Lower Urinary Tract Dysfunction in Pediatric Patients with Multiple Sclerosis: Diagnostic and Management Concerns.

BACKGROUND: Multiple sclerosis (MS) is increasing in the pediatric population and, as in adults, symptoms vary among patients. In children the first manifestations can sometimes overlap with acute neurological symptoms. Urological symptoms have not been much studied in childhood. We shared our experience with MS urological manifestation in children. METHODS: This article is a retrospective evaluation of all children with MS, according to the Krupp criteria, who also present with urological symptoms. We collected demographic and clinical history, the MR localization of demyelinating lesions, urological symptoms, and exams. RESULTS: We report on six MS pediatric cases with urological manifestation. Urinary symptoms, characterized by urinary incontinence in five patients and urinary retention in one patient, appeared in a different time frame from MS diagnosis. Urodynamic exams showed both overactive and underactive bladder patterns. Treatment was defined according to lower urinary tract dysfunction, using clean intermittent catheterization, oxybutynin, and intradetrusor Onabotulinum Toxin-A injection. A low acceptance rate of invasive evaluation and urological management was observed. CONCLUSIONS: The MS diagnosis was traumatic for all our patients. We believe it is important to address urological care in young people from the time of diagnosis for prompt management; it could be useful to include a pediatric urologist in multidisciplinary teams.

Megacystis-microcolon-intestinal hypoperistalsis syndrome: don't forget the bladder.

PURPOSE: Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a well described clinical condition, but reports are focused on microcolon and intestinal hypoperistalsis, while data on bladder management are scant. Aim of the study is to present urological concerns in MMIHS. METHODS: Retrospective evaluation of clinical data on urological management of MMIHS patients treated in the last 10 years. RESULTS: Six patients were enrolled (3 male, 3 female). Three girls had prenatal diagnosis of megacystis (1 vesicoamniotic shunt was placed). All patients had genetic diagnosis: 5 had ACTG2 gene mutations and 1 MYH11 mutation. All patients were addressed to our attention for urinary symptoms, such as urinary retention, urinary tract infections, acute renal injury. Two patients presented frequent stoma prolapses. All children underwent a complete urological evaluation, and then started a bladder management protocol (clean intermittent catheterization, via urethra or cystostomy-tube placement), with improvement of urinary infections, upper urinary tract dilation and stoma prolapses, if present. All patients had good renal function at last follow-up. CONCLUSION: We believe that MMIHS patients must be addressed soon and before onset of symptoms for a multidisciplinary evaluation, including an early assessment by a pediatric urologist expert in functional disorder, to preserve renal function at its best.

Thulium Laser for the Treatment of Posterior Urethral Valves in Infants.

Objective: Thulium laser (ThL) has become popular in urology, because of its powerful action on tissue, achieving optimal ablation and hemostasis. Aim of our article was to evaluate efficacy of ThL in infants affected by posterior urethral valve (PUV) ablation. Patients and Methods: Clinical charts of 25 infants (age ≤12 months) who underwent PUV ablation were retrospectively reviewed. According to our protocol, all patients performed voiding cystourethrography and cystoscopy 6 to 8 months after initial treatment. Several factors, including age and weight at surgery, operative time, postoperative bleeding, catheterization period, postoperative urinary retention, retreatment for valve remnants, and stricture at follow-up, were evaluated. Preoperative, intraoperative, and postoperative data were analyzed. Results: Mean age at primary surgery was 4.5 months (5 days-10.5 months) and mean weight at primary surgery was 5.7 kg (2.5-10.3 kg). Mean operative time was 29.5 minutes (range 15-50 minutes). None of the patients experienced intraoperative and postoperative bleeding. In all cases, postoperative catheterization period was 1 day. Residual valves were found in 6 of 25 (24%) patients. No cases of urethral stricture were registered during follow-up (48.4 months, range: 11-95). Analyzing literature data using other techniques, complication rate of ThL PUV ablation seems lower than standard treatments (electrofulguration, cold knife) and comparable with those reported with other laser techniques. Conclusion: PUV ablation with ThL has proven to be feasible and safe in infants. Further studies are needed to define the real effectiveness of this laser technology in PUV ablation. Miniaturized instruments and ThL technology make early PUV treatment feasible also in low body weight newborns.

