Long-term urology outcomes of anorectal malformation.

INTRODUCTION: Urological problems are a recognised feature of anorectal malformation (ARM). Previous assumptions of favourable long-term urinary outcomes are being challenged. OBJECTIVE: We hypothesised that urinary tract problems are common in ARM and frequently persist into adulthood. We retrospectively reviewed long-term renal and bladder outcomes in ARM patients. STUDY DESIGN: Patients with ARM born between 1984-2005 were identified from electronic hospital databases. Their case notes were reviewed. Renal outcomes included serum creatinine and the need for renal replacement therapy. Bladder outcomes included symptom review, bladder medication, need for intermittent catheterisation, videourodynamics and whether the patient had undergone augmentation cystoplasty. RESULT (TABLE 1): The case notes of 50 patients were reviewed. The median age at last follow up was 18 years (range 12-34 years). The level of fistula was noted to be high in 17 patients, intermediate in eight, and low in 10. Four had cloaca. Congenital urological abnormalities were present in 25 (50%). An abnormal spinal cord was present in 22 (44%) patients. VACTERL association occurred in 27 (54%). Chronic kidney disease stage II or above was found in 14 (28%) patients, of whom four required a renal transplant. Abnormal bladder outcomes were found in 39 (78%) patients. Augmentation cystoplasty with Mitrofanoff had been performed in 12. Of those who had not undergone cystoplasty, 17 had urinary symptoms, including urinary incontinence in 12. Of the 39 patients with abnormal bladder outcome, 19 (49%) did not have a spinal cord abnormality. There was no significant statistical association between level of ARM and abnormal renal outcome or presence of bladder abnormality. DISCUSSION: Adverse renal and bladder outcomes are common in our cohort of young people with ARM with a significantly higher incidence compared with current literature. We did not demonstrate an association between level of ARM or presence of spinal cord anomaly with persistent bladder problems. Congenital urological anomalies are more common in those who later have an abnormal renal outcome. Although this difference is statistically significant, one fifth of patients born with anatomically normal upper tracts develop reduced renal function, implying an important acquired component. CONCLUSION: Bladder problems and reduced renal function affect a significant proportion of young adults with ARM. Neither adverse outcome is reliably predicted from ARM level, congenital urological anomaly or spinal cord anomaly. We advise continued long-term bladder and kidney follow-up for all patients with ARM.

A Smartphone App for Supporting the Self-management of Daytime Urinary Incontinence in Adolescents: Development and Formative Evaluation Study of URApp.

BACKGROUND: Daytime urinary incontinence (UI) is common in childhood and often persists into adolescence. UI in adolescence is associated with a range of adverse outcomes, including depressive symptoms, peer victimization, poor self-image, and problems with peer relationships. The first-line conservative treatment for UI is bladder training (standard urotherapy) that aims to establish a regular fluid intake and a timed schedule for toilet visits. The success of bladder training is strongly dependent on good concordance, which can be challenging for young people. OBJECTIVE: This paper aims to describe the development of a smartphone app (URApp) that aims to improve concordance with bladder training in young people aged 11 to 19 years. METHODS: URApp was designed by using participatory co-design methods and was guided by the person-based approach to intervention design. The core app functions were based on clinical guidance and included setting a daily drinking goal that records fluid intake and toilet visits, setting reminders to drink fluids and go to the toilet, and recording progress toward drinking goals. The development of URApp comprised the following four stages: a review of current smartphone apps for UI, participatory co-design workshops with young people with UI for gathering user requirements and developing wireframes, the development of a URApp prototype, and the user testing of the prototype through qualitative interviews with 23 young people with UI or urgency aged 10 to 19 years and 8 clinicians. The app functions and additional functionalities for supporting concordance and behavior change were iteratively optimized throughout the app development process. RESULTS: Young people who tested URApp judged it to be a helpful way of supporting their concordance with a timed schedule for toilet visits and drinking. They reported high levels of acceptability and engagement. Preliminary findings indicated that some young people experienced improvements in their bladder symptoms, including a reduction in UI. Clinicians reported that URApp was clinically appropriate and aligned with the best practice guidelines for bladder training. URApp was deemed age appropriate, with all clinicians reporting that they would use it within their own clinics. Clinicians felt URApp would be of particular benefit to patients whose symptoms were not improving or those who were not engaging with their treatment plans. CONCLUSIONS: The next stage is to evaluate URApp in a range of settings, including pediatric continence clinics, primary care, and schools. This research is needed to test whether URApp is an effective (and cost-effective) solution for improving concordance with bladder training, reducing bladder symptoms, and improving the quality of life.

Urodynamic characterization of giggle incontinence in children.

AIMS: Giggle incontinence is a rare condition resulting in excessive urinary incontinence with laughter, where bladder function is otherwise "normal." Urodynamic descriptions of the condition to date are limited. We believe that giggle incontinence has characteristic urodynamic findings. We tested this hypothesis. METHODS: We retrospectively reviewed the urodynamic investigations of patients with giggle incontinence managed in a tertiary regional bladder unit between February 2014 and November 2019. RESULTS: We identified the studies of seven patients, median age 13.5 years (10.4-15.7) of whom 6 were female. All had videourodynamics. Two went on to have further invasive investigation; one had urethral pressure profile and one had ambulatory urodynamics. Detrusor overactivity (DO) was observed in six. DO was asensate in all. In five DO was triggered by laughter and was associated with laughter induced incontinence in four. Six had DO that was not provoked by laugher. In one amplitude of DO was proportional to vigour of laughter. In three patients there was identification of sudden pelvic floor relaxation during laughter resulting in incontinence. Stress urinary incontinence was not observed in any. CONCLUSIONS: Giggle incontinence is a complex phenomenon. Urodynamic diagnosis is challenging and is dependent on eliciting laughter. We present the first urodynamic demonstration that giggle incontinence is associated with laughter-induced, asensate DO and concurrent, momentary pelvic floor relaxation. We hope this will provide a more consistent basis for defining this condition in the future.

Dysfunctional voiding: the importance of non-invasive urodynamics in diagnosis and treatment.

In Dysfunctional voiding, failure of the external sphincter-pelvic floor complex to relax during micturition results in bladder outflow obstruction with a spectrum of presentation from more benign lower urinary tract dysfunction including recurrent urinary tract infections, to significant upper tract pathology and end-stage renal failure. There is no underlying neurological or anatomical cause and the condition is postulated to be a largely learnt behavior. Diagnosis relies on non-invasive urodynamics and in particular uroflowmetry, plus or minus EMG, which is also used in biofeedback, the mainstay of treatment. The etiology, presentation, diagnosis, and treatment with particular emphasis on non-invasive urodynamics are covered.

Electroneurostimulation for the management of bladder bowel dysfunction in childhood.

Both non-invasive and invasive electroneurostimulation (ENS) modalities for bladder bowel dysfunction have been studied and reported in children. A summary of the proposed mechanism of actions and the more commonly used and recently reported techniques and outcomes are described. This includes transcutaneous electrical nerve stimulation, functional electrical nerve stimulation, intravesical electrical nerve stimulation, percutaneous tibial nerve stimulation and sacral neuromodulation in conditions including overactive bladder, enuresis, dysfunctional voiding, constipation, combined bladder bowel dysfunction and neuropathic bladder and bowel dysfunction. There is a need for further high quality randomised trials as well as long-term outcomes to establish whether ENS is able to alter the long-term trajectory for an individual child with bladder bowel dysfunction.