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PURPOSE: Given the increasing prevalence of chronic kidney disease in people with spina bifida, we sought to determine if this is associated with an increase in end stage kidney disease. We examined population-based data to measure the frequency of procedures to establish renal replacement therapy—a marker for end stage kidney disease- among patients with spina bifida. MATERIALS AND METHODS: We used the State Inpatient Database and State Ambulatory Surgery and Services Databases from Florida, Kentucky, Maryland, and New York (2000 to 2014), which include encounter-level data. With a diagnosis code-based algorithm we identified all procedural encounters made by patients with spina bifida. We determined the percentage of these encounters that were for facilitating renal replacement therapy (i.e., arteriovenous anastomosis, renal transplantation). We assessed for changes over time in this percentage with the Cochran-Armitage trend test. Bivariate analysis was performed using Chi square test. RESULTS: Of all procedures performed on patients with spina bifida over this time, the proportion of procedures performed to establish renal replacement therapy significantly decreased in both the inpatient and outpatient setting (p=0.042 and p

Background There is a high reported rate of sexual dysfunction among women with spina bifida, but little is known about the etiology of this or how sexual satisfaction could be improved. Aim To identify, through the words of women with spina bifida, perceived causes of diminished sexual satisfaction and recommendations to optimize partnered sexual encounters. Methods In this qualitative study, we conducted semi-structured individual interviews with 22 women with spina bifida (median age 26.5 years, range 16–52 years) who have had a romantic partner. Using Grounded Theory, interviews were independently coded by 3 reviewers. Disagreements were resolved by consensus. Outcomes We identified overlapping themes of issues women experienced during sexual intimacy and strategies they learned to improve sexual encounters. Results 7 salient themes emerged from the data: (i) fear of rejection with resulting difficulty setting boundaries and the risk of coercion; (ii) conflict between spontaneity and self-care in sexual encounters; (iii) worry about incontinence during sex; (iv) trial and error in learning optimal sexual positions; (v) decreased genital sensation; (vi) safety considerations; and (vii) sharing advice with other women with spina bifida. Clinical Implications As sexual satisfaction is influenced by physical features as well as psychological, interpersonal, and sociocultural factors, optimizing sexual satisfaction of women with spina bifida is best managed with a holistic approach utilizing a biopsychosocial model. Strengths & Limitations The sample included women with a diverse range of functional impairments. Women were forthright with their comments and thematic saturation was reached. Recruitment was primarily from a single Midwestern institution, which may have limited sampled perspectives. Conclusion While women with spina bifida encounter challenges during sexual encounters, strategies focused on improving communication with partners and addressing specific physical considerations can potentially enhance their sexual experiences.

Transitioning to adult health care and functioning is crucial for youth and young adults with special health care needs, such as those with Turner Syndrome (TS). The International Turner Syndrome Consensus Group developed clinical practice guidelines to improve this transition. This study sought to evaluate how effectively they have been implemented and whether they align with the priorities of women with TS and families of girls and women with TS. A web-based survey was offered to those affiliated with major TS support organizations, with 1,338 successfully responding [n = 543 women (≥18 years); n = 232 parents of a woman (≥18 years); and n = 563 parents of a girl with TS (

Introduction: Acoustic radiation force imaging (ARFI) is a recently developed form of ultrasound imaging that allows in-vivo measurement of tissue stiffness. This technology could be useful at predicting bladder compliance in children. We hypothesize that tissue stiffness, as measured by ARFI, correlates with abnormal bladder compliance and capacity in patients with bladder dysfunction. Methods: Patients who presented for cystometrography (CMG) underwent ARFI of the bladder wall. Nine bladder wall shear wave speed (SWS) measurements were acquired using point and 2D ultrasound shear wave elastography. The mean for each ARFI technique was correlated to bladder compliance, calculated using Wahl’s dimensionless number. ARFI parameters also were correlated with bladder capacity. Results: A total of 25 patients were enrolled. Mean age at time of enrollment was 4.23.9 years (range: 2 months to 15 years). There was no significant correlation between bladder compliance and point shear wave speed measurements (r=-0.22, p=0.31) or 2D shear wave speed measurements (r =-0.35, p=0.1). A total of 19 patients had bladder capacity below EBC. There was no significant correlation between bladder capacity and point shear wave speed measurements (r =-0.08, p=0.7) or 2D shear wave speed measurements (r=-0.36, p=0.09). Conclusions: Our results did not demonstrate a significant correlation between bladder wall ARFI shear wave measurements and bladder compliance or bladder capacity. Further studies are warranted to determine whether ARFI may be used to predict abnormal urodynamic parameters in children.

