Experiences and preferences of women with CAH and parents about disclosure of childhood surgery.

INTRODUCTION: Complete disclosure of childhood genital surgery to patients with congenital adrenal hyperplasia (CAH) is a critical part of CAH care. There are no guidelines or uniform recommendations on the timing and content of surgical disclosure discussions. OBJECTIVE: Our objective was to describe the experiences and preferences of females with CAH and parents of females with CAH who underwent childhood genital surgery regarding surgical disclosure. METHODS: We conducted an anonymous cross-sectional online survey of females with CAH (46XX, ≥16 years [y] old) and parents of females with CAH who underwent genital surgery before age 4y in North America. Participants reported experiences, preferences, and advice about initial ("first time you were told") and complete disclosure ("told all details"). Non-parametric statistics and qualitative analysis were used. RESULTS: Participants included 59 females with CAH (median age: 37y, 92% White, 93% non-Hispanic) and 41 parents (median: 36y, 85% White, 93% non-Hispanic, daughter median: 26y). The 76% of females who received complete disclosure were younger (median age: 33y) and underwent surgery more recently (median decade: 1980s) than the 14% who received only initial disclosure (median: 47y, 1970s) and the 10% who did not receive any disclosure (median: 60y, 1960s, p = 0.0003, Summary Figure). Females reported median ages of initial and complete disclosure as 7-10y and 11-13y, respectively. Disclosure was preferred by 98% of females with initial disclosure by age 14y and complete disclosure by 18y. Parents reported similar findings. Most disclosures were by mothers (initial: 82%, complete: 64%). Doctors were more involved in complete vs. initial disclosures (complete: 47%, initial: 13%, p < 0.001). Qualitative analysis of advice about surgical disclosure revealed 8 themes. CONCLUSIONS: Disclosure of childhood genital surgery to women with CAH has increased over time. Although timing of disclosure varied, women preferred disclosure, and that it be initiated before age 14y and completed by age 18y.

Gender-affirming Robotic Tubularized Peritoneal Vaginoplasty: Feasibility and Outcomes.

OBJECTIVE: To evaluate the outcomes of robot-assisted tubularized peritoneal vaginoplasty (RATPV) for trans-feminine patients desiring genital gender-affirming surgery (gGAS). METHODS: We retrospectively reviewed all patients (adults ≥18 years old) who underwent RATPV for gGAS at our institution (July 2020-July 2022). Initial technique involved anastomosing the peritoneal flap to the introitus. The updated technique anastomosed the flap intraperitoneally to inverted penile shaft skin. Patient demographics, intraoperative details, and postoperative outcomes are described. RESULTS: In 33 patients, the median age was 26 years old (IQR:24-37) and BMI 26 (IQR:23-32). Median operative time was 406 minutes (IQR: 370-434) and robotic console time 177 minutes (IQR:154-220). Intraoperatively, the median vaginal depth was 17 cm and diameter 3 +cm. Median length of stay was 7 days. One-third (n = 11) of patients had a complication within 30 days postoperatively (40% Clavien Dindo I and 60% Clavien II). No patient had a Clavien III+ complication. At a median follow-up of 499 days (range: 137-835), some degree of vaginal stenosis occurred in 48% (11/23) of patients with the original approach versus 10% (1/10) of those with the newer approach (P = .05). Median vaginal depth was 11.3 cm. Three patients (9%) required revision vaginoplasty, all of whom had the original approach and had difficulty dilating postoperatively. All had clitoral sensation. CONCLUSION: RATPV is a safe and effective option for individuals seeking gender-affirming vaginoplasty that obviates the need for laser hair removal prior to surgery. More research is needed into long-term outcomes of this technique.

Parental decision regret after pediatric urologic surgeries compared to decisions of everyday life.

