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Abdominal radiographs are frequently used for evaluation of bowel and bladder dysfunction in pediatric urology. However, the dose of radiation delivered with each study is estimated from machine settings as opposed to measurement of the true entrance skin dose. In addition, the correlation of radiographic constipation with patient symptoms has been questioned. To evaluate the practices for obtaining abdominal radiographs and the true entrance skin dose of radiation for each examination in order to identify targets for radiation reduction. Pediatric urology patients were prospectively enrolled from June 2022 through June 2023. Dosimeters were attached to the navel to collect entrance skin doses from single view abdominal x-ray. Estimated doses were compared to measured entrance skin dose as well as patient characteristics. Exam parameters were evaluated to identify targets for radiation reduction. A total of 75 patients were recruited for this study with a median age of 10.0 years (IQR 6–14). Most evaluations were done to assess for bowel and bladder dysfunction (68 exams, 91%). The protocol for exams was not standardized resulting in 27% of patients undergoing a medium or high dose strength and 55% undergoing 1 or more image. The median estimated dose was 0.63 mGy (IQR 0.3–1.2 mGy). The median measured dose was 0.77 mGy (IQR 0.31–2.01 mGy) which was significantly different than the estimations (p < 0.001). The estimated dose, measured dose and estimate error were all found to be positively correlated with patient characteristics including age and body mass index (See Figure). Increasing age and body mass index also showed a higher likelihood of increased dose strength and image acquisition. The measured entrance skin dose of radiation is significantly higher than prior estimates. The measured dose but also the estimate error increased with patient age and size which is likely related to higher settings used for image acquisition as patients age. Standardized protocols using low dose settings and limiting image acquisition to the pelvis may reduce radiation exposure in children with bowel and bladder dysfunction while providing adequate diagnostic data. Radiation dose for abdominal radiographs is higher than previously estimated. Older and larger children received higher doses which may be mediated by increased dose strength and image acquisition. Standardization of protocols could lower radiation exposure. [ABSTRACT FROM AUTHOR]

Pediatric patients with lower urinary tract dysfunction (LUTD) experience a variety of medical and psychosocial concerns that can negatively impact their clinical management and quality of life. When initially described by Drs Hinman and Baumann, patients with a form of severe LUTD, later dubbed Hinman syndrome, were characterized as having a "general attitude of being failures." While this mention was noteworthy as it incorporated an understanding of the interplay between psychological factors and urologic conditions, there have been delays in implementing psychological intervention as a standard of care in patients with LUTD broadly, and perhaps too keen of a focus on youth diagnosed with Hinman syndrome specifically. A non-systematic reviewed of LUTD related to psychological management was performed. Clinical recommendations were developed by a multicenter and multidisciplinary team of care providers with topic expertise. The aim of this paper is to propose a systems thinking paradigm for how to involve psychology, or psychological principles, across the spectrum of patients with LUTD with the hope of improving attention to specific aspects of care that may improve clinical management. Children across the spectrum of LUTD are likely to benefit from psychological interventions and would benefit from such involvement early on to mitigate the impact of psychosocial concerns on medical outcomes. Pediatric psychologists are well-suited to identify and provide individualized care to patients in greatest need of intervention, such as through pre-procedural preparedness, addressing non-adherence, and with the use of evidence-based, targeted mental health interventions. Psychologists are also apt at implementing interventions while taking into consideration the severity of LUTD, in the context of the patient's psychological, developmental, cultural, familial, and social determinants of health considerations. Youth with severe forms of LUTD, such as Hinman syndrome, likely exemplify the challenges of LUTD that benefit from multidisciplinary intervention. Medical and psychological collaboration are key to ensuring symptom mitigation and emotional support for patients across the entire spectrum of LUTD. [ABSTRACT FROM AUTHOR]

Uroflowmetry is a non-invasive study used in the diagnosis and monitoring of treatment response for lower urinary tract disorders. For optimal clinical utility, uroflow studies rely on careful clinical interpretation by a trained provider, but currently there is a lack of accepted standardized normal values for the measured uroflow parameters in children. The International Children's Continence Society proposed standardizing the terminology for uroflow curve shapes. However, the patterning of curves is largely at the physician's subjective discretion. The aims of this study were to understand inter-rater reliability in interpreting uroflow curves and to define characteristics of uroflow curves that could be used to provide definitive criteria for uroflowmetry parameters. All members of the SPU Voiding dysfunction Task Force were invited to contribute deidentified uroflows to a centralized HIPAA complaint receiving database. All studies were then distributed to all raters for review. Each observer's findings were recorded according to ICCS criteria (ICCS), additional readings were done using a previously reported system which defined curves as smooth or fractionated (SF) and whether the shape resembled a bell, tower, or plateau (BTP). Flow indexes (Qact/Qest) (FI) for Qmax and Qavg were generated using formulas previously reported for children 4–12 and for patients≥12 years. A total of 119 uroflow studies were read by 7 raters and curves were contributed from 5 sites. Kappa scores for the 5 readers from different institutions were 0.34 and 0.28 for the ICCS and BTP methods, respectively (both considered fair agreement). Kappa for smooth and fractionated curves was 0.70 (for each; considered substantial agreement), which were the two highest agreement scores obtained throughout the study. Discriminant analysis (DA) revealed that the FI Qmax was the dominant vector, and that the ICCS uroflow parameters have a total of 42.8% prediction rate in the training sample. Using DA of a smooth/fractionated system, the total prediction rates were 72% and 65.5% for smooth and fractionated, respectively. Given the poor inter-rater agreement for analyzing uroflow curve pattern based on ICCS criteria in this study and others, one may find it reasonable to consider alternative approaches to describing and characterizing uroflow curves. Our study is limited by lack of EMG and post-void residual data. For a more objective uroflow interpretation and comparison of studies among different centers, we recommend using our proposed system (based on FI, and smooth vs. fractionated curve pattern), which is more reliable. [Display omitted] [ABSTRACT FROM AUTHOR]

