Lower urinary tract symptoms in children and adolescents with Williams-Beuren syndrome.

INTRODUCTION: Williams-Beuren syndrome (WBS) is a genetic condition caused by a microscopic deletion in the chromosome band 7q11.23. Individuals with WBS may present with congenital cardiovascular defects, neurodevelopmental disturbances and structural abnormalities of the urinary tract. Lower urinary tract symptoms (LUTS) seem to be frequent in this population, but studies on this topic are scarce and based on small case series. OBJECTIVE: To systematically evaluate the prevalence of lower urinary tract symptoms (LUTS) and the acquisition of bladder control in a large population with WBS. STUDY DESIGN: A cross-sectional study evaluating 87 consecutive patients with WBS; there were 41 girls and 46 boys. Genetic studies confirmed WBS in all patients. Subjects were clinically evaluated with: a history of LUTS obtained from the parents and child, a structured questionnaire of LUTS, a 3-day urinary frequency-volume chart, a quality of life question regarding LUTS, and physical examination. A history regarding the acquisition of bladder control was directly evaluated from the parents. RESULTS: Mean age of patients was 9.0 ± 4.2 years, ranging from 3 to 19 years. Based on the symptoms questionnaire and the frequency-volume chart, 70 patients (80.5%) were symptomatic. The most common symptom was urgency, affecting 61 (70.1%) patients, followed by increased urinary frequency in 60 (68.9%) patients, and urge-incontinence in 53 (60.9%), as shown in Summary Fig. More than half of the children reported nocturnal enuresis, including 61% of the girls and 52% of the boys. Twenty-three patients (25.6%) had a history of urinary tract infections. The mean age for acquisition of dryness during the day was 4.4 ± 1.9 years. Parents of 61 patients (70.1%) acknowledged that LUTS had a significant impact on the quality of life of their children. DISCUSSION: A high prevalence of LUTS was confirmed with a significant negative impact on quality of life in a large population of children and adolescents with WBS. It was shown for the first time that the achievement of daytime bladder control is delayed in children with WBS. Although LUTS are not recognized as one of the leading features of the syndrome, it is believed that it should be considered as a significant characteristic of the clinical diagnosis of WBS. CONCLUSIONS: LUTS are highly prevalent in children and adolescents with WBS and have a significant negative impact on patient's quality of life.

Early postoperative pelvic-floor biofeedback improves erectile function in men undergoing radical prostatectomy: a prospective, randomized, controlled trial.

Erectile dysfunction (ED) and urinary incontinence are common complications following radical prostatectomy (RP). Although pelvic-floor biofeedback training (PFBT) may improve urinary continence following RP, its effects on the recovery of potency are unknown. Fifty-two patients selected for RP were prospectively randomized for a treatment group (n=26) receiving PFBT once a week for 3 months and home exercises or a control group (n=26), in which patients received verbal instructions to contract the pelvic floor. Erectile function (EF) was evaluated with the International Index of Erectile Function-5 (IIEF-5) before surgery and 1, 3, 6 and 12 months postoperatively. Patients were considered potent when they had a total IIEF-5 score>20. Continence status was assessed and defined as the use of no pads. Groups were comparable in terms of age, body mass index, diabetes, pathological tumor stage and neurovascular bundle preservation. A significant reduction in IIEF-5 scores was observed after surgery in both groups. In the treatment group, 8 (47.1%) patients recovered potency 12 months postoperatively, as opposed to 2 (12.5%) in the control group (P=0.032). The absolute risk reduction was 34.6% (95% confidence interval (CI): 3.8-64%) and the number needed to treat was 3 (95% CI: 1.5-17.2). A strong association between recovery of potency and urinary continence was observed, with continent patients having a 5.4 higher chance of being potent (P=0.04). Early PFBT appears to have a significant impact on the recovery of EF after RP. Urinary continence status was a good indicator of EF recovery, with continent patients having a higher chance of being potent.

Influence of a hypercholesterolemic diet on the collagen composition of the bladder wall extracellular matrix in rats.