INTRODUCTION: Parents are at risk of decision regret (DR) for decisions affecting their children. The Decision Regret Scale (DRS) measures medical DR but lacks context outside of healthcare. OBJECTIVE: To compare parental DR 1) between common pediatric urologic surgeries and everyday decisions and 2) with preference to make a different choice. METHODS: We conducted a cross-sectional online survey of randomly selected parents >1year (y) after their children underwent: orchiopexy (males ≤10y), open ureteral reimplant (OUR, females 2-6y), open pyeloplasty (OP, ≤2y), or robotic pyeloplasty (RP, 5-17y) (2017-2021). Higher DRS scores indicate increased DR (none: 0, mild: 1-25, moderate: 30-50, strong: 55-75, very strong: 80-100). Parents completed DRS on four decisions: their child's surgery, most recent/current romantic relationship, most recent leased/purchased car, and most recent purchased meal. Parents reported if they would make the same choice (yes/no). Nonparametric statistics were used. RESULTS: We surveyed 191 parents (orchiopexy n = 52, OUR n = 50, OP n = 51, RP n = 38). The median parent age was 36y (mothers: 86%). Some DR was reported for all decisions, but with significant differences in DR severity. The lowest median DRS score was seen with surgery (orchiopexy 0 [IQR 0-10], OUR 0 [IQR 0-5], OP 0 [IQR 0-0], RP 0 [IQR 0-0]), with no difference between surgery groups (p = 0.78). This was followed by relationship (0, IQR 0-20), car (15, IQR 0-25), and meal (20, IQR 0-30, p < 0.001). Most parents did not report any DR regarding surgery (orchiopexy 69%, OUR 74%, OP 76%, RP 76%, with no difference between surgery groups p = 0.85, Summary Figure). Comparatively, 59% of parents did not have any regret about their relationship, 37% their car, and 28% their meal (p < 0.001). All surgical DR was mild or moderate. No parent (0%) would have chosen differently for their child's surgery versus 4-12% for non-surgical decisions (p < 0.001). Overall, increasing DR corresponded to increasing desire to have made a different choice (DRS≤10: 0%, DRS 45-50: 32%, DRS 55-60: 66%, DRS≥75: 100%, p < 0.001). CONCLUSION: Parental DR varied between urological surgical and non-surgical decisions. It was lowest after surgery. Some regret was reported after every decision, but the subset of parents with regret was smallest after surgical decisions. Positive DRS scores do not necessarily correspond to parents wishing they made a different choice.

Prevalence of sexual abuse and intimate partner violence in adults with spina bifida.

BACKGROUND: People with disabilities and chronic medical conditions are known to be at higher risk of sexual abuse (SA) and intimate partner violence (IPV). People with spina bifida (SB) are vulnerable, but little is known about the prevalence of abuse in this population. OBJECTIVE: To evaluate the prevalence and risk factors of SA and IPV in adults with SB. METHODS: An anonymous international cross-sectional online survey of adults with SB asked about history of SA ("sexual contact that you did not want") and IPV ("hit, slapped, kicked, punched or hurt physically by a partner"). RESULTS: Median age of the 405 participants (61% female) was 35 years. Most self-identified as heterosexual (85%) and were in a romantic relationship (66%). A total of 19% reported a history of SA (78% no SA, 3% preferred not to answer). SA was more frequently reported by women compared to men (27% vs. 5%, p < 0.001) and non-heterosexual adults compared to heterosexuals (41% vs. 15%, p < 0.001). Twelve percent reported a history of IPV (86% no IPV, 2% preferred not to answer). IPV was more frequently reported by women compared to men (14% vs. 9%, p = 0.02), non-heterosexuals compared to heterosexuals (26% vs. 10%, p = 0.002), and adults with a history of sexual activity versus those without (14% vs. 2%, p = 0.01). CONCLUSION: People with SB are subjected to SA and IPV. Women and non-heterosexuals are at higher risk of both.

Single-port hidden incision endoscopic (HIdES) pediatric nephrectomy via pfannenstiel incision.

Laparoendoscopic single-site surgery (LESS) and hidden incision endoscopic surgery techniques are increasingly used in pediatric urology. For pediatric nephrectomy, access through a single Pfannenstiel incision is novel and may offer cosmetic benefit. In this retrospective study, we describe this approach and assess operative outcomes associated with this technique. Patients who underwent LESS nephrectomy through a single Pfannenstiel incision had minimal blood loss, short length of stay, low risk of surgical complications, and satisfactory wound healing. The Pfannenstiel approach to LESS nephrectomy is feasible, versatile, and achieves excellent operative and cosmetic outcomes, although direct comparison to other approaches is warranted.

Immune Expression in Children With Vesicoureteral Reflux: A Pilot Study.

