Four-hour voiding observation with provocation test reveals significant abnormalities of bladder function in newborns with spinal dysraphism.

INTRODUCTION: Four-hour voiding observation with provocation test (VOP) using a scale, a damp detector and ultrasound for determination of residuals, is an easily performed non-invasive method for the evaluation of bladder function in newborns. Neonatal bladder function evaluated with VOP has been described for healthy newborns (HN) but not for children with spinal dysraphism (SD), for whom early bladder evaluation is essential for decisions regarding Clean Intermittent Catheterization and follow-up. The aim of the present study was to describe voiding observation with provocation test in newborns with spinal dysraphism and compare with corresponding data for healthy newborns. METHODS AND MATERIALS: At a tertiary hospital, a 4 h voiding observation with provocation (VOP) was performed in 50 neonates (22 girls, 28 boys) with spinal dysraphism (37 open SD, 13 closed SD) consecutively evaluated for possible neurogenic bladder-sphincter dysfunction (1998-2019). All newborns with open SD and 4/13 with closed SD had been through postnatal neurosurgery before the test. Mean age was 10 days. Voiding observation was performed during 4 h with visual observation the fourth hour recording behavior and urinary flow (e.g. stream, dribbling). Finally, bladder provocations (e.g. suprapubic compression) were performed, and any leakage was noted. Findings were compared to those of 50 healthy newborns (HN) earlier published (Gladh et al., 2002). There were no significant differences in background data such as gender, age or diuresis between newborns with SD and HN. RESULTS AND DISCUSSION: Voiding observation with provocation test of children with SD revealed significant differences compared to HN see summary table. Some children with SD had frequent small voids/leakages and low bladder volumes while three had no voiding and high volumes. Leakage during bladder provocation test and not voiding with a stream was not seen in HN but were common in newborns with SD (69% resp. 74%) (p < 0.01). A child with these findings should thus be investigated further. Identifying children needing Clean Intermittent Catheterization is important as well as being able to postpone or refrain from invasive urodynamic studies if not strongly indicated. VOP may give valuable information for these judgements. CONCLUSION: Newborns with spinal dysraphism differ from healthy newborns in many aspects of bladder function. Bladder function varies between newborns with closed and open spinal dysraphism. Many newborns with spinal dysraphism leak at bladder provocation and void without a stream but healthy newborns do not. Early determination of post-void residuals is mandatory in children with spinal dysraphism and non-invasive VOP gives this information in a standardized way, also adding information on frequency, voiding with a stream and leakage at provocation.

Independence does not come with the method--treatment of neurogenic bowel dysfunction in children with myelomeningocele.

AIM: The aim was to evaluate and compare different bowel regimes with regard to satisfaction, faecal incontinence and independence, and the relationship to quality of life among children with myelomeningocele (MMC). METHODS: A questionnaire, including the health-related quality of life instrument PedsQL 4.0™, was sent to all children aged seven to 16 years (n = 172) with MMC, treated at two centres in Sweden and one in Norway. The three centres cover a third of the population in the two countries. The response rate was 62%. RESULTS: Parents of children (30%) using antegrade colonic enemas (ACE) reported higher satisfaction (p = 0.01) than the parents of those (47%) using transanal irrigation (TAI). The children reported no significant difference. Children and parents in the ACE group reported more complete evacuation of the bowels than the TAI group. No significant difference was found in faecal incontinence or independent toileting. The children (40%) who emptied their bowels independently reported a higher quality of life. Children using TAI or ACE spent around one hour on the toilet at every bowel emptying. CONCLUSION: TAI and ACE are effective treatments, but time-consuming and difficult to perform independently. Higher parental satisfaction is obtained with ACE. Irrespective of method the children who can use the toilet independently report a higher quality of life, which makes efforts to support independence valuable.

Children with myelomeningocele and independence in the toilet activity: a pilot study.

OBJECTIVE: Regarding adult life and independence the most common obstacles for young adults with myelomeningocele (MMC) are cognitive dysfunction and difficulties in performing toilet activities. A step-by-step method with goal setting for the training of self-care in toilet activities for children with MMC was evaluated. METHOD: Twenty-two children with MMC and bladder and bowel dysfunction (12 girls, 10 boys) aged 3-17.2 (m 9.1) were included. The toilet activities were observed at home jointly by an occupational therapist and urotherapist. Goal-setting procedures of self-training were promoted. Observation scores before and after intervention were compared, the goal setting being evaluated on a Goal Attainment Scale (GAS). RESULTS: Fifteen children who trained in self-catheterization had a median observation score of 22 before and 37 after the training period (p = 0.002). Another seven trained in trans-rectal irrigation with a median score of 30 before and 49 after (p = 0.02). As a result of GAS all children improved, of whom 17 reached the goal or even more so than expected. CONCLUSIONS: In this pilot study mutual goal setting in a step-by-step training programme based on professional observation of the toilet activity at home showed a better outcome than traditionally performed training in a hospital setting or with traditional habilitation support.