PURPOSE: To investigate the effects of hypercholesterolemic diet on the collagen composition of urinary bladder wall. MATERIALS AND METHODS: Forty-five female 4-week-old Wistar rats were divided into three groups: 1) control group fed a normal diet (ND); 2) model of bladder outlet obstruction (BOO) group fed a ND; and 3) group fed a HCD (1.25% cholesterol). Total serum cholesterol, LDL cholesterol and body weight were assessed at baseline. Four weeks later, group 2 underwent a surgical procedure resulting in a partial BOO, while groups 1 and 3 underwent a sham similar surgical procedure. Six weeks later, all animals had their bladders removed; serum cholesterol and LDL cholesterol levels and body weights were measured. Morphological and morphometric analysis was performed by Picrosirius staining and collagen types I and III were identified by immunofluorescence. Statistical analysis was completed and significance was considered when p<0.05. RESULTS: Rats fed an HCD exhibited a significant increase in LDL cholesterol levels (p<0.001) and body weight (p=0.017), when compared to the groups fed a ND during the ten-week study period. Moreover, the HCD induced morphological alterations of the bladder wall collagen, regarding thin collagen fibers and the amounts of type III collagen when compared to the control group (p=0.002 and p=0.016, respectively), resembling the process promoted in the BOO model. CONCLUSIONS: A hyper-cholesterolemic diet in Wistar rats promoted morphological changes of the bladder types of collagen, as well as increases in body weight and LDL cholesterol.

Bladder compliance what does it represent: can we measure it, and is it clinically relevant?

AIMS: To report the conclusion of the Think Thank 8 on Compliance Discussions during the second ICI-RS meeting in 2010. METHODS: During a 3-day meeting a group of specialists discussed bladder compliance, what it represents, how it can be measured and if it is clinically relevant. RESULTS: Bladder compliance is the result of a mathematical calculation of the volume required for a unit rise of pressure measured during a cystometric filling. It gives an indication on how the different mechanisms in the bladder wall react on stretching. There is a need of standardization of measurement and suggestions for this are given in the text. Pitfalls are described and how to avoid them. There is a wide range of compliance values in healthy volunteers and groups of patients. Poor compliance needs to be defined better as it can have significant clinical consequences. Prevention and treatment are discussed. CONCLUSION: If compliance is correctly measured and interpreted, it has importance in urodynamic testing and gives information relevant for clinical management.

Neurological patients need evidence-based urological care.

AIMS: To report the conclusion of the Think Thank on Neurourology discussions during the first ICI-RS meeting in 2009. METHODS: During a 3-day meeting a group of specialists discussed evidence-based medicine in neurourology and made suggestions for future research. RESULTS: In the vast majority of patients with neurological disease bladder dysfunction occurs. The actual rules of diagnosis and treatment lack a study related evidence base. From a long list of possible research subjects, prevalence, detrusor pressure, imaging, catheterization and surgery have been first discussed. CONCLUSION: In each of these subjects, research items are suggested which can help to improve the care in this patient group.

Traditional suburethral sling operations for urinary incontinence in women.

BACKGROUND: Traditional suburethral slings are surgical operations used to treat women with symptoms of stress urinary incontinence. OBJECTIVES: To determine the effects of traditional suburethral slings on stress incontinence alone or stress with other types of urinary (mixed) incontinence in comparison with other management options. SEARCH STRATEGY: We searched the Cochrane Incontinence Group Specialised Trials Register (searched 22 December 2004), The UK National Research Register (Issue 1, 2001) and the reference lists of relevant articles. We hand searched the proceedings of the Brazilian Congress of Urology from 1991 to 2003, inclusive. SELECTION CRITERIA: Randomised or quasi-randomised trials that included traditional suburethral slings for the treatment of stress or mixed urinary incontinence. DATA COLLECTION AND ANALYSIS: All three reviewers independently extracted data from included trials onto a standard form and assessed trial methodological quality. The data abstracted were relevant to predetermined outcome measures. Where appropriate, a summary statistic was calculated: a relative risk for dichotomous data and a weighted mean difference for continuous data. MAIN RESULTS: Thirteen trials were identified including 760 women of whom 627 were treated with suburethral slings. Five compared suburethral slings with open abdominal retropubic colposuspension (Burch/Marshall-Marchetti-Krantz) and one compared suburethral slings with needle suspension (Stamey). In six trials, different types of suburethral sling were compared with each other. Nine types of slings were included (Teflon, polytetrafluoroethylene, prolene used for transvaginal tape (TVT), porcine dermis, lyophilised dura mater, fascia lata, vaginal wall, autologous dermis and rectus fascia). There were no comparisons of suburethral sling with anterior repair, laparoscopic retropubic suspension, peri-urethral injections or artificial sphincters. One trial compared surgery (including slings) with anticholinergic medication.There were no statistically significant differences between traditional slings and other types of continence surgery, or between one type of traditional sling and another sling. Confidence intervals around the estimates were wide, reflecting the few data available, and so clinically important differences could not be ruled out. AUTHORS' CONCLUSIONS: The data on sub urethral sling operations remain too few to address the effects of this type of surgical treatment. Few trials are reported by authors in a complete fashion and most information came from abstracts presented in annual meetings. The broader effects of suburethral slings could not be established since trials did not include appropriate outcome measures such as general health status, health economics, pad testing, third party analysis and time to return to normal activity level. Data obtained from thirteen trials did not provide reliable estimates because of their sizes, and heterogeneity of designs, populations studied, and types of comparisons made. Reliable evidence on which to judge whether or not suburethral slings are better or worse than other surgical or conservative management is currently not available.

