E. Van Laecke

One thousand video‐urodynamic studies in children with non‐neurogenic bladder sphincter dysfunction

Objective To ascertain the aetiology and epidemiology of non‐neurogenic bladder sphincter dysfunction (NNBSD) by assessing the results of prospective video‐urodynamic studies (VUD) in 1000 children.

TRANSCUTANEOUS NEUROMODULATION FOR THE URGE SYNDROME IN CHILDREN: A PILOT STUDY

PURPOSE Neuromodulation has been used to treat voiding dysfunction in adults. Due to its invasiveness it has rarely been used in children until now with the availability of transcutaneous neurostimulation. We evaluated clinical effects of transcutaneous neuromodulation on detrusor overactivity in children with the urge syndrome. MATERIALS AND METHODS Between May 1, 1998 and February 28, 1999, 15 girls (mean age 10.2 years) and 26 boys (mean age 10.7 years) with proved detrusor hyperactivity on videourodynamic study underwent neuromodulation. All children had been given anticholinergic therapy previously. Neurostimulation only was used in children in whom anticholinergics had no effect and those who had significant side effects. Anticholinergics were continued in children in whom they had a partial effect. Stimulation of 2 Hz. was applied for 2 hours every day. Surface electrodes were placed at the level of sacral root S3. After 1 month of trial stimulation those children who responded continued the treatment for 6 months, and were evaluated every 2 months. RESULTS Of the 41 children 15 boys and 13 girls responded after 1 month of trial therapy with an increase in bladder capacity, decrease in urgency, decrease in incontinence and/or better sensitivity. Of the 13 children who did not respond 9 lacked motivation and 4 had no clinical effect despite motivation. After 6 months of therapy a significant increase in bladder capacity, decrease in voiding frequency and decrease in incontinence periods were noted. Adverse effects were not observed. One year after therapy relapse was noted in 7 patients, leaving 21 of 41 children definitively cured. CONCLUSIONS Although preliminary, our results indicate that transcutaneous neuromodulation can improve symptoms of detrusor overactivity, as response to stimulation was noted in 76% of our patients and 56% were cured after 1 year. This therapeutic option is attractive for children because of its noninvasiveness and absence of adverse effects.

Pelvic‐floor therapy and toilet training in young children with dysfunctional voiding and obstipation

Objective To analyse experience in treating young children (4–5 years old) with urodynamically confirmed voiding dysfunction, using a noninvasive training programme.

The effect of botulinum-A toxin in incontinent children with therapy resistant overactive detrusor.

PURPOSE We determined the effect of detrusor injection of botulinum-A toxin in a cohort of children with therapy resistant non-neurogenic detrusor overactivity. This prospective study included therapy resistant children with overactive bladder. MATERIAL AND METHODS During the study period of 19 months 10 boys and 11 girls were included. All patients showed decreased bladder capacity for age, urge and urge incontinence. Main treatment duration before inclusion was 45 months. A dose of 100 U botulinum-A toxin (Botox) was injected in the detrusor. RESULTS Side effects were evaluated in all 21 included patients. The side effects reported were 10-day temporary urinary retention in 1 girl and signs of vesicoureteral reflux with flank pain during voiding in 1 boy, which disappeared spontaneously after 2 weeks. No further examinations were done since the boy refused. Two girls experienced 1 episode each of symptomatic lower urinary tract infection. Eight girls and 7 boys with a minimum followup of 6 months represent the study group for long-term evaluation. In this study group after 1 injection 9 patients showed full response (no more urge and dry during the day) with a mean increase in bladder capacity from 167 to 271 ml (p <0.001). Three patients showed a partial response (50% decrease in urge and incontinence) and 3 remained unchanged. Eight of the 9 full responders were still cured after 12 months, while 1 of the initially successfully treated patients had relapse after 8 months. The 3 partial responders and the patient with relapse underwent a second injection with a full response in the former full responder and in 1 partial responder. CONCLUSIONS Botulinum-A toxin injection in children with non-neurogenic overactive detrusor is an excellent treatment adjunct, leading to long-term results in 70% after 1 injection.