Hypospadias management in children with anorectal malformation: a multidisciplinary single center experience.

Anorectal malformation (ARM) is often associated to other congenital malformations, requiring a tailored management. Hypospadias' treatment in ARM is poorly described. Aim of study is to describe our experience in ARM-hypospadias patients especially in relation to occult spinal dysraphism (OSD). ARM patients treated from 1999 to 2022 were retrospectively reviewed, selecting male with hypospadias. Clinical data, hypospadias's severity, ARM sub-type (Group A: perineal fistula; Group B: urethral fistula, bladder fistula, no fistula), OSD, other associated malformations, NLUTD were evaluated. Exclusion criteria: uncomplete data. Among 395 ARMs, 222 were males, 22 (10%) had hypospadias. Two patients were excluded. Group A: 8 patients, Group-B: 12. Hypospadias: proximal 9 patients, distal 11. Neuro-urological evaluation was performed before hypospadias repair. Eleven patients (55%) had OSD. Four OSD patients presented NLUTD and underwent detethering and CIC (two via cystostomy button, two via appendicostomy); two of them had hypospadias repaired. All proximal hypospadias underwent two stages of surgery. Distal hypospadias was corrected in 4/11 cases. Hypospadias is quite common in ARM patients and its surgical management must be scheduled considering the possible OSD and NLUTD, with the possible need for intermittent catheterization. Complexity of ARM and hypospadias appears to be related to each other.

Onabotulinum Toxin A Intradetrusor Injections in Children with Neurogenic Lower Urinary Tract Dysfunction: Long-Term Histological Effects on the Bladder Wall.

BACKGROUND: In the last twenty-five years, Onabotulinum Toxin A (BTX-A) has gained increasing popularity for neurogenic lower urinary tract dysfunction (NLUTD) treatment. To maintain its efficacy, repeated BTX-A intradetrusor injections are required over time, with unknown effects on the bladder wall in children. The aim of this paper is to report long-term effects on the bladder wall in children treated with BTX-A. METHODS: Children with NLUTD not responsive to anticholinergics were treated with BTX-A, according to our protocol, with bladder wall control using endoscopic cold-cup biopsy. Specimens were evaluated considering edema, chronic inflammation, and fibrosis. RESULTS: Of the 230 patients treated from 1997 to 2022, we considered only specimens obtained in patients who had received ≥5 treatments (36 children), considered as the threshold to evaluate clinical effectiveness on long-term treatment with BTX-A. Most of them had congenital NLUTD (25 patients) and detrusor overactivity (27 patients). In all, increased edema and chronic inflammation with reduced fibrosis over time was reported; these data were not statistically significant. No difference was observed between patients with congenital and acquired diseases. CONCLUSIONS: Repeated intradetrusor BTX-A injections are not related to significant histological alterations in children, similarly with adults, and repeated injections could be considered safe.

Home pelvic floor exercises in children with non-neurogenic Lower Urinary Tract Symptoms: Is fitball an alternative to classic exercises?