Syringocele is a rare cystic dilatation of the duct of Cowper's gland, afflicting mostly the pediatric population. Syringoceles have a wide range of symptoms and may cause urethral obstruction. The authors analyzed to clarify the clinical manifestation, diagnostic approach, management, and incidence in the pediatric population. All patients (122 cases) diagnosed with a syringocele at the department of Pediatric Urology in a tertiary referral university children's hospital between August 1991 and October 2016 were analyzed retrospectively by assessing medical charts. The clinical manifestation, diagnostic findings, and follow-up are summarized in the table. Half of the patients (50.0%) also had typical posterior urethral valves (PUVs) and/or a single valve in the 12 o'clock position (flap-valve). The symptoms of open and closed syringoceles showed no significant difference. Treatment consisted of incision of the syringocele with a diathermia hook. The incidence of urinary tract infection (UTI) before and after surgery in the group that had a syringocele only was significant different. The overall incidence of syringoceles seen at urethrocystoscopy in this series was 3.0%. This series suggests that the presenting age is strongly related to the consequences of syringoceles, as the youngest half of the patients had significantly more UTIs at presentation than older patients, who presented with significantly more obstructive voiding symptoms, postvoiding residuals, and incontinence. In addition, the younger group had a significantly higher incidence of vesicoureteral reflux and dilatation of the upper urinary tract. The found association between syringoceles and PUV may be due to overgrowth of epithelium, as possible origin in both anomalies. With an incidence of 3.0%, syringoceles, in this tertiary referral series, should be considered in the differential diagnosis of obstructive urethral lesions. The presentation ranges between signs of severe obstructions in the prenatal and postnatal period to mild urinary incontinence problems at later age. Urethrocystoscopy proved to be useful in confirming the diagnosis and allows for immediate transurethral incision. Summary Table Table Clinical manifestation Most frequent presenting symptoms Urinary tract infection (UTI) (50.0%), Obstructive voiding symptoms (45.9%) Postvoiding residual urine (13.9%) Median age at diagnosis 3.0 (IQR: 0.0–9.0) years Diagnostics (UCS) − 83 closed syringoceles and 31 open syringoceles. − 8 patients had two coexisting syringoceles. − 22 patients had a syringocele without additional urethral abnormalities. Follow-up Median follow up 24.0 (IQR: 7.00–52.50) months 42 (34.4%) patients were symptom-free directly after surgery. 23 (18.9%) patients needed a second incision of the rim of the opened syringocele. [ABSTRACT FROM AUTHOR]

Summary Objective Bowel and bladder dysfunction (BBD) is a clinical syndrome defined by the coexistence of constipation and lower urinary tract symptoms. Although BBD is a common condition in pediatric urology and can cause significant stress to patients/parents, clinical diagnosis of BBD has not been standardized. Bowel and bladder dysfunction instruments have gained popularity over the past decade to aid in diagnosis. In this review, the currently published BBD symptom questionnaires were summarized and an analysis on their psychometric validation process was provided. Subjects/patients PubMed was searched for articles on BBD symptom questionnaires/instruments/surveys since 2000. Two investigators (R.Y.J. & M.S.K.) conducted the search in duplicate. The search was limited to English language. The included search terms were 'dysfunctional elimination syndrome', 'bowel and bladder dysfunction', 'dysfunctional voiding', 'voiding dysfunction symptom score', and 'dysfunctional voiding symptom scale'. Reference lists of included studies were screened for missed studies. Unpublished abstracts presented at the following scientific meetings were also manually searched for: the American Urological Association, the Society for Pediatric Urology, and the American Academy of Pediatrics Section on Urology. Exclusion criteria included articles in languages other than English, articles not related to BBD questionnaires, and articles not pertaining to pediatric patients. Results After initial search, a total of 54 articles were obtained. Of the seven questionnaires reviewed, only one did not measure sensitivity or specificity. Almost all questionnaires showed excellent discriminative property with an Area under the curve (AUC) >0.85. Criterion validity was not reported in any of the questionnaires. Known-group validation was used as a common method to evaluate construct validity. Internal consistency was reported in 2 studies, and only 1 study measured questionnaire responsiveness during initial validation. Conclusions Although BBD is a common pediatric urology condition; a large amount of heterogeneity exists in the questionnaires' psychometric testing and validation process. To further improve the diagnosis and management of BBD in pediatric patients, there is a need for consensus on the gold standard questionnaire measure. [ABSTRACT FROM AUTHOR]

Non-neurogenic lower urinary tract dysfunction (LUTD) is one of the most common reasons for presentation to a pediatric urologist, affecting up to 20% of children. Predicting who will benefit from RBUS as part of their work-up is challenging as the majority will have normal imaging. Our objective was to assess the utility of using the Dysfunctional Voiding and Incontinence Scoring System (DVISS) and urinary tract infection (UTI) history to predict which LUTD patients were most likely to have an abnormal RBUS as well as determine a DVISS cutoff to aid in making this prediction. We hypothesized that higher DVISS scores and a positive urinary tract infection (UTI) history would be associated with increased likelihood of RBUS abnormality. We retrospectively reviewed outpatients seen for LUTD from 5/2014-1/2016 who received an RBUS. Association between prior UTI, DVISS score, gender, and race and RBUS abnormality were evaluated using logistic regression analysis. Receiver operating characteristic (ROC) curves were created to evaluate the predictive model and a Youden index calculated to determine the optimal cutoff for DVISS score to predict abnormal RBUS. 15 of 333 patients (4.5%) had a clinically significant RBUS abnormality. Significantly more patients with abnormal RBUS had a positive UTI history and median DVISS was higher. UTI history and DVISS score were associated with RBUS abnormality whereas neither gender nor race were. A DVISS score cutoff of 12 was determined to be ideal for predicting abnormal imaging. Using DVISS≥12 and positive UTI history, patients with both risk factors were significantly more likely to have an abnormal RBUS than those with zero or one risk factor (Figure). To the best of our knowledge this is the first study to try to identify risk factors associated with RBUS abnormality in pediatric LUTD patients and create an evidence-based approach to imaging these patients. We found both DVISS cutoff ≥12 and positive UTI history to be useful to risk stratify LUTD patients' likelihood of abnormal RBUS. Limitations include the study's retrospective nature as well as the fact the population was drawn from a tertiary care pediatric hospital with a large referral population and the fact that the decision to order an RBUS was based on individual clinician preference and decision making. We found that DVISS score≥12 and UTI history are useful in guiding the decision to obtain RBUS in pediatric LUTD patients. [ABSTRACT FROM AUTHOR]