Renal preservation in children with neurogenic bladder-sphincter dysfunction followed in a national program.

PURPOSE: Neurogenic bladder-sphincter dysfunction (NBSD) constitutes the major reason for morbidity in children with spina bifida. The aim of this study was to identify risk factors for renal damage in children with NBSD followed according to the Swedish national guidelines. MATERIALS AND METHODS: Records and cystometries from 6 to 16 years (median 11) follow up of 41 consecutive children born 1993-2003 with NBSD were evaluated. The children were divided into a high pressure group (baseline pressure above 30 cmH(2)O at maximal clean intermittent catheterization volume in at least two cystometries) and a low pressure group. Most children (34/41) were followed from birth. RESULTS: Although renal scarring on DMSA-scintigraphy was found in 5/41 children, all but one had normal renal function. Two already had renal scars on entering the follow-up program at age 2.5 and 3 years. Renal scarring was more frequent in the high pressure group (P < 0.01). Most children with renal scars (4/5) had a combination of low compliant bladder and insufficient compliance with treatment and follow up. CONCLUSION: High baseline pressure is confirmed as a risk factor that, in combination with complex social issues, creates a demanding situation for families and professionals. A structured early follow up with treatment compliance effectively prevents renal damage.

Children and youth with myelomeningocele's independence in managing clean intermittent catheterization in familiar settings.

AIM: To examine the ability of children and youth with myelomeningocele to independently manage clean intermittent catheterization. METHODS: There were 50 participants with myelomeningocele (5-18 years); 13 of them had also participated in a previous hospital-based study. Their abilities and interest in completing the toilet activity were examined at home or in school using an interview and the Canadian Occupational Performance Measure (COPM). Actual performance was observed and rated. Background variables were collected from medical records and KatAD+E tests. RESULTS: In total, 48% were observed to perform the toilet activity independently, in comparison with 74% who self-reported independence. Univariate analyses found KatAD+E could predict who was independent. COPM failed to do so. Ability to remain focused and ambulation were predictors of independence, but age, sex and IQ were not. Multivariable analysis found time to completion to be the strongest predictor of independence. Four children were independent in their familiar environment, but not in the hospital setting, and six of 13 children maintained focus only in their familiar environment. CONCLUSIONS: Interviews were not sufficiently accurate to assess independence in the toilet activity. Instead, observations including time to completion are recommended. The execution of the toilet activity is influenced by the environmental context.

Voiding school for children with idiopathic urinary incontinence and/or bladder dysfunction.

OBJECTIVE: Individually applied urotherapy is first-line treatment in children with bladder dysfunction. A new concept of treatment for small groups of children was applied and evaluated. PATIENTS AND METHODS: Two hundred children, 116 of them girls, aged 3-14 years (median 7.2) with bladder dysfunction and incontinence received urotherapy in small groups (2-5), called voiding school (VS). Outcome was evaluated after 3 and 12 months by voiding/leakage diary and questionnaire, and at 3 months by uroflow and post-void residual urine as well. RESULTS: The outcome of VS was independent of age and gender. At follow up at 3 and 12 months, respectively, 35% and 40% of the children were cured and another 30% and 34% improved (P≤0.0001). Compared with the year before start of VS, urinary tract infections decreased from 34% to 6% (P<0.0001). Median residual urine decreased from 15 ml before VS to 6 ml after 3 months (P<0.001). CONCLUSION: The concept of VS is a good alternative to individual urotherapy, with the outcome of fewer urinary tract infections and improved continence. Urotherapy for groups of children compared to individual treatment is also expected to have financial benefits.

Quality of life in neurologically healthy children with urinary incontinence.

AIM: To bring forward the arguments for active treatment of urine incontinence in otherwise healthy children, a quality-of-life (QoL) study was performed. SUBJECTS AND METHODS: A self-rating QoL questionnaire, child-adjusted and validated, was completed by 120 neurologically healthy children, aged 6-16 y, with urinary incontinence. Another 239 age-matched children made up a control group. The two groups were compared both totally and in age-related subgroups (6-8, 9-12, >12 y) concerning the index for all questions, for universal parts (without questions dealing with incontinence) as well as for specific key domains. RESULTS: The patient group had a significantly lower index than the control group both with and without items related to incontinence (p<0.0001). Social situation, self-esteem and self-confidence were most influenced, particularly in the youngest children. Thirty-one children (13%) of the control group reported incontinence and did not score their QoL as good as their continent peers but better than the study patients. CONCLUSION: From the quality-of-life aspects, the study supports active treatment of urinary incontinence in children already at younger ages.