INTRODUCTION: Biofeedback with home pelvic floor exercises were recommended as non-pharmacologic treatment for non-neurogenic Lower Urinary Tract Symptoms (LUTS) in children. Fitball is recommended to improve all-over flexibility, balance, and coordination, especially for pelvic floor. Aim of the study was to investigate efficacy of standard home pelvic floor exercises versus exercises using fitball. METHODS: From April 2021 to February 2022 to all children arrived in our clinic with non-neurogenic LUTS nonresponder at urotherapy, our pelvic floor rehabilitative program was proposed. During the rehabilitation children performed: standard urotherapy, pelvic floor animated biofeedback therapy and pelvic floor exercises in a standard way and using a fitball. After the first session, patients received prescription to repeat at home the same exercises performed at hospital. Children who chose classic exercises were enrolled in group A and they who chose fitball in group B. Continence rate, pelvic floor muscles activity, adherence and satisfaction were evaluated by means of bladder diary, external pubococcygeus test and Likert-type psychometric scale (from 1 = very unsatisfied to 5 = very satisfied) respectively. RESULTS: Twenty-six children affected by LUTS were enrolled: 13 in group A and 13 in B. At 4th control urinary incontinence was reduced by 72,5% in A and 71.4% in B. Pubococcygeus test increased in both groups. Adherence at home was 92% in group A and 62% in group B. Satisfaction with the treatment (4 or 5 points) was 95% in both groups. Four patients of group A and all of group B decided to maintain home exercises including fitball. CONCLUSIONS: Our preliminary study shows that efficacy of home exercises, with or without fitball, is comparable. Satisfaction with fitball is high, regardless results obtained, because parents and children have learned a new, interesting and stimulating way to manage urinary problems. The opportunity to perform these exercises using fitball, it's an important finding for pediatric population.

Anorectal malformation, urethral duplication, occult spinal dysraphism (ARM-UD-OSD): a challenging uncommon association.

PURPOSE: Urethral duplication (UD) is a rare malformation, which can be associated with other anomalies, like anorectal malformations (ARM). ARM has been described with occult spinal dysraphism (OSD). No ARM-UD-OSD combination has been reported. AIM: To share our experience and to discuss the management of ARM-UD-OSD association. METHODS: We retrospectively reviewed records of five boys with UD. Four of these had ARM-UD-OSD association. ARM was the first diagnosis in all; OSD and UD was detected during screening for associated malformation. RESULTS: All patients underwent ARM correction, 3 after colostomy. All reached fecal continence, 3 are performing bowel management. Three patients underwent UD surgical correction. Because of symptoms' worsening, 2 children had detethering surgery. At a mean follow-up of 9.5 years, all patients have normal renal function, 3 are on clean intermittent catheterization (CIC) for neurogenic bladder (1 has a cystostomy, another one an appendicostomy). CONCLUSIONS: UD and OSD should be considered in patients with ARM. Children with these conditions associated must be centralized in a third-level Center and management carefully planned; in particular, urethral reconstruction should be weighed, considering CIC could be required. Suspicion of neurogenic bladder must be present in OSD patient.

Posterior urethral valves: Role of prenatal diagnosis and long-term management of bladder function; a single center point of view and review of literature.

Posterior Urethral Valves (PUV) are the most common cause of lower urinary tract obstruction. More severe forms are detected early in pregnancy (mainly type I), while other forms are usually discovered later in childhood when investigating lower urinary tract symptoms. Bladder dysfunction is common and is associated with urinary incontinence in about 55% (0%-72%). Despite the removal of the obstruction by urethral valve ablation, pathological changes of the urinary tract can occur with progressive bladder dysfunction, which can cause deterioration of the upper urinary tract as well. For this reason, all children with PUV require long-term follow-up, always until puberty, and in many cases life-long. Therefore, management of PUV is not only limited to obstruction relief, but prevention and treatment of bladder dysfunction, based on urodynamic observations, is paramount. During time, urodynamic patterns may change from detrusor overactivity to decreased compliance/small capacity bladder, to myogenic failure (valve bladder). In the past, an aggressive surgical approach was performed in all patients, and valve resection was considered an emergency procedure. With the development of fetal surgery, vesico-amniotic shunting has been performed as well. Due to improvements of prenatal ultrasound, the presence of PUV is usually already suspected during pregnancy, and subsequent treatment should be performed in high-volume centers, with a multidisciplinary, more conservative approach. This is considered to be more effective and safer. Primary valve ablation is performed after clinical stability and is no longer considered an emergency procedure after birth. During childhood, a multidisciplinary approach (pediatric urologist, nephrologist, urotherapist) is recommended as well in all patients, to improve toilet training, using an advanced urotherapy program with medical treatments and urodynamic evaluations. The aim of this paper is to present our single center experience over 30 years.

