Urological outcomes in adult females born with anorectal malformation or Hirschsprung disease.

INTRODUCTION: Women born with anorectal malformation (ARM) or Hirschsprung disease (HD) may have impaired urologic function resulting in sequelae in adulthood. This study assessed and compared self-reported urinary outcomes in adult females born with ARM or HD to a reference population. METHODS: This was an IRB approved, cross-sectional study of female-born patients with ARM or HD, who completed surveys between November 2021 and August 2022. Female patients between the ages of 18 and 80 years were included. Lower Urinary Tract Symptom Questionnaires were administered through REDCap and the responses were compared to a reference population using Chi-squared or Fisher's exact tests. RESULTS: Sixty-six born female patients answered the questionnaires, two of them identified as non-binary. The response rate was 76%. Median age was 31.6 years. The majority were born with cloaca (56.3%), followed by other type of ARMs (28.1%), complex malformation (9.4%), and HD (6.3%). A history of bladder reconstruction was present for 26.6%. Catheterization through a channel or native urethra was present in 18.8%. Two had ureterostomies and were excluded from the analysis. Seven had chronic kidney disease or end-stage renal disease, three with a history of kidney transplantation. Patients with cloaca had significantly higher rates of urinary incontinence, urinary tract infection, and social problems due to impaired urological functioning, when compared to an age-matched reference population (Table 3). CONCLUSION: This study emphasizes the need for a multi-disciplinary team that includes urology and nephrology following patients with ARM long term, especially within the subgroup of cloaca. LEVEL OF EVIDENCE: III.

Sexual function and fertility of adult males with anorectal malformations or Hirschsprung disease.

PURPOSE: The long-term impact of anorectal malformations (ARM) and Hirschsprung disease (HD) on sexual function is well recognized but understudied. This study evaluated self-reported sexual and fertility outcomes in adult males with ARM and HD. METHODS: This was an IRB approved, prospective study of males in the Adult Colorectal Research Registry who completed surveys between October 2019 and March 2022. Electronic surveys were administered after consenting to being contacted for research. Patients completed the International Index of Erectile Function (IIEF) questionnaire and provided information on fertility outcomes. RESULTS: Sixty-five patients completed outcome questionnaires: 11 (16.9%) had HD and 54 (83.1%) had an ARM. Nineteen patients reported some degree of erectile dysfunction per IIEF criteria, a greater proportion of whom have ARM (p = 0.046). Twenty (30.7%) have reported having children; there were no differences in rates between HD and ARM patients. Most patients had not attempted to conceive, but eight patients, all of whom have ARM, have pursued fertility investigation or treatments. CONCLUSION: More patients with ARM reported some degree of erectile dysfunction compared to those with HD. Additionally, some have required fertility treatments. Further investigation is warranted to ensure true low rates of sexual and fertility dysfunction in patients with HD.

Patient-reported urinary outcomes in adult males with congenital colorectal conditions.

PURPOSE: Long-term urinary outcomes for patients born with Hirschsprung disease (HD) and anorectal malformations (ARM) may impact their health and wellbeing into adulthood. This study describes self-reported long-term urinary outcomes in males with HD and ARM. METHODS: This was a prospective study of male patients in the Adult Colorectal Research Registry who completed surveys on urinary function between October 2019 and March 2022. Self-reported health and functional outcomes were summarized, and differences based on type of condition were compared. RESULTS: Sixty-seven patients completed the questionnaire (response rate: 59.1%), of which 17.9% (12) had HD and 82.1% (55) had an ARM. Rates of urinary incontinence and stress urinary incontinence were 16.4% (11) and 4.5% (3), respectively. On sub-analysis of patients with ARM, patients with sacral ratio (SR) of 0.4-0.69 reported higher UTI rates compared to those with SR ≥ 0.7 (57.9 vs 25.8%, p = 0.023). Renal failure rates were highest among patients with recto-bladder neck fistulas (66.0%, p = 0.012). CONCLUSION: Patients with HD and ARM report a variety of urological sequelae in adulthood. Outcomes appear to be more common in patients with ARM and may be impacted by both anatomy and sacral ratios. Transitional care to monitor and manage renal and urological function is imperative.