The Image Gently® campaign was launched by several radiological societies in 2007 to promote safe imaging in children. A goal of the campaign was to reduce ionizing radiation exposure in children. Given the recurrent nature of kidney stones, affected children are at risk for unnecessary ionizing radiation exposure from computerized tomography. We sought to determine whether the Image Gently campaign led to a decrease in the use of computerized tomography for evaluating children with nephrolithiasis. We hypothesized that the campaign was the primary cause of a reduction in the use of computerized tomography.We analyzed medical claims data from 2001 to 2015 identifying children with nephrolithiasis covered by the same commercial insurance provider. Using a difference in differences design, we estimated changes in computerized tomography use after the campaign started among patients less than 18 years old compared to a control group age 18 years or older with nephrolithiasis.We identified 12,734 children and 787,720 adults diagnosed with nephrolithiasis. Before 2007 quarterly rates of computerized tomography use during a stone episode (per 1,000 patients) were increasing at a parallel rate in children and adults (5.1 in children vs 7.2 in adults, p = 0.123). After the Image Gently campaign started the use of computerized tomography decreased in both groups but at a slightly higher rate in adults (difference in differences 2.96, 95% CI 0.00 to 5.91, p = 0.050).Although there has been a reduction in the use of computerized tomography among children with nephrolithiasis, given a similar trend seen in adults this change cannot be primarily attributed to the Image Gently campaign.

Introduction Women with spina bifida are sexually active, but most never discuss this topic with providers. Aim To determine what women with spina bifida understand about their sexual health, how they learned about it, what questions they have, and their experiences with their sexuality. Methods For this qualitative study, women with spina bifida ages 16 and older without marked developmental delay were individually interviewed. 25 women with spina bifida participated (mean age 27.1 years, range 16–52). Interviews were independently coded for themes by 3 reviewers, using Grounded Theory, with disagreements resolved by consensus. Main Outcome Measures We identified overlapping themes regarding the women’s perception and experience of their sexuality and sexual health education. Results 17 of the 25 (68%) participants had been or were currently sexually active. 5 themes emerged regarding their understanding of their sexuality and their sexual experiences: (i) being perceived as asexual, (ii) sources for sex education, (iii) need for spina bifida–specific sex education, (iv) impact of spina bifida–specific features on sexual encounters, and (v) perceived relationship between low sexual self-confidence and risk for sexual assault. Clinical Implications Women with spina bifida are sexual beings, but they are perceived as asexual by providers, which prevents them from getting adequate sexual health education and leaves them with misconceptions and unanswered questions, as well as vulnerable to sexual abuse. Strength & Limitations The strengths of this study include the diversity of women interviewed, including their age, severity of disability, and experiences with their sexuality, as well as the ability to reach thematic saturation. The limitation of this study is that most women received treatment at a single Midwestern tertiary referral center in the United States. Conclusion Including sexual health discussions in the usual care of women with spina bifida is critical to enhancing their sexual confidence and experience and preventing sexual abuse.

Acute pain after surgery is one of the most frequent indications for opioid prescribing in children. Opioids are often not stored or disposed of safely after their use, placing children and others in the home at risk for accidental ingestion or intentional misuse. We currently lack evidence-based guidelines for post-operative pain management after common ambulatory pediatric urologic procedures. Thus, each surgeon must decide if and how much opioid to prescribe based on his/her own assumptions of perceived post-operative pain.As part of an effort to establish opioid prescribing guidelines across two academic centers, the objectives of this study were to evaluate current variability in pediatric urologists' opioid prescribing factors and identify patients at greatest risk of being prescribed high doses of opioids after common ambulatory pediatric urologic procedures.We retrospectively evaluated post-operative opioid prescribing patterns after common ambulatory pediatric urology procedures (circumcision, orchiopexy, and hernia/hydrocele) at two major children's hospitals. Specifically, we evaluated if and how much opioid was prescribed for all children (18 years or younger) between 2016 and 2017. Bivariate analysis was performed using Kruskal-Wallis Test and Wilcoxon Rank Sum. Multivariable logistic regression was performed to determine patient, surgeon, and procedural factors that predicted the prescription of a high dose of opioids (greater than the median number of doses prescribed for that procedure).Over the two-year period, 811 circumcisions and 883 inguinal surgeries (inguinal orchiopexy and hernia/hydrocele) were performed. 94% of patients undergoing circumcision and 97% of those undergoing inguinal surgery were prescribed opioid analgesia. The median number of doses prescribed for circumcision was 20; for inguinal surgeries, 23.75% of patients received 15 opioid doses or more. Patients ages 0-2 years, who represented the largest age group (41% of all patients), received significantly more opioid doses than all other age groups, followed by those10 years (p 0.01). There was significant variation in opioid prescribing patterns by provider (p 0.01) (Figure 1) On multivariable logistic regression, younger age, pill form, and earlier year were all associated with a greater number of opioid doses prescribed for all surgeries.Across two institutions without a formal post-operative opioid prescribing policy for ambulatory pediatric urologic procedures, we observed considerable variability in provider prescribing patterns, with nearly all patients receiving an opioid, and those 0-2 years receiving the highest number of doses. This highlights the need for evidence-based guidelines for post-operative pain management after ambulatory pediatric urologic surgeries.