Button Cystostomy: Is it Really a Safe and Effective Therapeutic Option in Pediatric Patients With Neurogenic Bladder?

OBJECTIVE: To define safety and effectiveness of cystostomy button in the management of bladder drainage in pediatric patients with neurogenic bladder, and report our personalized surgical technique. MATERIALS AND METHODS: This study is a retrospective analysis of patients undergoing cystostomy button placement for bladder drainage from October 2009 to December 2015. Endoscopic and open surgical techniques and medium-term complication were analyzed and indications were recorded. RESULTS: Thirty-five patients, 16 (45.7%) females and 19 (54.3%) males, underwent cystostomy button placement for bladder drainage with a mean age of 8.6 ± 4.8 years (standard deviation) and a mean follow-up time of 37 months. There were 91.4% of patients who had a neurogenic bladder; a nonobstructive urinary retention was diagnosed in the remainder of cases. A medium-term complication was mostly represented by urinary tract infection observed in 10 of 35 patients that was the most representative cause of button removal (4 of 35). Other observed complications were button leakage (n = 2), decubitus (n = 1), and bladder stone (n = 1). No postoperative complication was observed and no differences were found in terms of complications in the two surgical approaches performed. CONCLUSION: Cystostomy button is a safe and effective treatment for bladder drainage in neurogenic pediatric patients and it is also well accepted by patients and caregivers. Cystostomy button, which may avoid mechanical concerns and most of the social discomfort, should be considered an alternative method to other bladder drainage modalities.

Long-term bladder function followup in boys with posterior urethral valves: comparison of noninvasive vs invasive urodynamic studies.

PURPOSE: We studied whether noninvasive urodynamic evaluation can be as effective and safe as invasive urodynamics in detecting lower urinary tract dysfunction and in preventing late onset renal failure during long-term management of boys with posterior urethral valves. MATERIALS AND METHODS: We evaluated 47 boys with posterior urethral valves using repeat urodynamics. A total of 28 patients with followup of at least 3 years and repeat evaluation of serum creatinine were included in the study. The first 14 boys in the series underwent cystometry and pressure-flow study at least every 3 years (group A), and the remaining 14 patients were monitored annually from age 5 with bladder diary, uroflowmetry, post-void residual urine on ultrasound and serum creatinine (group B). Lower urinary tract dysfunction and serum creatinine were compared (Fisher exact test and Mann-Whitney test) between groups A and B, and by stratifying patients into subgroups by age (5 to 6, 7 to 13 and older than 13 years). In all patients urodynamic diagnosis of lower urinary tract dysfunction was matched and confirmed with lower urinary tract symptoms. RESULTS: During followup the prevalence of lower urinary tract dysfunction did not differ significantly between group A (71% in boys 5 to 6, 43% in boys 7 to 13 and 85% in boys older than 13 years) and group B (36%, 43% and 60%, respectively). Late onset renal failure was observed in 2 boys in group A and 2 in group B. CONCLUSIONS: Noninvasive urodynamic evaluation seems to be as safe and effective as invasive urodynamic study in the long-term management of boys with posterior urethral valves. Based on these findings, invasive urodynamics may be reserved for cases of progressive deterioration of lower urinary tract dysfunction or renal function.

A simplified technique for botulinum toxin injections in children with neurogenic bladder.