Little is known about the effect of catheterization during pressure-flow studies (PFS) on voiding efficiency in children. Our objective was to determine the effect of urethral catheterization on Qmax and flow index (FI) during PFS compared to the free flow of uroflowmetry (UF). We retrospectively reviewed 63 consecutive children who underwent UF and PFS at our center on the same day (2019–2022). Voiding data was available for 46 patients. Patients first underwent a UF with full bladder, then PFS after urethral catheter insertion. Patients with urethral pathologies (n = 6), on clean intermittent catheterization (CIC) (n = 2) and with major comorbidities (n = 2) were excluded. Indications for UF/PFS were LUTS, recurrent UTIs, incontinence or neurosurgical pre-operative evaluation. Data was collected from the UF and the PFS and compared using paired t-test. The idealized Qmax and flow index (FI) were calculated for UF and PFS using the formulas described by Franco et al.: Male Qmax = 11.26 + 0.0701(TBC [total bladder capacity]) − 0.0000513(TBC); Female Qmax = 10.723 + 0.073(TBC) − 0.0000423(TBC), FI = Actual Qmax /Expected Qmax (Franco and et al., 2016; Franco et al., 2018; Franco and et al., 2016). Median age was 7 years old (IQR 5–11). Twenty-one (40%) patients were male and 32 (60%) patients were female. Of the 53 patients, 3 boys and 4 girls (n = 7; 13%) were unable to void with the catheter in place during PFS but able to void after its removal. Of the remaining 46 cases, the Qmax during PFS was 5 mL/s slower than the Qmax recorded on the UF without catheter, representing a decrease of 29% (12.3 vs 17.3 mL/s; p < 0.0001). The impact of urethral catheter during PFS was more important in males vs females (Qmax decreased by 7.7 vs 3.3 mL/s, or 45 vs 19%). The mean FI during PFS was 44%, which was a 30% reduction compared to the 74% FI obtained with UF (p < 0.00001). In males, the FI decreased by 37% on PFS, whereas it decreased 26% in females, similar to the Qmax decrease. Voiding efficiency, as assessed by Qmax and FI, is decreased during PFS compared to uroflow studies. Our data documents for the first time the impact of urethral catheterization on pediatric voiding efficiency. Abnormal flow rates and elevated PVRs should be used to guide patient management only if obtained by uroflow. Prospective validation comparing free flow with PFS studies will help characterize the impact of urethral catheter relative to bladder pathology, age, gender and catheter size used. [ABSTRACT FROM AUTHOR]

The pediatric urinary microbiome (urobiome) has been studied in the context of healthy children and children with genitourinary pathologies including neuropathic bladder, urinary tract infection (UTI) and nephrolithiasis. Little is known about the urobiome of children with bladder and bowel dysfunction (BBD), a condition that is an established risk factor of UTI. We hypothesized that the symptoms of a child with BBD may be related to urobiome composition. To evaluate the urogenital urobiome's role in BBD, we compared the urogenital urobiomes of children with and without BBD. We performed a prospective case–control pilot study at a single large, academic children's hospital. Cases included toilet trained prepubertal females over 2 years of age with BBD established through a validated scoring system and controls included asymptomatic, presumably healthy, children. Children were excluded if they had symptoms or lab work consistent with a concurrent UTI or antibiotic course for any reason within the prior 14 days. We performed 16 S ribosomal RNA gene sequencing and expanded quantitative urine culture on clean catch urine samples. To compare within sample (alpha) diversity, we used the Kruskal–Wallis test. To compare between sample (beta) diversity, we calculated the Bray–Curtis distance and performed the PERMANOVA test. Data from 25 children with BBD and 8 asymptomatic controls were analyzed. The demographic and clinical characteristics of the two comparison groups were similar, though a higher proportion of Black children were included in the asymptomatic control group. Neither alpha diversity nor beta diversity was significantly different between the two groups. The core microbiome of the BBD group included all the genera in the core urogenital urobiome of the controls, plus additional genera associated with opportunistic infection and/or UTI, including Escherichia , Campylobacter and Streptococcus. The results of both the 16 S sequencing and expanded quantitative urine culture in this small study suggest that the urogenital urobiomes of children with BBD do not differ significantly from those of asymptomatic children. However, the core urogenital urobiome of children with BBD included genera associated with opportunistic infection and/or UTI. This study was limited by the sample collection method ("clean catch" midstream voided urine samples, which introduce the possibility of vulvovaginal contamination), small sample size, and unequal balance of patient characteristics between the two study groups. The urogenital urobiomes of children with and without BBD do not appear to significantly differ. Larger studies are needed to confirm these findings. [ABSTRACT FROM AUTHOR]

Summary Introduction To study the pathophysiology of dysfunctional voiding, we have previously developed a model of stress-induced voiding dysfunction. We have shown that cyclosporine A (CsA), an inhibitor of the Ca 2+ –calmodulin complex, can prevent social stress-induced urinary retention. However, treatment with cyclosporine has not had an effect on the increase in the stress peptide corticotrophin-releasing factor (CRF) in Barrington's nucleus, which is involved in the micturition pathway. Objective We now investigate whether cyclosporine administered after stress can reverse the abnormal voiding phenotype, and whether it has effects on the bladder wall itself, or on the stress response within Barrington's nucleus. Materials and methods Six-week old Swiss–Webster mice were exposed to aggressor males for 1 h a day, followed by 23 h of barrier separation. In a long-term trial, 1 month of stress was followed by single-cage housing for 6 months. In a separate CsA reversal trial, mice either received CsA in drinking water or had plain drinking water during 1 month of single-cage housing during recovery. Bladder contractile function was examined on a Guth myograph. Nuclear translocation of myocyte enhancing factor (MEF)-2 and NFAT (nuclear factor of activated T cells) in the bladder was assessed using electrophoretic mobility shift assays (EMSAs). The expression of CRF was determined in Barrington's nucleus using in situ hybridization. Results Voiding dysfunction persisted for up to 6 months after stress exposure while mice recovered in single-cage housing. In the CsA reversal trial, voiding patterns improved when they received CsA in water during single-cage housing following stress, whereas those that underwent single-cage housing alone had persistent abnormal voiding (Fig. A). There was no difference between CRF levels in Barrington's nucleus between reversal groups ( p = 0.42) (Fig. B), possibly indicating a direct effect on the bladder rather than a persistent stress effect. There were no differences in the contractility of bladder wall muscle. CsA decreased the nuclear translocation of MEF-2 and NFAT induced by stress (Fig. C,D). Conclusion CsA reverses stress-induced urinary retention, but does not change the stress-induced CRF increase in Barrington's nucleus. Furthermore, bladder smooth muscle contractility is unchanged by CsA; however, there are changes in the levels of the downstream transcription factors MEF-2 and NFAT. We suspect that additional CsA responsive neural changes play a pivotal role in the abnormal voiding phenotype following social stress. Figure (A) Average number of voids in 10-week-old control Swiss–Webster male mice, compared with number of voids after 1 month of stress, and after stress followed by 1 month of single-cage housing alone or 1 month of single-cage housing with CsA administration. (B) There was no significant difference between stress and recovery groups on fluorescent in situ hybridization analysis; however, all were significantly increased from control (C,D) Electrophorectic mobility shift assay (EMSA) performed for quantification of nuclear protein levels of myocyte enhancing factor 2 (MEF-2) and nuclear factor of activated T cells (NFAT) using infrared-labeled DNA probes. [ABSTRACT FROM AUTHOR]