OBJECTIVE: To perform an exploratory, descriptive pilot study of the systemic and local immune environment in patients with vesicoureteral reflux (VUR) and bladder-bowel dysfunction (BBD). METHODS: Consecutive children with VUR undergoing intravesical ureteral reimplantation were enrolled. Patients were assessed for presence of BBD by reported patient history and validated questionnaire. Fresh blood and bladder tissue, collected at the time of surgery, were immediately processed for analysis. Immune cell compositions were determined via flow cytometry. Immune cell activation was also defined at the time of analysis. LegendPlex assay analysis was utilized to define levels of circulating chemokines and cytokines. RESULTS: A total of 7 patients were enrolled. Although percentages of circulating immune cells in the blood of those with VUR/BBD and VUR alone were similar, within bladder tissue, VUR/BBD demonstrated increased immune infiltrates compared to VUR alone. Bladder sample analysis showed that B cells, and Effector Memory and Naïve T cell percentages were significantly increased in VUR/BBD patients compared to VUR patients. T cell expression of PD1 was increased in bladder tissues of BBD/VUR. Additionally, analysis of circulating neutrophils displayed significantly increased upregulation of PDL-1 in patients with VUR/BBD vs those with VUR only. CONCLUSION: These pilot data suggest an immune-rich microenvironment is present within VUR. Severity of inflammation appeared to correlate with presence of BBD. This implies that targeting pelvic inflammation may be a novel therapy for children with VUR- or non-VUR-related BBD. Follow-up studies are currently underway.

Community perspectives on difference of sex development (DSD) diagnoses: A crowdsourced survey.

INTRODUCTION AND OBJECTIVES: Differences of sex development (DSD) engender ethical, social and psychosexual complexities that can complicate medical decision-making. We performed a web-based pilot study to estimate the utility value of a DSD diagnosis and to identify community concerns regarding DSD management. METHODS: A cross-sectional survey was posted on Amazon's Mechanical Turk, an online crowdsourcing platform. Respondents were ≥18y and were randomized to receive information on one of three common DSD conditions: Congenital Adrenal Hyperplasia (CAH), Mixed Gonadal Dysgenesis (MGD), and Partial Androgen Insensitivity Syndrome (PAIS). Time trade-off methodology was used to estimate utility values. Likert scale and statement-ranking questions were used to assess respondent perceptions. RESULTS: Of 1,628 respondents, median age was 34y; most respondents were parents (59.1%), white (77.1%), and previously unfamiliar with DSD (60.4%). The median overall utility value was 0.70 (IQR 0.50-0.90), similar to moderately severe chronic health conditions. Utility estimates varied based on the DSD scenario presented (0.80 CAH vs. 0.70 MGD vs. 0.80 PAIS, p = 0.0006), respondent gender (p < 0.0001), race (p = 0.002), religion (p = 0.005), and prior knowledge of DSD (p < 0.0001). Reported concerns included gender identity (23.4%), urinary function (20.5%) and surgical complications (17.4%). Most (67.5%) supported early surgical intervention at 6-18 mo; 10.4% thought surgery should occur ≥18 y. COMMENT: Limitations of this study include that survey participants were aware of the nature of the study, thus some respondents may have participated to skew the results. Given the nature of this pilot study, the representation of families with children with DSD within the study is severely limited given the rarity of DSDs. This means that their opinions may be diluted by the large sample size. However, because utility values are classically estimated according to community opinions, the utility data presented should be taken to reflect that of the specific sample studied and is not reflective of that of families with a vested interest in such cases. CONCLUSIONS: Community-based respondents perceived that DSD conditions were associated with a reduction in utility values (0.70-0.80), on par with moderately severe chronic health conditions. Estimates varied based on respondents' gender, race, religion and prior knowledge of DSD. Gender identity was the most concerning aspect for respondents.

Delayed presentation of urethrocutaneous fistulae after hypospadias repair.