PURPOSE: Botulinum toxin type A has revolutionized the treatment of neurogenic bladder dysfunction. The original injection technique used a rigid cystoscope and a flexible collagen needle. To date botulinum toxin type A injection techniques have not been standardized. We present our experience in pediatric patients using a new flexible injection system. MATERIALS AND METHODS: We treated 24 patients 3.8 to 17.5 years old who had neurogenic bladder dysfunction with botulinum toxin type A bladder and/or sphincter injection using a rigid cystoscope and the new N-DO™ endo-injector needle system. Another 24 patients 3.6 to 17.8 years old were treated with a 3.7Fr standard flexible needle and served as controls. Operative time, hospital stay, complications and efficacy were considered. Selection criteria and treatment were the same in the 2 groups. The 10 IU/kg dose was determined according to European Association of Urology guidelines. RESULTS: All patients received botulinum toxin type A bladder injection while 11 patients in the endo-injector group and 5 controls also received urethral injection. In the endo-injector needle and control groups average operative time was 12.4 and 17.3 minutes for the bladder, and 5.1 and 10.1 minutes for the urethra, respectively (each p <0.05). All patients were discharged home the day after the procedure. No complications were observed. Urodynamics revealed an average maximum detrusor pressure decrease of 25 and 21 cm H(2)O, and an average bladder capacity increase of 75 and 80 ml in the endo-injector and control groups, respectively (p not significant). CONCLUSIONS: While retaining efficacy, the endo-injector needle technique appears to be more rapid than the standard procedure for botulinum toxin type A injection for neurogenic bladder dysfunction. Whether patients may be treated with sedation only remains to be clarified.

Current state of nerve stimulation technique for lower urinary tract dysfunction in children.

PURPOSE: A variety of electrical nerve stimulation methods has been used through the years to treat lower urinary tract dysfunction. Relevant literature was reviewed to analyze techniques and available biomedical devices, technique applicability, indications and usefulness in pediatrics. MATERIALS AND METHODS: An extensive search was performed on PubMed® and MEDLINE® for scientific publications on intravesical, transcutaneous, sacral spine and root, and tibial nerve stimulation in children with lower urinary tract dysfunction of nonneurogenic and neurogenic origin. Relevant articles and controlled studies in adult patients were also considered. The search covered the period 1990 to 2009 and we found approximately 400 articles, of which 29 related to pediatrics. RESULTS: Due to feasibility problems with placebo studies the majority of the studies were noncontrolled, some of them clinical trials on acute urodynamic changes during electrical stimulation or experimental research in animals. Overall only a few randomized trials were found. Regarding types of electrostimulation and indications in children the recent literature emphasizes stimulation far from the anal-genital region, such as sacral transcutaneous electrical nerve stimulation, mainly for refractory overactive bladder. Intravesical stimulation is the procedure of choice to enhance sensation in patients with incomplete neurogenic lesions. Percutaneous tibial nerve stimulation is tolerated by children but has been poorly studied. Sacral neuromodulation using implanted devices remains questionable and needs further clarification of its indications. Magnetic stimulation has rarely been used in children to date. More experimental studies are needed to assess the method of action and refine the parameters of stimulation. CONCLUSIONS: Clinical controlled trials vs sham devices and predictable variables for successful response are urgently needed to address an apparently renewed focus on the use of nerve stimulation in the treatment of pediatric lower urinary tract symptoms.

Validity of the international consultation on incontinence questionnaire-pediatric lower urinary tract symptoms: a screening questionnaire for children.