Objective To investigate lower urinary tract dysfunction in pre-toilet trained infants with and without history of febrile UTI (f-UTI). Materials and Methods Pre-toilet trained infants with f-UTI (Group 1) from pediatric nephrology and urology clinics, and those without f-UTI (Group 2) from infant-care centers were enrolled for the present study. Infants in Group 1 underwent four-hourly (4-H) observations for at least one month after treatment for UTI. Voided volume (VV) and post-void residual urine (PVR) were measured by weighting diaper and suprapubic ultrasound after finishing voiding, respectively. Average PVR was defined as the mean value of PVR during 4-H observation. Interrupted voiding was defined as two or three voidings within 10 min. Voiding efficiency was defined as VV/(VV + PVR). Results The mean ages of Group 1 ( n = 64) and Group 2 infants ( n = 56) were 10.6 ± 7.5 months vs 10.2 ± 5.1 months, respectively ( p = 0.70). Group 1 infants had significantly higher voiding frequency (3.0times ± 1.2 vs 2.6times ± 0.9, p = 0.04), average PVR (14.5 ml ± 14.2 vs 8.9 ml ± 8.8, p < 0.01) and lower voiding efficiency (71.2% ± 20.5 vs 80.2% ± 18.5, p = 0.01) than Group 2. ROC curve analysis showed that the optimal cutoff values for PVR and voiding efficiency to differentiate Group 1 and Group 2 infants were 10 ml and 80%, respectively. Group 1 infants had significantly more repeat elevated PVR (≧ 10 ml) and repeat low voiding efficiency (≦ 80%) than Group 2 (44.8% vs 22.4%, p = 0.03; 62.0% vs 28.6%, p < 0.01, respectively). Conclusion Pre-toilet trained infants with f-UTI were associated with elevated PVR and lower voiding efficiency than normal controls. [ABSTRACT FROM AUTHOR]

Objective Social stress can suppress the voiding reflex, with resultant diminished voiding frequency and increased volumes. The calcineurin-NFAT (nuclear factor of activated T cells) pathway is important in memory development. It was hypothesized that interruption of the calcineurin-NFAT pathway might prevent social stress-induced voiding dysfunction. Methods Mice were subjected to social stress in an established resident-intruder model for 1 h, followed by 23 h of barrier separation. NFATc3, NFATc4 knockout (KO) and wild-type (WT) mice were studied. At two weeks, voiding patterns were collected; this was followed by sacrifice. Corticotropin-releasing factor (CRF) mRNA expression in Barrington's nucleus (BN) was determined by in-situ hybridization. Results Social stress decreased voiding frequency and increased voided volumes in WT strains. At baseline, NFATc3 KO mice showed decreased voids and increased volumes, while the NFATc4 KO mice resisted social stress. However, CRF mRNA increased in WT mice following social stress and was increased at baseline in NFATc3 KO mice. It was found that CRF mRNA did not increase following social stress in NFATc4 KO mice. The administration of CsA to WT mice normalized voiding patterns following social stress, albeit with no effect on CRF mRNA in BN. Conclusion Disrupting the calcineurin-NFAT axis by either genetic or pharmacologic approaches confers resistance to the development of social stress-induced voiding and dysfunction. [ABSTRACT FROM AUTHOR]

Objective Research has demonstrated the effect of adverse childhood experiences (ACEs) on later physiologic function and illness development. In the urologic literature, the relationship between bladder dysfunction and neuropsychiatric disorders is well documented. Observations in pediatric urology clinical practice suggest that a blend of these two areas of research can inform care of patients with voiding dysfunction. Methods Retrospective review of 216 patients seen in a single pediatric urology clinic by a single provider over a 24-month period. A descriptive, correlational study design was used to assess the extent to which ACEs and neuropsychiatric disorders affected resolution of symptoms when patients were treated with a bowel and bladder retraining program. Patients were selected using diagnostic codes related to voiding dysfunction and a retrospective chart review was conducted. Results A majority of patients who were seen for voiding dysfunction (60%) had at least one psychosocial factor. There is a greater prevalence of ACEs (51%) than neuropsychiatric disorders (25%). Children with either ACEs or neuropsychiatric disorders dropped out of treatment at a higher rate than those with neither. When factors were looked at separately, neuropsychiatric disorders were more likely to impede treatment progress than ACEs. Conclusions ACEs and neuropsychiatric disorders affect patients' ability to make progress with bowel and bladder retraining and to stay in treatment. Efforts specifically aimed at maintaining therapeutic relationships with patients who have ACEs are needed to fully treat this group, which typically has a high drop-out rate but high rate of resolution if they are able to stay involved in treatment. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: Urinary retention is a known complication of using an extravesical approach for ureteral reimplantation, especially in bilateral cases. The etiology may be secondary damage to pelvic nerves during ureteral dissection. Recent literature suggests that it is possible to visually identify these nerves during a robotic assisted laparoscopic approach. We performed an exploratory study to identify and document them in pediatric patients by means of electrophysiologic recordings. Material and methods: Seven consecutive patients undergoing robotic assisted laparoscopic ureteral reimplantation with extravesical approach were prospectively enrolled in the study. Following dissection of the ureter below the level of vas deferens in male and the uterine artery in female, staying close to the adventitia and approaching the ureterovesical junction, the fibers as described in human cadaveric studies were identified dorsomedial to the ureter and preserved. Stimulating and recording electrodes were passed through to record post-synaptic compound muscle action potentials of the bladder. Results: Even though the nerve fibers were visually identified, we were unable to consistently and reproducibly record compound muscle action potentials after stimulation of putative pelvic plexus fibers at the distal ureter, despite modulation in stimulation intensity, pulse characteristics, signal recording sensitivity and stimulator probe variation. Conclusions: In this pilot study, the inconsistent findings raise questions about the exact location of the neurovascular bundle, nature of bladder smooth muscle electrophysiology and the appropriate methodology of evaluation. This may provide a reason to reexamine the intraoperatively expected location of pelvic plexus nerve fibers. [Copyright &y& Elsevier]

Abstract: Introduction: Children with attention deficit hyperactivity disorder (ADHD) show an increased prevalence of enuresis and other daytime voiding symptoms (DVS). There is also some evidence toward an increased prevalence of enuresis among children with autism spectrum disorder (ASD), but with no data available with respect to DVS or response to medical treatment. The aim of this study was to assess enuresis and DVS, along with treatment outcomes, in children with ASD, to aid urological management. Methods: A retrospective observational study on the incidence of enuresis and other DVS in 671 children with/without ADHD/ASD was performed. Symptomatic improvement ≥50% was required to be considered positive. Complete resolution of symptoms for 3 months after cessation of treatment was considered cure. Results: Symptomatic improvement with desmopressin or anticholinergic treatment was seen in 76% of patients without ADHD/ASD, 85% of patients with ADHD, and 100% of patients with ASD. Cure was seen in 61% of patients without ADHD/ASD, 48% of patients with ADHD, and 50% patients with ASD. Mean time to cure was 9 months in those without ADHD/ASD (N = 319), 10 months in those with ADHD (N = 62), and 8 months in those with ASD (N = 10) (P = 0.69). Conclusion: Despite the small sample size of patients with ASD, our data show a favorable trend toward efficacy of desmopressin and anticholinergic therapy in these children with enuresis and DVS. [Copyright &y& Elsevier]