Age-related urologic problems in the complex urologic patient.

PURPOSE: Improved medical care throughout childhood and adolescence has enabled patients with complex urological abnormalities to live longer into adulthood. These patients are now at risk of developing common, age-related, urological conditions. This review aims to review existing data and make recommendations in areas where expert opinion is currently lacking METHODS: This review represents the joint SIU-ICUD (Société Internationale d'Urologie-International Consultation on Urological Disease) consultation on congenital lifelong urology. The results of this analysis were first presented at a joint consultation of the ICUD and SIU at the 2018 SIU annual conference in Seoul, South Korea. RESULTS: BPH may present differently in patients with neurogenic bladder. Thorough assessment of neurological status, bladder and sphincter function is required before offering any bladder outlet surgery. Prostate specific antigen screening should be offered to men aged 50-69 with neurogenic bladders if they have good life expectancy. Multi-parametric MRI and transperineal biopsy would be the investigations of choice if feasible. Surgery for localized disease should only be done by surgeons with the relevant expertise. Bladder cancer in this patient group is more likely to present at a later stage and have a worse prognosis. Parenthood is achievable for most, but often requires assistance with conception. Pregnant women who have had previous urogenital reconstructive surgery should be managed in appropriate obstetric units with the involvement of a reconstructive urologist. CONCLUSIONS: Most evidence regarding complex urogenital abnormalities comes from the pediatric population. Evidence regarding common, age-related urological issues is generally from the 'normal' adult population. As patients with complex congenital urological conditions live longer, more data will become available to assess the long-term benefits of intervention.

The efficacy of onabotulinumtoxinA in patients with previous failed augmentation cystoplasty: Cohort series and literature review.

AIMS: To investigate the role of onabotulinumtoxinA (BTX-A) injections in patients with failed augmentation cystoplasty for neuropathic or idiopathic detrusor overactivity (NDO or IDO). METHODS: A retrospective review of all cystoplasty patients who underwent onabotulinumtoxinA injection at a tertiary center between 2008 and 2019 was performed. Details including indications and time from cystoplasty, video-urodynamic parameters, onabotulinumtoxinA dose, and clinical outcomes were analyzed. Telephone interview was performed for patients that requested repeat onabotulinumtoxinA injections. The interview included a modified PGIC7 and UDI6 questionnaires. A positive clinical response was considered improvement of overactive symptoms sufficient to merit repeat onabotulinumtoxinA injection and a modified PGIC7 of four or above. RESULTS: Thirty patients were identified (11 men and 19 women). The indications for augmentation were IDO (n = 18) or NDO (n = 12). Mean age at the time of cystoplasty was 42 years (range, 10-61). Interval between cystoplasty and initial onabotulinumtoxinA was 98 months (range, 3-271). Video-urodynamics before onabotulinumtoxinA revealed low compliance in 13 patients, DO in 22 patients, and combined low compliance/DO in 10. The median maximum cystometric bladder capacity was 338 mL (range, 77-570 mL). Thirteen patients responded to onabotulinumtoxinA injections. Higher peak DO pressure was associated with a significantly higher chance that the patient would experience benefit from the injections P = .026). The patients that responded to onabotulinumtoxin A underwent a total of 115 procedures (mean, 8.8 injections) over a mean 88 months (range, 20-157 months). CONCLUSIONS: Forty-three percent of patients responded well to intra-detrusor onabotulinumtoxinA injections. This avoided the need for more invasive surgery and had a positive impact on their quality of life.

Diagnosis and surgical outcomes of ectopic ureters in adults: A case series and literature review.