PURPOSE: Lower urinary tract symptoms are common in pediatric patients. To our knowledge no validated instruments properly designed to screen lower urinary tract symptoms in the pediatric population have been published to date. In the International Consultation on Incontinence Questionnaire Committee the psychometric properties of a screening questionnaire for pediatric lower urinary tract symptoms were assessed. MATERIALS AND METHODS: The 12-item International Consultation on Incontinence Questionnaire-Pediatric Lower Urinary Tract Symptoms was developed in child and parent self-administered versions, and produced in English, Italian and German using a standard cross-cultural adaptation process. The questionnaire was self-administered to children 5 to 18 years old and their parents presenting for lower urinary tract symptoms (cases) or to pediatric/urological clinics for other reasons (controls). A case report form included history, urinalysis, bladder diary, flowmetry/post-void residual urine volume and clinician judgment on whether each child did or did not have lower urinary tract symptoms. Questionnaire psychometric properties were evaluated and data were stratified into 3 age groups, including 5 to 9, 10 to 13 and 14 to 18 years. RESULTS: A total of 345 questionnaires were completed, of which 147 were negative and 198 were positive for lower urinary tract symptoms. A mean of 1.67% and 2.10% of items were missing in the child and parent versions, respectively. Reliability (Cronbach's α) was unacceptable in only the 5 to 9-year-old group. The high ICC of 0.847 suggested fair child/parent equivalence. Sensitivity and specificity were 89% and 76% in the child version, and 91% and 73.5% in the parent version, respectively. CONCLUSIONS: The questionnaire is an acceptable, reliable tool with high sensitivity and specificity to screen for lower urinary tract symptoms in pediatric practice. Problems related to literacy suggest use of the child versions for patients older than 9 years. In research this questionnaire could be used to recalibrate the prevalence of lower urinary tract symptoms in children.

Long-term efficacy of percutaneous tibial nerve stimulation for different types of lower urinary tract dysfunction in children.

PURPOSE: We evaluated the efficacy of percutaneous tibial nerve stimulation for different types of pediatric lower urinary tract dysfunction. MATERIALS AND METHODS: A total of 14 children with idiopathic overactive bladder, 14 with dysfunctional voiding, 5 with underactive bladder, 4 with underactive valve bladder and 7 with neurogenic bladder resistant to conventional therapy underwent percutaneous tibial nerve stimulation weekly for 12 weeks. The stimulation effect was evaluated by comparing bladder diary, flowmetry and urinalysis before and after treatment. Improved patients were followed by bladder diary and urinalysis. Followup data at 1 and 2 years were compared with those obtained after stimulation. Data were analyzed using Fisher's exact test. RESULTS: Symptom improvement was significantly greater in nonneurogenic than in neurogenic cases (78% vs 14%, p <0.002). Of patients 18% with underactive bladder and 50% with underactive valve bladder were unresponsive. Of 14 overactive bladder cases 12 and all 14 of dysfunctional voiding were improved (p not significant). Of improved patients 5 of 12 with overactive bladder and 12 of 14 with dysfunctional voiding were cured (p <0.01). On uroflowmetry voided volume and post-void residual urine became normal in a greater number of dysfunctional voiding than overactive bladder cases (57% vs 20% and 57% vs 25%, each p not significant). At 1 year of followup the cure rate was greater in dysfunctional voiding than in overactive bladder cases (71% vs 41%) and it remained the same at the 2-year evaluation. Chronic stimulation was necessary to maintain results in 29% of dysfunctional voiding and 50% of overactive bladder cases. CONCLUSIONS: Percutaneous tibial nerve stimulation is reliable and effective for nonneurogenic, refractory lower urinary tract dysfunction in children. Efficacy seems better in dysfunctional voiding than in overactive bladder cases. There is evidence that percutaneous tibial nerve stimulation should be part of the pediatric urology armamentarium when treating functional incontinence.

Bladder after total urogenital mobilization for congenital adrenal hyperplasia and cloaca--does it behave the same?