Abstract: Objective: The role of magnetic resonance imaging (MRI) in detecting occult spinal dysraphism (OSD) in children with voiding dysfunction and a normal neurological examination is still under debate. The aim of this study was to assess the correlation of sacral skin lesions with OSD detected on MRI, in a population of children with resistant lower urinary tract symptoms (LUTS). Patients and methods: A total of 114 children over 5 years of age with urinary tract infection (UTI) and/or LUTS and normal neurological examination were enrolled. All children underwent sacral neurological examination, urine analysis and cultures, renal/bladder ultrasound, voiding cystourethrogram and urodynamic examination. After a treatment period of 6 months, the patients were re-evaluated and spinal MRI was performed in 61 with ongoing LUTS or UTI. Results: Nineteen of 61 children (31%) had cutaneous stigmas. MRI detected spinal abnormality in 2/42 children with a normal sacral examination in comparison to 7/19 children with an abnormal sacral finding (Chi-squared test, P < 0.005). The sensitivity and specificity of an abnormal sacral finding in predicting MRI abnormality were 0.76 and 0.77, respectively. Urodynamic parameters did not predict an abnormal spinal MRI. Conclusions: Abnormal sacral findings, but not urodynamic studies, are strong predictors of OSD. A normal sacral examination does not rule out OSD. [Copyright &y& Elsevier]

Abstract: Objective: To determine the relationship between vesicoureteric reflux (VUR), urinary tract infection (UTI), renal damage and the pattern of non-neurogenic lower urinary tract dysfunction (LUTD), and to reveal the possible risk factors for renal damage in children with LUTD. Methods: For the years 2004–2010, demographic, clinical, laboratory and urodynamic study reports of children with LUTD were retrospectively reviewed. Results: Of 96 patients, there were diagnosed 70 with overactive bladder (OAB), 8 pure dysfunctional voiding (DV) and 18 OAB plus DV. The rate of VUR, UTI and renal damage in patients with OAB plus DV and pure DV was higher than in patients with OAB alone. VUR was significantly higher among the patients who had UTI. Renal scarring was detected in 25 patients, of whom 78% had OAB plus DV and 75% DV. The presence of VUR was associated with a significant increase in the rate of renal damage, and dilating reflux caused significantly greater damage compared to non-dilating reflux. Conclusion: OAB plus DV and DV are major risk factors for VUR, UTI and renal damage. The presence of VUR in children with LUTD plays an important role with regard to UTI and renal damage, with dilating VUR a major risk factor associated with renal damage. [Copyright &y& Elsevier]

Abstract: Purpose: To study the relationship between the age of completion of toilet training during daytime and the onset of dysfunctional elimination syndrome (DES). Methods: The present study was designed as a case-control study. A total of 80 patients with DES were allocated to the case group and 80 patients age- and gender-matched without DES were allocated to the control group. The patients with DES were seen in a tertiary center and the control patients were seen in a primary care center. Early completion of toilet training was considered to be before 24 months. Results: Patients were aged 3–17 years (mean: 7.55); 24 were male (30%) and 56 female (70%). The most frequent lower urinary tract symptoms were: urgency (77%), incontinence (75%), holding maneuvers (30%), high urinary frequency (61%) and low urinary frequency (16%). There was a history of urinary tract infection (UTI) in 62% of the DES group. The first UTI episode was before toilet training in 40%. The completion of toilet training was before the age of 2 years in 48% of DES patients and in 50% of the control group (p = 0.752) [odds ratio (OR) 0.91, 95% confidence interval (CI) 0.49–1.62]. Conclusion: The completion of toilet training before 24 months of age was not associated with DES. Some patients had DES symptoms such as UTI, infrequent voiding and constipation before toilet training. These findings suggest that DES may precede toilet training and this possibility should be confirmed in further studies. [Copyright &y& Elsevier]

Abstract: Non-neurogenic elimination disorders cover a wide spectrum of lower urinary tract and bowel dysfunctions, observed in the absence of a neurological background or lower urinary tract malformation. We reviewed conditions responsible for incontinence during bladder filling with normal voiding patterns (such as overactive bladder, giggle incontinence, post-void dribbling), and dysfunctional voiding syndromes. Dysfunctional elimination syndrome usually includes detrusor–sphincter dyscoordination, small-capacity overactive bladder or decompensated large poorly contractile bladder, and large-volume post-micturition residuals, occasionally associated with bowel dysfunction. At the most severe end of the spectrum lies the non-neurogenic neurogenic bladder syndrome, characterized by the association of a severe impairment of the upper urinary tract with a dysfunctional elimination syndrome. It must be emphasized that if the term ‘non-neurogenic’ relates to the absence of a neurological lesion, it is however conceivable that these conditions actually do have an underlying neurological cause that remains to be identified. [Copyright &y& Elsevier]

Vesicostomy is a simple, well-tolerated, and reversible procedure with few complications that safeguards upper urinary tract (UUT), decreases VUR grade, decreases UTI, and preserves renal function and should be considered in patients with PUV who have undergone prior valve ablation and bladder function not improved, and in myelodysplastic children that do not respond to catheter drainage [ 1–4 ]. We evaluated the temporary vesicostomy as a safeguard of the UUT in children with bladder outlet obstruction, bladder dysfunction, and high-grade VUR with sepsis and assessed the possible predictors of the UUT's morphological and functional improvement since these have been rarely explored in previous reviews. We evaluated the outcome and complications of temporary vesicostomy who were operated on 69 children at our center from 2014 to 2019. There were 63 (91.3%) boys and 6 (8.7%) girls who underwent vesicostomy at a mean age of 15.38 ± 2.74 months old. Twenty-nine cases (42%) were diagnosed as primary VUR, 23 (33.3%) had posterior urethral valve (PUV), and 5 (7.2%) had voiding dysfunction, while 12 (17.4%) were neurogenic bladder. Twenty-five (36.2%) patients were diagnosed prenatally and the remainder (44, 63.8%) postnatally. All patients were evaluated at least one year postoperatively. UTI was diagnosed in all cases before vesicostomy; 47 (68.1%) had a single episode of UTI and 22 (31.9%) had recurrent episodes of UTI. Mean serum creatinine was 1.75 ± 0.13 (0.7–4.8) mg/dl. Serum creatinine and the rate of UTI significantly improved (p-value <0.01). Seven (10.1%) cases were complicated with prolapse, 8 (11.6%) with stenosis, and 10 (14.5%) with peristomal dermatitis, all of them were males. About 75.4% of cases developed morphological improvement, while 24.6% of cases not improved (p-value = 0.0001). Improvement or stability of glomerular filtration rate (GFR) was seen in 84.1%, while 15.9% deteriorated GFR (p-value = 0.0001). This deterioration is associated with prenatal renal dysplasia. Age less than one year, abdominal swelling, severe HUN, grade V VUR and recurrent UTI before vesicostomy all independently affect functional improvement after vesicostomy. Vesicostomy is a simple, reversible, and well-tolerated surgery with few complications that is indicated in children with bladder outlet obstruction, bladder dysfunction, and high-grade VUR to protect UUT, improve renal function, decrease VUR, hydronephrosis, and febrile UTI. Age less than one year, abdominal swelling, severe HUN, grade V VUR and recurrent UTI before vesicostomy all were predictors that independently affect morphological and functional outcomes after vesicostomy. [ABSTRACT FROM AUTHOR]