Tap-water enema for children with myelomeningocele and neurogenic bowel dysfunction.

AIM: To evaluate the outcome of transrectal irrigation (TRI) using clean tap water without salt in children with myelomeningocele and neurogenic bowel problems. METHODS: 40 children (21 boys and 19 girls; aged 10 mo to 11 y) with myelomeningocele and neurogenic bowel dysfunction were treated with TRI given by a stoma cone irrigation set daily or every second day. A questionnaire on the effects on faecal incontinence, constipation and self-management was completed by the parents, 4 mo-8 y (median 1.5 y) after start. Effects on rectal volume, anal sphincter pressure and plasma sodium were evaluated before and after the start of irrigation. RESULTS: At follow-up, 35 children remained on TRI, four had received appendicostomy, while one defecated normally. For all children but five (35/40; 85%) the procedure worked satisfactorily, but a majority found the procedure very time consuming and only one child was able to perform it independently. All children were free of constipation; most (35/40) were also anal continent. Rectal volume and anal sphincter pressure improved, while plasma sodium values remained within the normal range. CONCLUSION: Transrectal irrigation with tap water is a safe method to resolve constipation and faecal incontinence in children with myelomeningocele and neurogenic bowel dysfunction, but it does not help children to independence at the toilet.

A new fast-melting oral formulation of desmopressin: a pharmacodynamic study in children with primary nocturnal enuresis.

OBJECTIVE: To determine the pharmacodynamic properties of a new oral lyophilisate formulation of desmopressin (in single doses of 30, 60, 120, 240, 360 or 480 microg) in children with known primary nocturnal enuresis (PNE) and thus identify those dosages that could provide a duration of action corresponding to a typical length of night-time sleep in children with PNE; additional objectives were to determine the safety and tolerability of desmopressin in this population. PATIENTS AND METHODS: Children with PNE (mean three or more wet nights/week), aged 6-12 years, were randomized into a double-blind, placebo-controlled study. An overhydration technique was used before dosing to suppress endogenous vasopressin production and thereby ensure that any antidiuresis could be attributed to treatment. Dosing with desmopressin or placebo occurred when urinary production was >0.13 mL/min/kg. Urinary volume, osmolality and duration of urinary-concentrating action (above three threshold levels: 125, 200 and 400 mOsm/kg) were determined as endpoints. RESULTS: All 72 participants receiving desmopressin had a pharmacodynamic response to the drug, while there was no change in urinary output in the 12 placebo-treated patients. There was a clear relationship between desmopressin dose and duration of action and osmolality during action, although the three highest-dose groups had similar results. The mean duration of action of desmopressin at the lowest osmolality threshold level was 3.6-10.6 h, according to dose; for the highest threshold, the values were 1.3-8.6 h. CONCLUSION: Desmopressin, as the oral lyophilisate, causes a marked decrease in urinary output in hydrated children with PNE. A small dose range (120-240 microg) is likely to control diuresis for a period corresponding to a night's sleep (7-11 h) in most children with PNE. However, some patients might require a higher dose to obtain antidiuresis for the complete night.

[Children with meningomyelocele become adults!]. / Barn med ryggmärgsbråck blir vuxna!

The survival for children with spina bifida has dramatically increased during the last 30 years. In Sweden today 40-50 adolescents with spina bifida are reaching adulthood each year the next 10 years. Children with spina bifida are from birth to adulthood followed by a multidisciplinary medical and paramedical team within the habilitation organisation. However, from the age of 18 this responsibility is discontinued, often with less readiness in adult medicine to meet the spina bifida adolescents and their special needs. Facing adolescence and adulthood both children and parents need a careful preparation from several points of view for the transition. It is also most important to prepare the adult medical disciplines about the special needs of this group. This process has to start early to reach successful management, including improvement in self-care.

Outcome of the bladder cooling test in children with nonneurogenic bladder problems.

PURPOSE: The bladder cooling test (BCT) engages a primitive neonatal spinal reflex that becomes suppressed by descending signals in older children and may reappear with suprasacral lesions. We assessed the outcome of the BCT in a large group of children with nonneurogenic bladder problems. MATERIALS AND METHODS: The BCT was evaluated in a consecutive series of 178 girls and 106 boys, 1 month to 18 years old with bladder problems without overt neurology. The test was performed at the end of routine cystometry by a rapid control infusion of body warm saline followed, after fluid evacuation, by the same volume of cold saline (3 to 10C). The test was considered positive if a detrusor contraction greater than 30 cm H2O was evoked by the cold but not the warm fluid. RESULTS: Most children younger than 4 years had a history of pyelonephritis (29 of 34) and/or had vesicoureteral reflux (grade IV to V in 26 of 34). For those younger than 2 years 87% of the BCTs were positive while only 21% of the tests were positive in 2 to 3-year-old children. Most children older than 4 years had idiopathic urge incontinence, and greater than 50% of the BCTs were positive in the youngest (less than 6 years) with a gradual decline to 0% at age 13 years. CONCLUSIONS: Conversion of positive to negative BCTs at about age 2 years presumably represents normal maturation while positive tests in older incontinent children suggest delayed maturation of the central neuronal control of the bladder.