PURPOSE: Followup of total urogenital mobilization for persistent urogenital sinus is well established anatomically and functionally. Nevertheless, studies comparing bladder function in different subsets of patients with urogenital sinus, such as congenital adrenal hyperplasia and cloaca, are scant. MATERIALS AND METHODS: We reviewed the records of patients with congenital adrenal hyperplasia and cloaca who underwent total urogenital mobilization and urodynamics in the last 10 years. Those with a short urogenital sinus (less than 2.5 cm) not requiring an abdominal approach and without spinal dysraphism were selected for study. Urodynamics were performed postoperatively before and after toilet training, and compared between patients with congenital adrenal hyperplasia and cloaca. Methods, definitions and units conformed to International Continence Society/International Children's Continence Society standards. For the emptying phase we defined bladder outlet obstruction as maximum detrusor pressure greater than 70 cm H(2)O and underactive detrusor as maximum detrusor pressure less than 20 cm H(2)O plus post-void residual urine greater than 25 ml. RESULTS: Six patients with congenital adrenal hyperplasia and 6 with cloaca met study criteria. Three patients with congenital adrenal hyperplasia and 4 with cloaca underwent urodynamics before and after toilet training at a median age of 2 (range 2 to 4) and 5 years (range 3 to 8), respectively. Urodynamics were done in 1 patient with congenital adrenal hyperplasia before toilet training, and in 2 with congenital adrenal hyperplasia and 2 with cloaca after toilet training. All patients had normal urodynamics except 1 with congenital adrenal hyperplasia and detrusor overactivity, which normalized after toilet training. In all cloaca cases urodynamics were abnormal. Before toilet training bladder outlet obstruction was found in 2 patients, detrusor underactivity was found in 1 and detrusor overactivity was found in the remaining 1. After toilet training a detrusor underactivity pattern was found in 4 patients and bladder outlet obstruction was found in 2. All patients except 1 with cloaca had post-void residual urine before and after toilet training (median 100 ml, range 25 to 200). After toilet training all patients with congenital adrenal hyperplasia became spontaneously dry and all with cloaca were placed on clean intermittent catheterization. CONCLUSIONS: In the long term patients with cloaca show bladder outlet obstruction or underactive/acontractile detrusor patterns, which are not noted in patients with congenital adrenal hyperplasia. Therefore, in patients with cloaca urogenital sinus length may not be as good an indicator of functional results as it is in patients with congenital adrenal hyperplasia. Whether additional rectal dissection and repositioning surgical procedures in cloaca cases may have a role in explaining such a difference remains to be clarified.

Reliability of bladder volume measurement with BladderScan in paediatric patients.

OBJECTIVE: To evaluate the reliability of estimates of bladder volume (BV) in children made with the BladderScan BVI 2500 (BS) and their agreement with standard ultrasound (US) measurements. MATERIAL AND METHODS: BV was measured using both US and BS in 92 children (41 females, 51 males; age range 3 months to 16 years) who underwent standard US measurements for various reasons. Patients were stratified into three groups according to age (3-35, 36-83 and > 83 months) and BV (< 20%, 20-50% and > 50% of expected bladder capacity for age). US and BS measurements were compared by means of the percentage difference and Pearson's correlation coefficient (r); limits of clinical agreement were evaluated by means of Bland-Altman analysis. RESULTS: Overall, a difference of -12.9% and a correlation coefficient of r = 0.98 were found between US and BS. The percentage difference was higher in younger patients (-27.8%) and for low volumes (-24.8%). Correlation analysis confirmed this trend in different age (3-35 months, r = 0.74; 36-83 months, r = 0.93; > 83 months, r = 0.97) and BV (< 20%, r = 0.70; 20-50%, r = 0.94; >50%, r = 0.97) groups. Bland-Altman analysis showed large limits of clinical agreement between the two methods in terms of overall measurements (-45 to 29.3 ml) and in both age (-25.3 to 56.9 ml) and BV (-27.5 to 52.5 ml) groups. CONCLUSIONS: A good correlation between US and BS measurements of BV was found in children aged > 7 years and in those with a BV > 20% of expected bladder capacity. Thus, BS avoids the need for standard US equipment to assess BV for schoolchildren with voiding dysfunction. Nevertheless, a dedicated BS instrument should be used in younger children.

Amniotic fluid index and fetal bladder outlet obstruction. Do we really need more?