Abstract: Objective: Voiding disorders are commonly encountered in paediatric urology practice. Urinary incontinence often leads to the impairment of self-esteem but can also cause renal damage, when recurrent urinary tract infection (UTI) or vesico-ureteral reflux (VUR) exists. The aim of this study was to assess the efficacy of a biofeedback training program in children with a long history of voiding disorders. Patients and methods: Between 1998 and 2002, 60 children with voiding disorders without neuropathic disease, aged 5–14, were treated. There were 48 girls and 12 boys. The main symptoms were daytime incontinence for 90%, urge syndrome for 78%, and night-time incontinence for 60%. UTIs were noted in 62% of the children, for 37% of them in association with a VUR. Ten weekly sessions were planned for all the children. They were given instructions on toilet behaviour and posture, and pelvic floor training. They had to supervise their voiding frequency and liquid intake at home using a chart. Biofeedback procedure used surface perineal electrodes. The exercises focused on relaxation of the perineum. Results: The first results were recorded 6 months after the last session: 96% of the children with daytime incontinence and 83% of the children with night-time incontinence were cured or improved; 84% of the children were free from infection and VUR was cured in 50% of cases. After a mean follow-up of 21 months, 8% of the children with daytime incontinence and 33% with night-time incontinence relapsed, with a significant difference between primary and secondary enuresis. A breakthrough UTI was observed in 19% of cases. Conclusion: This non-invasive training program was effective in the treatment of daytime incontinence, UTI and VUR. There was an improvement in secondary but not primary enuresis. To prevent relapse, additional support sessions seem to be necessary. [Copyright &y& Elsevier]

Abstract: Objective: To evaluate the long-term response to high-intensity, short-term biofeedback in children with severe voiding dysfunction. Patients and methods: We retrospectively reviewed patients who underwent short-term, high-intensity biofeedback therapy from 1996 to 2004. Improvement was classified based on clinical and radiographic findings. Patients were categorized as having Hinman''s syndrome when, in addition to urinary incontinence, at least four of the following categories were present: sphincter dyssynergia, bladder trabeculation, large post-void residual (PVR), hydronephrosis, vesicoureteral reflux (VUR) and urinary tract infections. There were 14 patients (eight males and six females), 13 of whom had Hinman''s syndrome. Age when biofeedback was initiated varied from 5.6 to 12.9years (μ =8.9±2.2). Before biofeedback, all had large PVRs, bladder trabeculation and sphincter dyssynergia. Nine had hydronephrosis and five had VUR. One patient had renal failure. Results: Before biofeedback, the mean PVR was 109ml (25–270ml); after biofeedback, this decreased to 21ml (0–150ml), including two patients who eventually failed treatment. All 14 patients were able to relax their external sphincter and reduce the PVR during biofeedback and on short-term follow up. Long-term follow up (μ =59.4months) in 12 patients established that seven had a durable response with remission of symptoms, reduced PVR and radiographic improvement. In three, symptoms partially recurred over time and two failed treatment completely. Conclusion: Short-term, high-intensity biofeedback achieves a durable response in the majority of children with Hinman''s syndrome. Long-term follow up is needed to assure compliance. [Copyright &y& Elsevier]

Abstract: Purpose: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare, congenital and usually fatal condition of unknown etiology. It is characterized by abdominal distension caused by a distended, non-obstructed urinary bladder and intestinal hypoperistalsis with functional intestinal obstruction. Previous studies reported vacuolar degenerative changes in the smooth muscle cells of bowel and bladder suggesting that MMIHS may be due to a visceral myopathy. The aim of this study was to examine the expression of contractile, cytoskeletal and extracellular matrix proteins in the detrusor muscle of MMIHS patients. Material and methods: Bladder specimens were obtained from six MMIHS patients. Normal bladder specimens were obtained during partial cystectomy and served as controls. Single fluorescence immunohistochemistry for α-smooth muscle actin (SMA), desmin, dystrophin, vinculin and collagen types I and III was carried out. Specific connective tissue stains (trichrome Masson, van Gieson) and electron microscopical investigations were also performed. Results: Trichrome Masson and van Gieson staining demonstrated markedly increased dense connective tissue between the layers of the detrusor muscle in MMIHS compared to controls. Collagen type I immunoreactivity was markedly increased and SMA, desmin and dystrophin immunoreactivity was markedly reduced in the bladder muscle of MMIHS compared to controls. Electron microscopy revealed vacuolar degenerative changes in smooth muscle cells and an abundance of connective tissue between these cells. Conclusion: These data suggest that the detrusor muscle in MMIHS is strikingly abnormal and is the likely cause of voiding dysfunction. [Copyright &y& Elsevier]

Clean intermittent catheterization (CIC) is fundamental in managing children with impaired bladder function. However, it is not always feasible via the native urethra predominately males with intact sensation. In the current study, the experience regarding a modified vesico-cutaneous fistula (VCF) technique for CIC in children is reported. From January 2018 to January 2020, the modified VCF was performed to cases with impaired bladder function for CIC. Before surgery, patients were evaluated thoroughly. The anteroposterior diameter (APD) of the renal pelvis was considered as an indicator of the degree of affection of the upper urinary tract. Postoperative attacks of UTIs, leakage from the fistula, fistula stenosis, and the need for revision were recorded. The technique was accomplished relying on the principles of open Stamm gastrostomy (Fig. 1). Eight cases underwent a modified VCF due to different pathologies. Their ages, at time of the surgery, ranged from 1 month to 144/ 12 years. The median postoperative follow up period was 12 months. A leakage between catheterizations was experienced in 1 case. Stenosis of the fistula occurred in 1 case and it required surgical revision. No symptoms or signs of urinary tract infection were encountered. The mean APD of the renal pelvis was reduced from 29.8 mm (SD: ±14.6 mm), in the preoperative evaluation, to 17.6 mm (SD: ±10.85 mm) in the postoperative follow up. Vesico-cutaneous fistula is a new technique that provides easy and direct way for CIC. In the current study, a modified VCF technique was introduced to the enrolled patients as a simple maneuver, to make easy self-catheterization. Despite the limitations of this study due to the lack of a control group, the small number of cases, and the short follow up period, this modified technique was able to create a short and straightforward channel to access the bladder for intermittent catheterization. It could be done in redo cases. Results showed an acceptable degree of continence and low rate of complications. Moreover, it was socially accepted among our patients. Intermittent catheterization in children, with abnormal bladder function, can be done easily and efficiently via a modified VCF. [ABSTRACT FROM AUTHOR]