Gonadal pathology and tumor risk in relation to clinical characteristics in patients with 45,X/46,XY mosaicism

CONTEXT Gonadectomy is avoided whenever possible in boys with 45,X/46,XY. However, no clinical markers are currently available to guide clinicians in predicting gonadal tumor risk or hormone production. OBJECTIVE The objective of the study was to test the hypothesis that gonadal histology and risk for development of a malignant germ cell tumor are reflected by the clinical presentation of a 45,X/46,XY individual. DESIGN The design of the study was the correlation of clinical data [external masculinization score (EMS), pubertal outcome] with pathology data (gonadal phenotype, tumor risk). SETTING This was a multicenter study involving two multidisciplinary disorder of sex development teams. PATIENTS Patients included genetically proven 45,X/46,XY (and variants) cases, of whom at least one gonadal biopsy or gonadectomy specimen was available, together with clinical details. INTERVENTIONS Patients (n = 48) were divided into three groups, based on the EMS. Gonadal histology and tumor risk were assessed on paraffin-embedded samples (n = 87) by morphology and immunohistochemistry on the basis of established criteria. MAIN OUTCOME MEASURES Gonadal differentiation and tumor risk in the three clinical groups were measured. Clinical outcome in patients with at least one preserved gonad was also measured. RESULTS Tumor risk in the three groups was significantly related to the gonadal differentiation pattern (P < 0.001). In boys, hormone production was sufficient and was not predicted by the EMS. CONCLUSIONS The EMS reflects gonadal differentiation and tumor risk in patients with 45,X/46,XY. In boys, testosterone production is often sufficient, but strict follow-up is warranted because of malignancy risk, which appears inversely related to EMS. In girls, tumor risk is limited but gonads are not functional, making gonadectomy the most reasonable option.

A high easy-to-treat complication rate is the price for a continent stoma.

Objective To evaluate the conduit‐related complications and their treatment in Mitrofanoff continent urinary diversion and antegrade colonic enema (ACE) procedures.

The Role of Pelvic-Floor Therapy in the Treatment of Lower Urinary Tract Dysfunctions in Children

The pelvic-floor is under voluntary control and plays an important role in the pathophysiology of lower urinary tract (LUT) dysfunctions in children, especially of non-neuropathic bladder sphincter dysfunction. The following therapeutic measures can be applied to try to influence the activity of the pelvic-floor during voiding: proprioceptive exercises of the pelvic-floor (manual testing), visualization of the electromyographic registration of relaxation and contraction of the pelvic-floor by a curve on a display (relaxation biofeedback), observation of the flow curve during voiding (uroflow biofeedback), learning of an adequate toilet posture in order to reach an optimal relaxation of the pelvic-floor, an individually adapted voiding and drinking schedule to teach the child to deal consciously with the bladder and its function and a number of simple rules for application at home to increase the involvement and motivation of the child. In children however with persisting idiopathic detrusor instability additional therapeutic measures may be necessary to improve present urologic symptoms (incontinence problems, frequency, urge) and to increase bladder capacity. Intravesical biofeedback has been used to stretch the bladder and seems to be useful in case of sensory urge. Recently a less invasive technique, called transcutaneous electrical nerve stimulation (TENS), has been applied on level of S3 with promising results in children with urodynamicaly proven detrusor instability, in which previous therapies have failed.

Bladder neck closure for treating pediatric incontinence

Aims of Study: In order to evaluate the effects of bladder neck closure (BNC) for treatment of pediatric incontinence, on the quality of life of those children, we reviewed the files of 17 children who underwent this procedure during the last 5 years. Information on previous surgery before BNC, continence and complications after BNC and patient satisfaction are gathered.Material and Methods: During the study period, 17 children (9 male, 8 female) underwent BNC with continent diversion. Ten children (5 male, 5 female) suffered neurogenic incontinence in meningomyelocele, 5 children (4 male, 1 female) had bladder exstrophy, 1 girl suffered iatrogenic incontinence after treatment of an ectopic ureterocele and 1 girl suffered structural incontinence after pelvic fracture. Previous surgery for incontinence was done in 12 children with 36 procedures. Primary BNC was done in 5 children. For continent diversion the appendix was used in 13, the ureter in 2, a Monti procedure in 1, and an ileal valve in 1. In 9 children, ileal bladder augmentation was performed at the time of BNC. Four children were augmented before. Mean age at time of operation was 13.5 years. Mean follow–up is 35 months.Results: After BNC, all patients were completely dry. One girl suffered some stomal incontinence during the night. This disappeared after recent bladder augmentation. Patient satisfaction is extremely high after surgery. All patients feel happy with their stoma and do not regret the choice they made. As for complications, urinary tract infections were seen in 9 patients. Stomal complications were seen in 8 patients. Three patients had some difficulty with catheterization, which could be solved with dilatation. Appendiceal polyps, which could be removed under local anesthesia, were seen in 3 and stomal stenosis which needed re–intervention in 2. In 1 patient, three revisions of the stoma were done, in the other patient, two surgical corrections. Out of 8 patients showing stomal complications, only 2 needed revision under anesthesia. All complications are seen in the first 6 months after the continent diversion.Conclusions: We present a series of patients who underwent BNC for treatment of incontinence. BNC is the ultimate bladder neck reconstruction. However, regarding the high success rate, the low complication rate and the high patient satisfaction, we must consider BNC as an important procedure for the reconstructive surgeon. If reconstruction fails, closure must be considered. Compared to most other procedures for bladder neck reconstruction, BNC gives the highest continence rate. Good patient compliance and strict medical follow–up of the upper tract is mandatory in these patients.