PURPOSE: With the constant advances in technology and expertise of prenatal diagnosis, necessity of early counseling in cases of bilateral obstructive uropathy has become of paramount importance. To better evaluate fetal renal function new biochemical (serum and urine) fetal markers have been introduced in the literature. However, they are only available at select centers and always requiring an invasive approach. Furthermore, paucity of normal controls sometimes makes interpretation of results difficult. Owing to this growing interest towards biochemical evaluation of fetal renal function, assessment of amniotic fluid, which is mostly expression of fetal urination, has progressively fallen into disrepute, and studies comparing the amount of amniotic fluid with renal function are scant. MATERIALS AND METHODS: In a 3-year period 28 cases of bilateral obstructive uropathy were seen prenatally at the Artemisia Medical Center. All cases were initially reviewed at 17 to 20 weeks of gestation when a distended fetal bladder with thickened wall and enlarged kidneys were visualized. At the same time ultrasonographic assessment of amniotic fluid was performed by calculating the amniotic fluid index. An amniotic fluid index less than the 25th percentile was considered below average and an index less below the 5th percentile was considered oligohydramnios, whereas an index between 50th and 75th percentiles was considered normal. All cases were subsequently evaluated for renal function up to age 1 year. Impaired renal function was defined as a serum creatinine greater than 1.2 mg/dl before age 1 year. RESULTS: Of the 28 cases 18 had an index of oligohydramnios (group 1) and 10 had a normal index (group 2). No significant variations were observed in amniotic fluid index at repeated consultations throughout pregnancy. Two cases in group 1 and 1 case in group 2 were lost to followup while in the other group 1 cases intrauterine death occurred. Bilateral obstructive uropathy was confirmed in all instances after birth as valves in 18 cases and urethral atresia in 3. Of the 12 surviving group 1 cases there were 3 neonatal deaths from severe lung hypoplasia, and in the remaining 9 cases mean serum creatinine at each evaluation before age 1 year was 1.3 +/- 0.2 mg/dl. All patients in group 2 survived with a mean serum creatinine at each evaluation of 0.6 +/- 0.1 mg/dl (p <0.05). CONCLUSIONS: Despite widespread use of prenatal biochemistry, evaluation of amniotic fluid by the amniotic fluid index remains a reproducible and inexpensive method to predict renal function in cases of bilateral obstructive uropathy of any origin. It retains its validity not only in severe, but also in milder reductions. Conversely, intact amniotic fluid mostly invariably predicts normal renal function at long-term evaluation. For a better understanding of the disease such information is to be promptly conveyed to the prospective parents at each prenatal consultation.

How to investigate neurovesical dysfunction in children with anorectal malformations.

PURPOSE: Neurovesical dysfunction (NVD) is common in children with anorectal malformation (ARM). NVD is mainly related to tethered cord or iatrogenic injury but how to investigate it is still debated. We evaluate the usefulness of routine magnetic resonance imaging (MRI) and urodynamics (UDS) for ARM. MATERIALS AND METHODS: A total of 89 children were screened for sacral, spinal or urological anomalies using sacrum x-ray, MRI, renal and spinal ultrasound, uroflowmetry and/or 4-hour voiding observation. UDS was performed in 60 patients with suspected NVD. Mean +/- SD followup was 9.8 +/- 5.2 years. RESULTS: Of the 89 patients 29 presented with urinary tract anomalies. The prevalence of sacral (53 cases) and spinal cord (54) anomalies was no different between patients with low, intermediate and high ARM. Spinal cord tethering was present in 13 patients with a normal sacrum x-ray. NVD was found in 31 of the 89 patients (hyperreflexia 21 and hypo-areflexia 10), and was associated with sacral and spinal anomalies in 23, occult spinal dysraphism without bone lesion in 3 and sacral anomalies in 5. The incidence of NVD was 40% of cases with low and 51% with high ARM. CONCLUSIONS: Because tethered cord occurs in children without sacral anomalies as well as in those with low ARM, we recommend evaluation of all patients using MRI. When MRI is positive UDS should be performed. We agree with a previous suggestion to evaluate all males with rectourethral fistula and females with cloaca malformations. Finally we recommend a noninvasive evaluation for all other children and UDS when neurogenic dysfunction is suspected.