Objective To provide urologists with a practical guide for how to provide sexual health counseling to girls and women with spina bifida. Methods The recommendations and research of several sources were synthesized to create this guidance, including clinical guidance from the Spina Bifida Association and American College of Obstetricians, the current literature on the sexual health of girls and women with spina bifida, and the multidisciplinary experience of the authors. Results Sexual health education should be viewed by urologists as a continuous discussion, starting in early childhood and gradually building through adolescence. Developing a plan for when and how to bring it up, utilizing parents as educational partners, identifying who will provide the detailed one-on-one counseling if not the primary urologist, establishing a referral network for specialized care (eg, adolescent gynecologist, physical therapist, or sex therapist), becoming familiar with how spina bifida impacts sexual health, and being prepared for challenges are key to providing these girls and women with competent sexual health education. Urologists should also screen for abuse at each visit and be familiar with reporting and resources for when abuse is identified. Conclusion This guidance can serve to direct urologists in providing competent sexual health education to girls and women with spina bifida. This will ensure these girls and women receive the basic education they need, and that they can be referred to appropriate sexual health experts as indicated.

Introduction As most adults with spina bifida are either sexually active or interested in becoming sexually active, providers should understand how spina bifida impacts sexual function and options for treatment. Objectives The objective of this study is to summarize the current literature describing how features of spina bifida impact sexual function in men and women, effective available treatment options for sexual dysfunction, and to identify research gaps. Methods Searches were conducted in PubMed, CINAHL Complete, PsychInfo, Cochrane Central, Scopus, and Web of Science Core Collection databases using keywords related to spina bifida and sexual function. 34 primary research studies were included. Results Most men (56–96%) can achieve an erection, although it may be insufficient for penetration. Although 50–88% ejaculate, it is often dripping, retrograde, or insensate. Twenty percent to 67% achieve orgasm. Generally, men with lower lesions and intact sacral reflexes have better outcomes, although some men with all levels of lesion report good function. Sildenafil is efficacious at treating erectile dysfunction for most men. The “TO-MAXimize sensation, sexuality, and quality of life” procedure may improve sexual function in selected men with low-level lesions. Female sexual function and treatment is less well understood. Women may experience decreased arousal, difficulties with orgasm, and pain. No treatment has been studied in women. Bowel and bladder incontinence during intercourse appears to be bothersome to men and women. Although both men and women have diminished sexual satisfaction, their sexual desire appears to be least impacted. Present studies are limited by studies’ small, heterogeneous populations, the misuse of validated questionnaires in the sexually inactive population, and the lack of a validated questionnaire specific to people with spina bifida. Conclusions Spina bifida impacts the sexual function of both men and women. Future studies should seek a better understanding of female sexual function and treatment, use validated questionnaires appropriately, and ultimately create a validated sexual function questionnaire specific to this population. Streur CS, Corona L, Smith JE, et al. Sexual Function of Men and Women With Spina Bifida: A Scoping Literature Review. Sex Med Rev 2021;9:244–266.

Background As more women with spina bifida (SB) enter their reproductive years, the number having children is significantly increasing. However, little is known about their understanding of their ability to get pregnant or their experiences in considering, planning, or interacting with providers during a pregnancy. Objective We sought to determine what women have been told and understand about their reproductive health, their attitudes towards having children, and their experiences interacting with providers when seeking reproductive health care. Methods In this exploratory study employing qualitative research methods and following Grounded Theory, interviews with women with SB 16 years or older were transcribed verbatim and analyzed by three coders. Results Interviews of 25 women with SB ages 16–52 (median 26) revealed the following themes about their reproductive health perceptions and experiences: 1) poor understanding of reproductive health and potential, 2) interest in having a family, 3) facing provider's opposition to their reproductive goals, 4) going into pregnancy and delivery unprepared, 5) the importance of provider support for reproductive goals. Five women experienced an unintended pregnancy. Conclusions Although having children is important to most women with SB in this study, they report a poor understanding of their reproductive potential with several noting unintended pregnancies. They feel uninformed and unprepared during pregnancy and face discouragement from providers. Those experiencing supportive providers report a more positive experience. This demonstrates the urgent need to educate women with SB about their reproductive health and the providers who care for them how to support and counsel these women.