OBJECTIVES: To evaluate functional outcomes of surgery in patients with ectopic ureters diagnosed in adulthood; to review the world literature. PATIENTS AND METHODS: Patients were referred over 10 years. Data including surgical history, presenting symptoms, diagnostic modalities, interventions and functional outcomes were collected; and the world literature reviewed. RESULTS: 9 women and 1 man had a mean age of 37 (range 20-58). All women were referred with lifelong leakage. The man was referred with storage LUTS. Three women had heminephrectomy/nephrectomy with the distal ureter left in-situ prior to referral. MRI was the predominant diagnostic tool but often took senior review to confirm the diagnosis. Excision of the ectopic ureter was performed with bladder neck reconstruction in 6 women, with concomitant rectus fascial sling in one patient. 4 of 6 (67%) women were cured of their stress incontinence with the bladder neck reconstruction alone, whilst two required further intervention. Three women had nephroureterectomy alone as primary treatment of which two were cured. The single male had heminephrectomy and excision of seminal vesicle. CONCLUSIONS: Ectopic ureter is a rare diagnosis in adults but should be considered in patients who describe lifelong incontinence. Fine slice MRI is the imaging of choice. Excision of the ureter with bladder neck reconstruction alone was successful in two-thirds of patients, whilst further stress urinary incontinence surgery was required in one-third. Malignancy in ureteric remnants is described in 8% of the world literature. Patients should be counselled of this risk if the ureter is left in-situ.

The effects of recreational ketamine cystitis on urinary tract reconstruction - a surgical challenge.

OBJECTIVES: To identify the rate of postoperative complications in patients who require surgical reconstruction for ketamine-induced urinary tract dysfunction and to identify any predictors for poor postoperative outcome with subsequent management strategies. PATIENTS AND METHODS: A retrospective review of data collected between 2007 and 2017 of all patients with ketamine-induced urinary tract disease was performed. Evaluation included computed tomography urogram, cystoscopy, and biopsy. Indications and outcomes for surgical intervention were assessed. RESULTS: In all, 44 patients were identified of which 68% were male. The mean (range) age at presentation was 31 (23-55) years. All bladder biopsies confirmed an eosinophilic inflammatory infiltrate. A significant proportion of patients (81.8%) were found to have reduced cystoscopic bladder capacity of <300 mL (mean 196, range 25-550 mL). In all, 29 patients were treated conservatively with a view to symptom resolution. Two patients underwent dilatation for urethral strictures. Four patients underwent repeated intra-detrusor onabotulinum toxin injection with minimal subjective symptom relief. Two of these patients proceeded to have major reconstruction. Indications for urinary tract reconstruction included intractable symptoms, high-pressure compliance loss with renal compromise and ureteric obstruction. Patients were advised to abstain from ketamine use for a minimum of 6 months prior to consideration of surgical intervention. A total of 14 patients underwent major reconstruction. Surgical intervention included ileal conduit urinary diversion, augmentation cystoplasty with or without Mitrofanoff channels, ureteric re-implantation, and cystectomy with neobladders. Complications included anastomotic leaks, ureteric strictures, adhesional small bowel obstruction, renal failure, and sepsis. Overall, complications occurred in 10/14 patients. CONCLUSION: In a tertiary high-volume reconstructive unit, ketamine patients were at high risk of significant perioperative complications. There did not appear to be any other common factor apart from their use of ketamine, and the significant inflammatory change associated with this. We recommend meticulous preoperative evaluation and multidisciplinary consultation for all patients to determine optimal treatment strategies.

Leading contributors to success of transitional urology units.

PURPOSE OF REVIEW: There is increasing recognition of the need for life-long care in patients who have congenital urological anomalies. This study looks at the factors surrounding transition and suggests those, which may improve the success. RECENT FINDINGS: One of the most challenging phases in this care is adolescence. There are the physical and psychological changes that all adolescents go through. Patients need medical surveillance through this time in order to ensure good care and patient safety. For affected patients there are other major challenges - first the relationship of their underlying condition, its treatment and the effect that either of these will have on their relationship with normality. The second big challenge is that in the midst of these changes our patients have to move from a paediatric to an adult centred environment.Transition is the term used to define the gradual shift of healthcare responsibility from the parents to the patient and all the factors above will affect this important time in life and care. SUMMARY: Transition can be a challenge to both patients and the healthcare team. The key to success is a strong multidisciplinary team to try and maintain patient engagement.

The transition of young adults with lifelong urological needs from pediatric to adult services: An international children's continence society position statement.