Oral lyophylizate formulation of desmopressin: superior pharmacodynamics compared to tablet due to low food interaction.

PURPOSE Desmopressin is a standard treatment for monosymptomatic nocturnal enuresis. Different formulations are promoted as bioequivalent, although these claims are not supported by comparative pharmacodynamic data in children. Food interaction is known to influence the bioavailability of desmopressin. We compared the pharmacodynamics of the 2 most frequently used desmopressin formulations, tablet and lyophilizate, with a standardized meal, allowing extrapolation to clinical reality, where the interval between evening meal and medication intake is limited for school-age children. We hypothesized there would be a faster pharmacodynamic response, and greater concentrating and antidiuretic activity for the fast dissolving (melt) formulation compared to the tablet with simultaneous food intake. MATERIALS AND METHODS Two tests were performed on separate days in identical standardized conditions, starting with a 15 ml/kg water load. After achieving maximal diluting capacity a standardized meal was administered, followed by desmopressin tablet (t test) or melt (M-test). Diuresis rate and urinary osmolality were measured hourly. Paired data from 4 girls and 15 boys with a mean age of 12.1 years were obtained. RESULTS In the early response phase more than 25% of patients had a higher diuresis rate with tablet vs melt formulation, reaching statistical significance in the plateau phase (urine collected at hours 3 to 5, p <0.02) and in duration of action (urine collected at hours 5 to 8, p <0.005). For desmopressin melt smaller standard deviations in diuresis rate were remarkable. Concentrating capacity demonstrated no significant differences between formulations in the early response phase, in contrast to the plateau phase (p <0.036) and duration of action (p <0.001). CONCLUSIONS With meal combination desmopressin melt formulation has a superior pharmacodynamic profile to tablet, making it more suitable for the younger age group with a limited interval between meal and drug administration.

Reconstructive Surgery for Severe Penile Inadequacy: Phalloplasty with a Free Radial Forearm Flap or a Pedicled Anterolateral Thigh Flap

Objectives. Severe penile inadequacy in adolescents is rare. Phallic reconstruction to treat this devastating condition is a major challenge to the reconstructive surgeon. Phallic reconstruction using the free radial forearm flap (RFF) or the pedicled anterolateral thigh flap (ALTF) has been routinely used in female-to-male transsexuals. Recently we started to use these techniques in the treatment of severe penile inadequacy. Methods. Eleven males (age 15 to 42 years) were treated with a phallic reconstruction. The RFF is our method of choice; the ALTF is an alternative when a free flap is contraindicated or less desired by the patient. The RFF was used in 7 patients, the ALTF in 4 patients. Mean followup was 25 months (range: 4–49 months). Aesthetic and functional results were evaluated. Results. There were no complications related to the flap. Aesthetic results were judged as “good” in 9 patients and “moderate” in 2 patients. Sensitivity in the RFF was superior compared to the ALTF. Four patients developed urinary complications (stricture and/or fistula). Six patients underwent erectile implant surgery. In 2 patients the erectile implant had to be removed due to infection or erosion. Conclusion. In case of severe penile inadequacy due to whatever condition, a phalloplasty is the preferred treatment nowadays. The free radial forearm flap is still the method of choice. The anterolateral thigh flap can be a good alternative, especially when free flaps are contraindicated, but sensitivity is markedly inferior in these flaps.