable channel. None of the patients in the SCLU group had spontaneous perforation or small bowel obstruction. CONCLUSION: Patients with SCLU are at decreased risk for bowel obstruction and spontaneous perforation, but are not devoid of other long-term complications including bladder stones, vesicoureteral reflux and augment failure. Most of the risks and benefits of augmentation cystoplasty performed using ileum, colon, or SCLU appear similar.

Management of the valve-bladder syndrome and congenital bladder obstruction: the role of nocturnal bladder drainage.

Valve-bladder syndrome often develops after the resolution of posterior urethral valves, but is also found after the resolution of congenital bladder obstruction. The features of this syndrome include the persistent dilation of the upper urinary tracts, a thick-walled, noncompliant urinary bladder, urinary incontinence, and polyuria secondary to nephrogenic diabetes insipidus. Nocturnal bladder management, which involves timed emptying of the bladder or continuous drainage, has been recommended in conjunction with diurnal timed voiding therapy as an adjunct to the treatment of valve-bladder syndrome. This treatment is derived from the hypothesis that valve-bladder syndrome is caused by congenital obstruction, and that the resultant changes in detrusor muscle are associated with a persistent bladder dysfunction characterized by chronic overdistention of the urinary bladder. Such overdistention is exacerbated by polyuria, and can be a cause of secondary hydronephrosis. Bladder dysfunction and overdistention is usually treated during waking time, but occasionally this is not effective on its own, and nocturnal therapy is used as well. To date, there are a few sets of data that suggest overnight bladder drainage can bring about profound improvements in the degree of upper-tract hydronephrosis, renal function, or bladder function. Nocturnal bladder drainage seems, in these initial reports, to be a simple and safe therapeutic maneuver. This review discusses the etiology of valve-bladder syndrome and examines each of the studies which have investigated nocturnal bladder drainage in its treatment.

Testicular infarction in a 12-year-old boy with Wegener's granulomatosis.

We present the case of a 12-year-old boy with testicular infarction secondary to Wegener's granulomatosis. Scrotal exploration revealed no evidence of testicular torsion. The testis was left in place, and, after systemic therapy, partial reperfusion was present.