Suburethral sling operations for urinary incontinence in women.

BACKGROUND: Suburethral slings are surgical operations used to treat women with urinary incontinence. They were originally designed for recurrent stress incontinence, but have also been used recently for primary cases. OBJECTIVES: To determine the effects of suburethral slings on stress or mixed urinary incontinence in comparison with other management options. SEARCH STRATEGY: We searched the Cochrane Incontinence Group's trials register, The UK National Research Register (Issue 1, 2001) and the reference lists of relevant articles. We hand searched the proceedings of the Brazilian Congress of Urology from 1991 to 1999, inclusive. Date of most recent search: March 2001. SELECTION CRITERIA: Randomised or quasi-randomised trials that included suburethral slings for the treatment of urinary incontinence. DATA COLLECTION AND ANALYSIS: Both reviewers independently extracted data from included trials onto a standard form and assessed trial methodological quality. The data abstracted were relevant to predetermined outcome measures. Where appropriate, a summary statistic was calculated: a relative risk for dichotomous data and a weighted mean difference for continuous data. MAIN RESULTS: Seven trials were identified including 682 women - 457 treated with suburethral slings and 225 with other procedures. Four compared suburethral slings with open abdominal retropubic suspensions (Burch/Marshall-Marchetti-Krantz) and one compared suburethral slings with needle suspension (Stamey). In the two last trials, different types of suburethral sling were compared with each other. Six types of slings were included (Teflon, polytetrafluoroethylene, prolene used for tension free vaginal tape (TVT), porcine dermis, lyophilized dura mater and rectus fascia). There were no comparisons of suburethral sling with anterior repair, laparoscopic retropubic suspension, peri-urethral injections, artificial sphincters or conservative management. In respect of short-term cure, overall rates are similar (RR 0.93; 95% CI 0.68 to 1.27) in comparison to open abdominal retropubic suspension. This mainly reflects the results of one larger trial on TVT. However, for long term results, data are too few to give a reliable estimate. Data were too few to address whether other types of suburethral slings were as effective as open abdominal retropubic suspension or needle suspension. There were no detectable differences in terms of voiding dysfunction, urge incontinence or detrusor instability between suburethral slings and abdominal or needle suspensions, but the data were few and the confidence intervals wide. About one in 11 had a complication during TVT, most commonly bladder perforation, but none had serious consequences. In the small trial which compared autologous (rectus fascia) with synthetic (Goretex) slings, 11/32 vs 2/16 women were not cured after a year (RR 0.36, 95% CI 0.09 to 1.45) which is not statistically significant but fewer women with autologous slings had complications (0/32 vs 5/16; RR 21.35, 95% CI 1.25 to 363.78). Two women in the Goretex group had late sling erosion of the urethra requiring removal of the Goretex, although their incontinence remained cured. REVIEWER'S CONCLUSIONS: Preliminary results from a larger trial provide reassuring evidence about the performance of the less invasive TVT sling procedure. Cure rates after TVT were similar to those following open abdominal retropubic suspension, but with confidence intervals of around 10% absolute difference. About one in 11 women had a complication during TVT, most commonly bladder perforation, but none had serious consequences. Long term results are awaited. The data were too few to address whether other types of suburethral slings were as effective as open abdominal retropubic suspension or needle suspension. There was limited evidence from one small trial that slings made of Goretex had more complications than slings made of rectus fascia. The broader effects of suburethral slings could not be established since trials did not include appropriate outcome measures such as general health status, health economics, pad testing, third party analysis and time to return to normal activity level. Evidence that suburethral slings may be better or worse than other surgical or conservative management is lacking because no trials addressed these comparisons.

Suburethral sling operations for urinary incontinence in women.