Several studies have revealed that electrical stimulation is an effective modality for treatment of lower urinary tract (LUT) dysfunction via raising the capacity or compliance of the bladder or maybe by pelvic floor relaxation and reducing detrusor pressure as well. This study aimed to assess the efficacy of transcutaneous interferential (IF) electrical stimulation on LUT symptoms as well as urine flow parameters in children with primary bladder neck dysfunction (PBND). We reviewed the charts of all children with persistent LUT symptoms who underwent IF electrical stimulation between 2010 and 2017. Twenty-three neurologically and anatomically normal children (mean age: 7.7 years, range: 5–13) with LUT symptoms were included in this study. Children had different LUT symptoms such as hesitancy, straining, urinary incontinence and constipation with no sufficient response to medical treatment (α –blocker) for at least 6 months. IF electrical stimulation was performed for 15 sessions, two times per week. All children were symptomatic and had abnormal urine flow pattern with an electromyographic (EMG) lag time of more than 6 s on uroflowmetry with EMG. A voiding chart, uroflowmetry with EMG and kidney and bladder ultrasounds were performed before and after the treatment for all children. Maximum and average urine flow rates, EMG lag time and postvoid residual volume were analyzed. In addition, alpha blocker therapy was continued during IF therapy. Mean maximum and average urine flow rates improved from 14.1 to 7.6–19.7 ml/s and 9.5 ml/s, respectively, while mean EMG lag time decreased from 11.7 to 5.2 s after the treatment (all P < 0.05). Also postvoid residual volume decreased significantly from 35.6 to 7.6 ml at the end of treatment courses. Pelvic floor EMG lag time, a documented parameter on uroflowmetry with EMG, defined as the time from the start of pelvic floor relaxation during a volitional voiding effort (the first stage of normal voiding) to the start of urine flow. Results showed that IF therapy decreased pelvic floor EMG lag time in children with PBND. In addition, increase in mean maximum and average urine flow rates in our patients indicated that pelvic IF therapy and behavioral modification improved voiding dysfunction in most of the patients and probably decreased bladder neck activity during voiding. Future studies with larger sample size are needed to support this finding. IF therapy appears safe, effective and reproducible in improvement of PBND in children. Image 1 [ABSTRACT FROM AUTHOR]

Summary Introduction Urethral meatotomy as treatment for meatal stenosis is a common pediatric urology procedure; however, little is known about the patient experience following this procedure. Objective We aim to evaluate clinical factors associated with patient-reported symptom improvement after urethral meatotomy. Study design The families of boys undergoing urethral meatotomy between 2/2013 and 8/2016 received a survey by mail 6 weeks after surgery. Families were queried on changes in symptoms using a Likert-type scale (5 = much improved, 4 = somewhat improved, 3 = no change, 2 = somewhat worse, and 1 = much worse). Patient and procedure characteristics of the respondents were obtained via chart review. These included surgical indication(s) (abnormal stream, dysuria, or storage symptoms), postoperative complications, reoperation, and unplanned postoperative communications. Patients who had procedures other than simple urethral meatotomy were excluded. Descriptive statistics were compiled, and generalized estimating equations used to determine the associations of patient and procedure characteristics with symptom improvement. Results We sent 629 surveys and received 194 responses (30.4%). Twelve respondents were excluded for complex procedures or miscoding. The majority of respondents were privately insured (74%) and were between 5 and 12 years old (45%) or 1 and 4 years old (42%). The most frequent surgical indication was abnormal stream (72%) followed by pain (21%) and storage symptoms (15.5%). Nine respondents had minor complications (4.9%). Four patients had restenosis requiring repeat urethral meatotomy. After surgery, a majority (79%) were “much improved,” 16% were “somewhat improved,” 3% had “no change,” and 1% were “somewhat worse.” No family reported “much worse.” Those patients who had “abnormal stream” as a surgical indication were significantly more likely to report “much improved” (OR 1.83, p = 0.014) than those without. Patient-reported improvement was not associated with suture use, patient age, insurance, surgeon, or location of the procedure (Table). Discussion Little has been written about patient-reported outcomes following urethral meatotomy. Our study affirms that the majority of boys improve following this procedure. However, improvement is significantly more likely if the child has a preoperative indication of an abnormal stream, such as deflection or spraying. Boys with symptoms of dysuria, frequency, or incontinence may be experiencing sequelae of meatal stenosis that simply take longer to improve. Alternatively, the meatal stenosis may be incidental to the primary symptoms. Conclusions A majority of families report substantial symptomatic improvement after urethral meatotomy. However, boys undergoing urethral meatotomy for reasons other than a urinary stream abnormality are less likely to experience improvement. Table Patient characteristics and key outcomes stratified by patient-reported improvement. Not “much improved” “Much improved” p -value ( n = 38) ( n = 144) Patient characteristics Indication ( n ; %) Stream abnormality 26/141 (18.4%) 115/141 (81.6%) 0.014 ∗ Pain 11/41 (26.8%) 30/41 (73.2%) 0.40 Storage symptoms 6/30 (20.0%) 24/30 (80.0%) 0.90 Suture used ( n ; %) 25/131 (19.1%) 106/131 (80.9%) 0.21 Outcomes Complications 5/9 (55.6%) 4/9 (44.4%) 0.02 ∗ Reoperation 3/4 (75.0%) 1/4 (25.0%) 0.02 ∗ ∗ A p -value <0.05 was considered significant. [ABSTRACT FROM AUTHOR]