INTRODUCTION: Children with urinary tract disorders managed by teams, or individual pediatricians, urologists, nephrologists, gastroenterologists, neurologists, psychologists, and nurses at some point move from child-centered to adult-centered health systems. The actual physical change is referred to as the transfer whilst the process preceding this move constitutes transition of care. Our aims are twofold: to identify management and health-service problems related to children with congenital or acquired urological conditions who advance into adulthood and the clinical implications this has for long-term health and specialist care; and, to understand the issues facing both pediatric and adult-care clinicians and to develop a systems-approach model that meets the needs of young adults, their families and the clinicians working within adult services. METHODS: Information was gleaned from presentations at an International Children's Continence Society meeting with collaboration from the International Continence Society, that discussed problems of transfer and transitioning such children. Several specialists attending this conference finalized this document identifying issues and highlighting ways to ease this transition and transfer of care for both patients and practitioners. RESULTS: The consensus was, urological patients with congenital or other lifelong care needs, are now entering adulthood in larger numbers than previously, necessitating new planning processes for tailored transfer of management. Adult teams must become familiar with new clinical problems in multiple organ systems and anticipate issues provoked by adolescence and physical growth. During this period of transitional care the clinician or team assists young patients to build attitudes, skills and understanding of processes needed to maximize function of their urinary tract-thus taking responsibility for their own healthcare needs. Preparation must also address, negotiating adult health care systems, psychosocial, educational or vocational issues, and mental wellbeing. CONCLUSIONS: Transitioning and transfer of children with major congenital anomalies to clinicians potentially unfamiliar with their conditions requires improved education both for receiving doctors and children's families. Early initiation of the transition process should allow the transference to take place at appropriate times based on the child's development, and environmental and financial factors. Neurourol. Urodynam. 36:811-819, 2017. © 2016 Wiley Periodicals, Inc.

Adolescent and adult outcomes in women following childhood vaginal reconstruction for cloacal anomaly.

PURPOSE: We examined outcomes in female adolescents and women who underwent vaginoplasty in childhood during genitourinary reconstruction for cloacal anomalies. MATERIALS AND METHODS: We retrospectively reviewed the medical notes on girls and women attending an adult specialist center for genitourinary anomalies. Data were collected on vaginal reconstruction, menstruation, sexual and reproductive function, and urological and gastroenterological outcomes. RESULTS: We identified 19 patients with a mean age of 22 years (range 13 to 35), of whom 16 (84%) underwent vaginoplasty in the first year of life. Nine of these 16 patients (56%) had required 1 (7) or 2 (2) further vaginal reconstructions to facilitate menstruation or sexual activity. The remaining 7 patients (44%) required no further vaginal reconstruction. Nine of the 19 patients (47%) had associated müllerian anomalies, obstructed menstruation developed in 5 (26%) and 1 required hemihysterectomy. Eight patients were sexually active, of whom 1 experienced difficult penetration. Three patients attempted to conceive, including 1 with a complex preterm delivery and 2 undergoing fertility treatment. Of the patients 74% underwent further reconstruction of the renal tract and 36% had an enteric stoma. CONCLUSIONS: This study confirms the complexity of vaginal reconstruction in this group with a notable vaginoplasty revision rate. Müllerian anomalies were identified in almost half of the patients, a higher incidence than previously reported, and in a quarter obstructed menstruation developed in puberty. A specialist team with gynecologic input should treat patients with cloacal anomalies. Outcome data are sparse. There remains a need for well planned, prospective cohort studies that include assessments of psychological, sexual and reproductive outcomes.

Sexual function and health-related quality of life in women with classic bladder exstrophy.

OBJECTIVE: To investigate sexual function and quality of life in adolescent and adult women with classic bladder exstrophy (BE). MATERIALS AND METHODS: A two-part observational cross-sectional study with a questionnaire arm and a retrospective case review arm was performed. The study was undertaken in a centre providing a tertiary referral gynaecology and urology service. Outcomes were sexual function and quality-of-life scores. RESULTS: A total of 44 patients with BE were identified from departmental databases and included in the study, of whom 28 (64%) completed postal questionnaires. Sexual function scores and quality-of-life visual analogue scales were significantly poorer compared with normative data. CONCLUSIONS: Bladder exstrophy has a detrimental psychological impact on women. In future, methodical multidisciplinary paediatric follow-up research will help to identify predictors of better and worse adolescent and adult outcomes. Development and evaluation of cost-effective psychological interventions to target specific problems is also warranted.