BACKGROUND: Suburethral slings are surgical operations used to treat women with urinary incontinence. They were originally designed for recurrent stress incontinence, but have also been used recently for primary cases. OBJECTIVES: To determine the effects of suburethral slings on stress or mixed urinary incontinence in comparison with other management options. SEARCH STRATEGY: We searched the Cochrane Incontinence Group's trials register, The National Research Register and the reference lists of relevant articles. We hand searched the proceedings of the Brazilian Congress of Urology from 1991 to 1999, inclusive. Date of most recent search: January 2000. SELECTION CRITERIA: Randomised or quasi-randomised trials that included suburethral slings for the treatment of urinary incontinence. DATA COLLECTION AND ANALYSIS: Both reviewers independently extracted data from included trials onto a standard form and assessed trial methodological quality. The data abstracted were relevant to predetermined outcome measures. Where appropriate, a summary statistic was calculated: a relative risk for dichotomous data and a weighted mean difference for continuous data. MAIN RESULTS: Five trials were identified including 206 women - 126 treated with suburethral slings and 80 with other procedures. Three compared suburethral slings with open abdominal retropubic suspensions (Burch/Marshall-Marchetti-Krantz) and one compared suburethral slings with needle suspension (Stamey). In the fifth trial, different types of suburethral sling were compared with each other. Six types of slings were included (Teflon, polytetrafluoroethylene, Goretex, porcine dermis, lyophilized dura mater and rectus fascia). There were no comparisons of suburethral sling with anterior repair, laparoscopic retropubic suspension, peri-urethral injections, artificial sphincters or conservative management. There is one ongoing trial with Tension-free Vaginal Tape (TVT) versus Burch colposuspension, results expected in August 2000. For each outcome data were available only from individual trials. There were no detectable differences in terms of voiding dysfunction, urge incontinence or detrusor instability between suburethral slings and abdominal or needle suspensions, but the data were few and the confidence intervals wide. More peri-operative complications were reported after suburethral slings than after needle suspension (9/10 vs 2/10; RR 4.5, 95% CI 1.3 to 15.8), and length of stay was longer after sling operations than after abdominal or needle suspension. In the trial which compared autologous (rectus fascia) with synthetic (Goretex) slings, 11/32 vs 2/16 women had not improved after a year (RR 2.75, 95% CI 0.69 to 10.95), but fewer had complications (0/32 vs 5/16; RR 0.05, 95% CI 0 to 0.8). Two women in the second group had late sling erosion of the urethra requiring removal of the Goretex, although their incontinence remained cured. REVIEWER'S CONCLUSIONS: There were few data available to compare suburethral sling operations with other surgical management (abdominal and needle suspension, or different types of slings). In general, the quality of trials was poor with no clear method of randomisation, small numbers of women studied, short follow-up and scanty information on outcome measures. There is not enough evidence on which to judge whether suburethral slings are better or worse than other surgical or non surgical managements.

Incontinent epispadias: surgical treatment of 38 cases.

We review 38 cases of surgically corrected incontinent epispadias with a followup of 5 months to 18 years. In 20 cases the Leadbetter, in 8 the Tanagho and in 8 the Young-Dees techniques of bladder neck reconstruction were used. Of 3 patients with minimal (15 to 25 ml.) bladder capacity the Arap procedure was performed in 1, while small constriction of the bladder neck to improve the bladder capacity and compliance was done in 2. In 1 of the latter patients a 60 ml. capacity was achieved and a secondary Leadbetter operation provided an excellent result. Continence was attained after the initial operation in 18 patients, followup is too short to determine the result in 3 and 15 did not acquire urinary control. Revision of the bladder neck plasty was performed in 11 patients, which resulted in continence in 4 and partial continence in 2. Among 34 patients with an adequate followup 22 (73.3 per cent) are continent and 8 (26.4 per cent) are incontinent. The results were similar with the 3 techniques.

Sacral agenesis: why is it so frequently misdiagnosed?

Thirty-four patients with sacral agenesis were seen from 1954 to 1983, cases of meningomyelocele excluded. Five recognizable and consistent patterns of bone malformation were identified. Urodynamic examinations were done in 10 of the 34 patients. Their evaluation and response to treatment are analyzed; we tried to determine and establish the possible causes for its late diagnosis and consequences regarding the upper urinary tract.

Urethral pull-through operation and long-term follow-up.

Pull-through urethroplasty is appropriate for acute or delayed treatment of lesions of the membranous and proximal bulbar urethra. the length of the stricture itself appears to be more important in predicting success than the precipitation event which produces the stricture.

Micturition and the male genitourinary response to sacral root stimulation.