Summary Introduction Lower urinary tract dysfunction (LUTD) involves faults in the filling and emptying phases of bladder function in toilet-trained children with no previous infection or any other obvious pathology. Lower urinary tract dysfunction is associated with conditions such as vesicoureteral reflux, recurrent urinary infection, behavioral alterations and decreased quality of life. The literature suggests an association between LUTD and obesity; however, the association between each individual symptom and obesity has yet to be evaluated. Objective To evaluate the association between excess weight and LUTD in children and adolescents in a community-based sample. Study design This cross-sectional study included 423 children and adolescents aged 5–17 years, and randomly selected in public places and schools between May and July 2015. The participants and their mothers completed the Dysfunctional Voiding Scoring System (DVSS) questionnaire, except for the questions on constipation and with the addition of a question on enuresis. They also completed the Rome III questionnaire, in which two positive responses defined the presence of constipation. Participants were classified as being of normal weight, overweight or obese, which was based on the BMI-for-age indicator. Results Mean age was 9.7 years (SD 2.9), with girls comprising 50.6% of the sample and adolescents 52.5%. The prevalence of LUTD was 7.1%, with 13.5% of participants being overweight and 12.1% obese (Figure). Constipation was present in 5.9% of participants and enuresis in 10.8%. In the multivariate analysis, three factors were independently and significantly associated with a positive DVSS: age <10 years (β = 0.76; 95% CI: 0.34–1.18), constipation (β = 1.79; 95% CI: 0.88–2.70) and obesity (β = 0.89; 95% CI: 0.25–1.52). Discussion Only bladder filling symptoms were associated with obesity. This may be explained by the fact that both obese individuals and those with emptying symptoms were shown to have activation alterations in the same brain regions. One limitation of this study was the use of questionnaires alone to diagnose LUTD and constipation. Conclusion Only the bladder-emptying symptoms of LUTD appear to be associated with obesity. This hypothesis may serve as a basis for future studies. Summary Fig Distribution of patients ( n = 423) according to weight, constipation and lower urinary tract dysfunction (LUTD). Summary Fig [ABSTRACT FROM AUTHOR]

Summary 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. Summary Fig Frequency of urinary symptoms in patients with Williams-Beuren syndrome. Summary Fig [ABSTRACT FROM AUTHOR]

Summary Background Feminizing genitoplasty techniques have diversified to encompass the spectrum of atypical genitalia in 46,XX congenital adrenal hyperplasia (CAH) patients. However, long-term outcomes evaluating postoperative continence following complex vaginoplasty remain scarce. Objective The aim was to review our surgical experience and assess postoperative urinary continence outcomes in CAH patients following complex, primary vaginoplasty. Study design We retrospectively reviewed CAH patients who underwent complex, primary vaginoplasty at a single center by a single surgeon from 1996 to 2013. We collected data on clinical characteristics, surgical techniques and postoperative urinary continence outcomes. In patients who had reached toilet-training age, continence outcomes were reviewed. Results Twenty-four patients met the inclusion criteria. The median age at the time of surgery was 8 months (interquartile range [IQR] 1 year–6 months). The median length of follow up was 6 years (IQR 10 years–3 years). These patients required complex reconstruction because of more extensive virilization: total urogenital mobilization (TUM, 10), pull-through vaginoplasty (9), partial urogenital mobilization (3), or pull-through vaginoplasty with partial urogenital mobilization (2). After surgery, 15 out of 24 (62.5%) reported age appropriate toilet-training without further complaints. Seven (29.2%) patients reported daytime incontinence or enuresis that resolved with conservative management. Two patients reported day and night incontinence requiring urethral bulking procedures following TUM. Discussion Twenty-two (91.7%) of our patients achieved continence without further surgical intervention. Of these patients, the TUM patients had serious incontinence issues. Our study represents a review of clinically significant cases from the female CAH population. The limitations of our study include a relatively small sample size and retrospective design. Conclusion Females with CAH and urogenital virilization most often achieved age appropriate toilet-training following vaginoplasty. Daytime incontinence and enuresis resolved without operative management in the vast majority of cases. TUM carries a risk of severe incontinence and may require further procedures to achieve urinary continence. Table Comparison of vaginoplasty techniques according to presurgical measurements of urogenital sinus and urethra, and postoperative urinary continence outcomes. Table Vaginoplasty Technique No. Urogenital Sinus Length (cm) Mean ± SD Urethral Length (cm) Mean ± SD Continence Enuresis Voiding Postponement Dysfunctional Voiding Continuous Incontinence Total Urogenital Mobilization 10 2.1 ± 0.6 1.7 ± 0.4 4 2 1 1 2 Pull-through 9 2.7 ± 1.0 1.4 ± 0.7 7 1 1 0 0 Partial Urogenital Mobilization 3 2 ± 0 2.1 ± 0.1 3 0 0 0 0 Pull-through with Partial Urogenital Mobilization 2 2.1 ± 1.0 1.6 ± 0 1 1 0 0 0 [ABSTRACT FROM AUTHOR]

Summary Introduction Uroflowmetry (UF) alone is often inadequate or unreliable to diagnose lower urinary tract dysfunction (LUTD). Therefore, other non-invasive tests, such as ultrasound (US), post-voiding residual volume (PVR) assessment and symptom scales, are used as well for objective definition of the problem. Objective The aim of this study was to investigate the possible predictive function of the non-invasive diagnostic tests for the response to treatment. Study design The prospective registry data of 240 patients with LUTD, from November 2006 to September 2013, were retrospectively analyzed. All patients were aged 5–14 years old. Patients with a previous diagnosis of vesicoureteral reflux (VUR), neurogenic bladder, monosymptomatic nocturnal enuresis (NE) were excluded from the study. Uroflowmetry, US, PVR and the Dysfunctional Voiding and Incontinence Symptom Scale (DVISS) were performed on every patient at their first visit and follow-ups. A DVISS <9 was considered as the DVISS response; parental opinion was based on International Continence Society criteria of clinical response. Time passed until clinical response was the last outcome parameter. Results Mean age was 8.2 years. Median follow-up was 60.5 months. A total of 62% of patients had complete response, 28.1% had partial response, and 9.7% had no response. Demographic variables were not associated with clinical outcome. Co-existing enuresis nocturna, multiple pharmacotherapy, and increased DVISS were associated with longer time until clinical response. Post-voiding residual volume assessment was the only test to have a prognostic value. Discussion Resolution rates of LUTD ranged from 40 to 90%. High resolution rate could be attributed to the long follow-up period, and the chance of spontaneous resolution. Treatment modalities and co-existing NE were associated with longer time until clinical response. Only PVR was associated with prognosis. This was the first study in literature to report such findings. It was seen that the normalization of pathologic patterns was a good sign for treatment success. The DVISS results showed significantly higher rates of incontinence compared to initial symptoms defined by the patients and/or their parents. This showed the importance of using scoring systems to better define the severity of symptoms. It was hard to establish a standardized cut-off value for bladder wall thickness on US. However, US was a good test for diagnosing additional pathologies. Conclusion Increased PVR was the single tool that was associated with prognosis and, therefore, should always be performed after UF. In addition, DVISS can help parents be counseled about their treatment expectations. [ABSTRACT FROM AUTHOR]