[The pediatrician's approach to bed-wetting. A survey shows that hospital clinics adhere to current recommendations]. / Så behandlar barnläkaren sängvätning. Enkät visar att landets sjukhuskliniker följer gällande rekommendationer.

On behalf of the Swedish Enuresis Academy a survey how children with primary enuresis nocturna are handled, a questionnaire about investigation and treatment of the condition was sent to all pediatric clinics in Sweden. It was found, that the care of children with enuresis nocturna is initiated at the age of 5-6 years. After a careful history (especially to exclude daytime incontinence problems or signs of bladder dysfunction), a clinical investigation of the child and an urinalysis (dipslide), treatment is started with bedalarm or desmopressin owing to what the child and parents have chosen after having been offered both alternatives. Bedalarm is available at all pediatric clinics, mostly for hire. Follow up is usually performed by telephone by a specialist nurse or urotherapist. Most children with enuresis nocturna are handled at primary care clinics by general practitioners. To complete the survey a similar study in the primary health care system is therefore suggested.

Intravesical electrical stimulation in the treatment of micturition dysfunction in children.

AIMS: To evaluate the results of intravesical electrical stimulation (IVES) in an open prospective study to treat underactive detrusor in children. The treatment was offered as an alternative to clean intermittent catheterization (CIC). METHODS: Forty-four children were included, 21 girls and 3 boys (6-16 years, md 10) with idiopathic, 9 girls and 11 boys (4-18 years; md 13) with neurogenic underactive detrusor. IVES was given by a catheter electrode in the bladder (cathode) with the anode attached to the suprapubic abdominal skin. Continuous stimulation at 20 or 25 Hz was delivered by battery powered stimulators giving unipolar square-wave pulses (0,2 or 0,7 ms). Stimulation intensity was adjusted individually according to the acceptance of the child (12-64 mA). IVES was initially given at the clinic but 18 children had additional treatment at home. Effect of treatment was monitored by micturiton/incontinence diary, reports of bladder sensation, recordings of urinary flow, residual volume and frequency of urinary tract infections. RESULTS: The IVES-treatment was completed by 39/44 children. Long term normalization of the voiding (md 2,5 years follow up) was obtained for 20/24 children with idiopathic problems (83%) and 8/20 with neurogenic problems (40%). Another four had much improved bladder function. The neurogenic group required more stimulation sessions than the idiopathic group. Of those on CIC, 11/15 who completed IVES could discontinue the catheterization. The frequency of urinary tract infections and incontinence decreased significantly (P < 0.01). CONCLUSIONS: It is concluded that IVES is a promising method to treat the underactive detrusor in children.

Urethrovaginal reflux--a common cause of daytime incontinence in girls.

OBJECTIVE: The objective of this study was to estimate the frequency of urethrovaginal reflux as the cause of daytime incontinence in school-age girls, and to study the characteristic symptoms and the effect of simple instructions intended to amend the problem. MATERIAL AND METHODS: Girls with urethrovaginal reflux were identified in a group of 169 girls, aged 7 to 15 years, referred to a specialist clinic because of daytime incontinence. They were evaluated by a noninvasive screening protocol, including a careful history and neurourologic examination, bladder diaries, urine analysis, uroflows, and residual urine determined by ultrasound. Girls with urethrovaginal reflux were instructed by a urotherapist on how to achieve better toilet habits. RESULTS: Urethrovaginal reflux was found in 21 (12.4%) of 169 girls as the sole (19) or contributing (2) cause of their daytime urinary incontinence. They all had a typical history of small leakage 5 to 10 minutes after voidings during the day, confirmed by a specific bladder diary. All were neurologically healthy, and all but 2 had a normal bladder function. The latter 2 girls had residual urine and asymptomatic bacteriuria. At follow-up after median 2 years, all girls were free from postmicturition leakage, but the 2 with residual urine remained daytime incontinent with cystometrically proven phasic detrusor overactivity. CONCLUSIONS: Urethrovaginal reflux is a common cause of urinary incontinence in girls. The diagnosis is easily obtained by an adequate history, completed with a specific bladder diary. The problem is easily resolved by proper voiding instructions.