The sacral roots, because of their anatomic and functional organization, are well suited to chronic stimulation. Data from our previous experiments on female dogs suggested that effective voiding responses to stimulation could be obtained by using the ventral root of S2, the sacral nerve in canines with the greatest detrusor representation. Elimination of dorsal root afferents from the stimulus field was essential to the minimization of spinal reflex activity. A selective somatic neurotomy was also necessary to attenuate maximally sphincter activity. In the male, the presence of the prostate gland, the greater urethral length, and the erectile response to stimulation might result in compression of the urethra, compromising micturition. Sacral root stimulation was thus studied using both spinalized and nonspinalized male dogs; the results are discussed with a view toward the applicability of sacral root stimulation to effect micturition in male paraplegics, who comprise the largest percentage of this group of patients.

Sacral root stimulation in controlled micturition. Peripheral somatic neurotomy and stimulated voiding.

Stimulation of the sacral ventral roots with the largest detrusor representation was found to be moderately successful for bladder emptying in acute canine experiments. However, removal of spinal reflexes arising from sacral root stimulation did not eliminate sphincteric resistance. For that reason ventral roots were stimulated after peripheral somatic neurotomy. Unilateral pudendal nerve sectioning was compared with selective sectioning of the somatic fibers of S2, the root with the greatest detrusor representation in canines. Both were equally effective in significantly reducing outlet resistance. Selective nerve section has one advantage: it preserves more of the nerve pathways involved in reflexogenic erection.

Peyronie disease: surgical treatment with muscular aponeurosis.

A new method for surgical correction of Peyronie disease by grafting autologous muscular aponeurosis was tried in 4 patients. Early results were encouraging, with disappearance of penile curvature and of pain on erection. The urologist can perform the operation without the assistance a plastic surgeon must usually give when dermal grafts are used.

Urinary bladder and sphincter responses to stimulation of dorsal and ventral sacral roots.

The sacral spinal roots have a long intraspinal yet extradural pathway that makes them ideal for electrode implantation for the control of micturition. In the hope of identifying a specific motor pathway to the bladder within these roots, a study of 10 mongrel female dogs was undertaken: bladder and sphincter responses to stimulation of individual sacral roots, and to stimulation of the dorsal and ventral components of each root, were recorded urodynamically. The root producing greatest detrusor response was then divided into its motor and sensory components, and the central and peripheral ends of the latter were stimulated. We found that sphincter resistance to bladder emptying was diminished by removing the afferent dorsal roots from the stimulus field. The physiologic implications of this finding are discussed.

Feasibility of inducing micturition through chronic stimulation of sacral roots.

Past attempts at inducing micturition in paraplegics by electric stimulation of the spinal cord micturition center have been generally unsuccessful. Failure has most often resulted from inability to separate the response of the detrusor from that of the sphincter. Because the sacral nerves provide some organizational specificity over the spinal cord and are practically suited to chronic stimulation, experiments were carried out in dogs to find out whether or not stimulation of these roots could induce micturition. Results of acute dog studies did suggest that micturition might effectively be performed by stimulation of the ventral root of the sacral nerve having the greatest detrusor representation. Specificity of bladder response was improved by dividing somatic fibers peripherally. Selected division of somatic fibers from the sacral root stimulated was compared with complete pudendal neurotomy. (The former procedure has the advantage of preserving perineal sensation.) The principles learned from the acute studies were then applied to "chronic" dogs--5 paraplegic and 1 normal. Effective voiding was achieved on a long-term basis. Over-all results were very encouraging. Further research is necessary before application of this technique to humans can be considered.

The male genitourinary sphincter mechanism in the dog.

In an effort to locate and characerize a genital sphincter in the male posterior urethra, the intraluminal changes in urethral pressure under both sympathetic and parasympathetic stimulation were studied. It appears that the urethra has a specific genital sphincteric length corresponding to the supramontanal portion. The urethral length is seemingly under sympathetic control, so that backflow of semen does not occur during ejaculation. The urinary sphincter apparently involves the entire posterior urethra and is primarily under parasympathetic influence.

Neurologic control of prostatic secretion in the dog.

A model to record the volume of prostatic secretion was developed. The influence of both divisions of the autonomic nervous system on the prostate by pharmacologic stimulation and blockade, and by direct electrical nerve stimulation was observed. The prostate responded to parasympathetic stimulus by increasing secretion. Under sympathetic stimulation, the secretion collected in the acini and ducts was expulsed into the urethra by contraction of the smooth muscle fibers surrounding these structures. A correlation between these findings and the normal sexual function may be made: Early in the excitation phase there is parasympathetic activity with increased secretion. With onset of orgasm and emission, sympathetic activity initiates the contractile activity of the genital duct system and the prostatic muscular element. As a result, the secretion is transported to the prostatic urethra (emission) to be later ejaculated by the genital skeletal musculature of the perineum, mainly the ischiocavernosus and bulbospongiosus.