The long-term management and outcomes of cloacal anomalies.

Cloacal anomalies occur when failure of the urogenital septum to separate the cloacal membrane results in the urethra, vagina, rectum and anus opening into a single common channel. The reported incidence is 1:50,000 live births. Short-term paediatric outcomes of surgery are well reported and survival into adulthood is now usual, but long-term outcome data are less comprehensive. Chronic renal failure is reported to occur in 50 % of patients with cloacal anomalies, and 26-72 % (dependant on the length of the common channel) of patients experience urinary incontinence in adult life. Defaecation is normal in 53 % of patients, with some managed by methods other than surgery, including medication, washouts, stoma and antegrade continent enema. Gynaecological anomalies are common and can necessitate reconstructive surgery at adolescence for menstrual obstruction. No data are currently available on sexual function and little on the quality of life. Pregnancy is extremely rare and highly risky. Patient care should be provided by a multidisciplinary team with experience in managing these and other related complex congenital malformations. However, there is an urgent need for a well-planned, collaborative multicentre prospective study on the urological, gastrointestinal and gynaecological aspects of this rare group of complex conditions.

Functional properties and connective tissue content of pediatric human detrusor muscle.

The functional properties of human pediatric detrusor smooth muscle are poorly described, in contrast to those of adult tissue. Characterization is necessary for more informed management options of bladder dysfunction in children. We therefore compared the histological, contractile, intracellular Ca2+ concentration responses and biomechanical properties of detrusor biopsy samples from pediatric (3-48 mo) and adults (40-60 yr) patients who had functionally normal bladders and were undergoing open surgery. The smooth muscle fraction of biopsies was isolated to measure proportions of smooth muscle and connective tissue (van Gieson stain); in muscle strips, isometric tension to contractile agonists or electrical field stimulation and their passive biomechanical properties; in isolated myocytes, intracellular Ca2+ concentration responses to agonists. Pediatric detrusor tissue compared with adult tissue showed several differences: a smaller smooth muscle-to-connective tissue ratio, similar contractures to carbachol or α,ß-methylene ATP when corrected for smooth muscle content, and similar intracellular Ca2+ transients to carbachol, α,ß-methylene ATP, raised K+ concentration or caffeine, but smaller nerve-mediated contractions and greater passive stiffness with slower stress relaxation. In particular, there were significant atropine-resistant nerve-mediated contractions in pediatric samples. Detrusor smooth muscle from functionally normal pediatric human bladders is less contractile than that from adult bladders and exhibits greater passive stiffness. Reduced bladder contractile function is not due to reduced smooth muscle contractility but to greater connective tissue deposition and to functional denervation. Significant atropine resistance in pediatric detrusor, unlike in adult tissue, demonstrates a different profile of functional neurotransmitter activation. These data have implications for the management of pediatric bladder function by therapeutic approaches.

Examining long-term outcomes of bladder exstrophy: a 20-year follow-up.

OBJECTIVE: To examine long-term quality-of-life, urinary continence and sexual function outcomes in patients diagnosed with bladder exstrophy (BE). PATIENTS AND METHODS: A total of 65 patients with BE and follow-up of at least 20 years were identified. After informed consent for inclusion in the study, the patients were asked to complete three validated questionnaires, the Short-Form 36 quality-of-life questionnaire (SF-36), the International Consultation on Incontinence Questionnaire (ICIQ) and the International Index of Erectile Function (IIEF), to assess quality of life, perceived urinary continence and sexual function. RESULTS: In all, 21 patients responded to the questionnaires, yielding a 32% response rate. High scores in each of the eight dimensions of the SF-36 reflected a positive perception of quality of life by respondents; calculated scores were compared with those of a normal control group. The only significant difference found between the groups was that the study population perceived their general health to be poorer than those in the control group. High scores on the ICIQ indicate high levels of subjective incontinence in patients, with scores ranging from 0 to 21. Reporting scores of 0 (continent), 12/21 patients perceived their continence to be normal, 9/21 patients had scores >2 (mild), with one patient scoring a 16, and 11 (severe incontinence), patients reported no identifiable leakage during normal activities. There was a 29% response rate for the IIEF (15 patients completed this). IIEF scores were broken down into five dimensions and mean scores were calculated. The mean scores showed mild to moderate dysfunction in each category, including overall satisfaction with sexual experience. CONCLUSIONS: The patient-reported quality of life in patients with BE was normal in all dimensions, with the exception of perception of general health. Half of the patients reported normal continence and had no complaints of urinary leakage. Sexual function in males was significantly affected across all dimensions, with mild to moderate dysfunction.