INTRODUCTION AND OBJECTIVES:Women with spina bifida (SB) are sexually active, but most never discuss this topic with providers. This leaves them vulnerable to potentially harmful misconceptions and...

Introduction Although pediatric urologists have taken responsibility for initiating discussions on sexual and reproductive health with spina bifida patients, research shows that very few girls and women with spina bifida have ever discussed this topic with any physician. Aim We sought to better understand pediatric urologists’ gaps in knowledge and training needs in the sexual and reproductive health education of women with spina bifida with the goal of creating a tool kit to equip providers to have these discussions. Methods In this qualitative study, pediatric urologists were interviewed separately about their current practices, perceived barriers, knowledge gaps, and recommendations for the tool kit until thematic saturation was reached. The interviews were recorded and transcribed verbatim, then analyzed using grounded theory by 3 independent reviewers. Main Outcome Measures To evaluate the perspectives and practices of pediatric urologists, we identified the overlapping themes of the interviews. Consensus on themes was reached. Results 10 Pediatric urologists participated in the study, including 5 men and 5 women, of whom 4 were fellows and 6 were attending physicians (mean years of practice 18, range 6–31 years). The mean number of patients followed up in the respective spina bifida clinics or by the provider was 434 (range 24–1,500). The following themes regarding pediatric urologists’ experience providing sexual and reproductive health education to women with spina bifida emerged. Pediatric urologists’: (i) lack of formal training; (ii) knowledge gaps such as spina bifida sexuality, fertility, and pregnancy experience; (iii) barriers to having sexual and reproductive health conversations such as lack of comfort and lack of time; (iv) facilitators of these conversations such as a long-term relationship with the patient and the patient’s own initiative; (v) desire to learn and provide competent care; and (vi) recommendations for a web-based tool kit that would include content to address the knowledge gaps and training about how to start sexual and reproductive health conversations. Clinical Implications These findings can provide the beginning concepts for the development of training on providing sexual and reproductive health education for pediatric urologists’ care for women with spina bifida. Strengths & Limitations This study gives the perspectives of 10 pediatric urologists with a diversity of backgrounds, but all of whom care for a large number of spina bifida patients. This does not give the perspectives of the spina bifida women themselves, which will be evaluated in the next phase of the study. Conclusion Pediatric urologists are not trained and do not feel prepared to provide sexual and reproductive health education for girls and women with spina bifida. However, they do see it as their scope of practice and wish to acquire competence in this area.

This study investigates patient-reported opioid consumption after pediatric operations.

Summary Introduction Urologic issues are persistent and important causes of morbidity and mortality in patients with myelomeningocele. Classically, patients with elevated bladder pressures despite adherence to clean intermittent catheterization (CIC) and pharmacotherapy undergo augmentation cystoplasty (AC). Currently, there is little understanding of which infants are more likely to require AC later. Objective In this context, the authors studied whether unfavorable urodynamic or imaging findings in patients with myelomeningocele during infancy could predict future AC. The authors hypothesized that infants born with elevated bladder pressures, vesicoureteral reflux (VUR), and/or hydronephrosis would be more likely to undergo AC. Study design The authors retrospectively identified patients with myelomeningocele at their institution who were followed-up since infancy ( Results A total of 97 patients met the inclusion criteria. The median follow-up time was 13.8 years. Augmentation cystoplasty was performed for 17 patients (17.5%) at a median age of 114 months (9.5 years). Detrusor leak point pressure/EFP was greater than 40 cm H2O in 34.0% (33/97) of infant cystometrogram studies, while 30.9% (30/97) had VUR on infant VCUG and 20.6% (20/97) had hydronephrosis on infant renal ultrasound. Patients with DLPP/EFP greater than 40 cm H2O or VUR during infancy were more likely to undergo AC (P = 0.02 and P = 0.03, respectively). Binomial logistic regression revealed that DLPP/EFP greater than 40 cm H2O (odds ratio [OR]: 4.28, 95% confidence interval [CI]: 1.34–13.62) and VUR (OR: 3.73, 95% CI: 1.18–11.77) were independent risk factors for future AC. Discussion Infants with myelomeningocele and elevated bladder pressures and VUR should be closely monitored by urodynamic testing and imaging studies. Parents can be counseled regarding the potentially higher risk for future AC in these patients. Nonetheless, the majority of high-risk infants will safely avoid AC with conservative management.