Barriers to Transitional Care in Spina Bifida.

The health care needs children with spina bifida evolve over their lifetime; continued, regular contact with appropraitely trained, multidisciplinary providers is crucial to a patient's health and quality of life. Substantial research has been conducted to improve the transition process starting at an early age; however, there continue to be strong barriers to successful transition. This article reviews key aspects of the care of patients with spina bifida, the impact of inadequate transition to adult care, barriers to transition, and offers a potential vision for the future.

Long term outcomes in classic bladder exstrophy - The adult picture.

With continued improvements in medical care and surgical reconstruction, more patients with classic bladder exstrophy (CBE) are living into adulthood, than ever before. With improved survival, a greater emphasis on adult issues and improving quality of life (QOL) for these individuals is of increasing importance. This review aims to summarize data on long-term considerations for the adult with exstrophy and to highlight areas of future research and collaboration. Key conclusions are that continence or dryness are achievable alongside the ability to enjoy sexual relationships and a good quality of life.

An Exploratory Analysis of the Association between Healthcare Associated Infections & Hospital Financial Performance.

Purpose: Healthcare-associated infections (HAIs) place a significant financial burden on United States hospitals. HAI treatments extend hospital lengths of stay and increase hospital operational costs while significantly reducing hospital profit margins. Given these challenges, the research aim of this study was to explore the association between HAIs and hospital financial performance. A better understanding of this relationship can assist hospital leaders in optimizing the use of scarce financial resources to reduce HAI prevalence. Methods: Data for calendar year 2022 for active short-term acute care hospitals (n = 1454) in the US were analyzed using multiple linear regression analysis. We explored two derived dependent variables, operating expense per staffed bed and operating expense per discharge. The independent variables included four healthcare-associated infection rates: methicillin-resistant Staphylococcus aureus (MRSA) infection rate, Clostridium difficile (C. diff) infection rate, Catheter-Associated Urinary Tract Infection (CAUTI) rate, and Central Line Associated Blood Stream Infections (CLABSI). Appropriate organizational and market-level variables that may independently influence hospital financial performance were included as control variables. Results: The results revealed that C. diff (ß: 0.037, p < 0.05) and CAUTI (ß: 0.031, p < 0.05) rates were positively associated with an increase in operating expense per staffed bed, while increases in MRSA (ß: 0.042, p < 0.001), C. diff (ß: 0.062, p < 0.001), and CAUTI rates (ß: 0.039, p < 0.001) were correlated with increased operating expenses per discharge. Conclusions: This study demonstrates that specific HAIs may be associated with increased hospital expenses. Proactively targeting these infections through tailored interventions may lead to reduced hospital costs, improved financial performance, and economic stability.

Considerations and Outcomes for Adolescents and Young Adults With Cloacal Anomalies: A Scoping Review of Urologic, Colorectal, Gynecologic and Psychosocial Concerns.

The objective of this scoping review is to provide a summary of the current literature regarding adolescents and young adults with histories of cloacal anomalies. Preferred Reporting Items for Systematic Reviews and Meta-analysis Extension for Scoping Reviews were used. Data were categorized into four domains-urologic, colorectal, gynecologic/obstetric, and sexual/psychosocial. The current literature has poor study quality and mostly consists of retrospective studies of small cohorts with varying definitions of outcomes. Women with cloacal anomalies are at high risk for urologic dysfunction but can maintain kidney health and achieve social continence with medical and surgical management. Sexual function and adult healthcare transition are areas ripe for improved future research.