BACKGROUND: Delayed urethrocutaneous fistula (UCF) presentation after hypospadias repair is rarely reported. The aim of this study is to report our experience with delayed UCF presenting more than 5 years after hypospadias repair. METHODS: We conducted a retrospective review of patients who underwent UCF repair (CPT codes 54,340 and 54,344) at our institution between 1997 and 2017. Delayed UCF presentation was defined as a single normal urinary stream after initial hypospadias repair and subsequent presentation of a UCF/s urinary stream more than 5 years after initial hypospadias or UCF repair. Demographic and clinical data were reviewed after approval from our institutional review committee. RESULTS: We identified 12 patients with delayed UCF. The mean age at hypospadias repair was 12.3 months (Range 6-32). The mean time to delayed UCF presentation was 11.5 years (Range 7.1-15.8). Four patients with delayed UCF (33.3%) required additional surgery for UCF recurrence with a mean time to recurrence of 2.2 years (Range < 1-5.6). CONCLUSIONS: Delayed UCF presentation can occur more than 15 years after initial repair. Pubertal penile skin changes and increased genital awareness in older children may be contributing factors as all but one presented at age 10 years or older. LEVEL OF EVIDENCE: III.

Bone scan positivity in non-metastatic, castrate-resistant prostate cancer: external validation study

ABSTRACT Introduction: Tables predicting the probability of a positive bone scan in men with non-metastatic, castrate-resistant prostate cancer have recently been reported. We performed an external validation study of these bone scan positivity tables. Materials and Methods: We performed a retrospective cohort study of patients seen at a tertiary care medical center (1996-2012) to select patients with non-metastatic, castrate-resistant prostate cancer. Abstracted data included demographic, anthropometric, and disease-specific data such as patient race, BMI, PSA kinetics, and primary treatment. Primary outcome was metastasis on bone scan. Multivariable logistic regression was performed using generalized estimating equations to adjust for repeated measures. Risk table performance was assessed using ROC curves. Results: We identified 6.509 patients with prostate cancer who had received hormonal therapy with a post-hormonal therapy PSA ≥2ng/mL, 363 of whom had non-metastatic, castrate-resistant prostate cancer. Of these, 187 patients (356 bone scans) had calculable PSA kinetics and ≥1 bone scan. Median follow-up after castrate-resistant prostate cancer diagnosis was 32 months (IQR: 19-48). There were 227 (64%) negative and 129 (36%) positive bone scans. On multivariable analysis, higher PSA at castrate-resistant prostate cancer (4.67 vs. 4.4ng/mL, OR=0.57, P=0.02), shorter time from castrate-resistant prostate cancer to scan (7.9 vs. 14.6 months, OR=0.97, P=0.006) and higher PSA at scan (OR=2.91, P <0.0001) were significantly predictive of bone scan positivity. The AUC of the previously published risk tables for predicting scan positivity was 0.72. Conclusion: Previously published risk tables predicted bone scan positivity in men with non-metastatic, castrate-resistant prostate cancer with reasonable accuracy.

Pediatric Neurogenic Bladder and Bowel Dysfunction: Will My Child Ever Be out of Diapers?

CONTEXT: Managing patient and parent expectations regarding urinary and fecal continence is important with congenital conditions that produce neurogenic bladder and bowel dysfunction. Physicians need to be aware of common treatment algorithms and expected outcomes to best counsel these families. OBJECTIVE: To systematically evaluate evidence regarding the utilization and success of various modalities in achieving continence, as well as related outcomes, in children with neurogenic bladder and bowel dysfunction. EVIDENCE ACQUISITION: We performed a systematic review of the literature in PubMed/Medline in August 2019. A total of 114 publications were included in the analysis, including 49 for bladder management and 65 for bowel management. EVIDENCE SYNTHESIS: Children with neurogenic bladder conditions achieved urinary continence 50% of the time, including 44% of children treated with nonsurgical methods and 64% with surgical interventions. Patients with neurogenic bowel problems achieved fecal continence 75% of the time, including 78% of patients treated with nonsurgical methods and 73% with surgical treatment. Surgical complications and need for revisions were high in both categories. CONCLUSIONS: Approximately half of children with neurogenic bladder dysfunction will achieve urinary continence and about three-quarters of children with neurogenic bowel dysfunction will become fecally continent. Surgical intervention can be successful in patients refractory to nonsurgical management, but the high complication and revision rates support their use as second-line therapy. This is consistent with guidelines issued by the International Children's Continence Society. PATIENT SUMMARY: Approximately half of children with neurogenic bladder dysfunction will achieve urinary continence, and about three-quarters of children with neurogenic bowel dysfunction will become fecally continent. Most children can be managed without surgery. Patients who do not achieve continence with nonsurgical methods frequently have success with operative procedures, but complications and requirements for additional procedures must be expected.

Patterns of inpatient care for prostate cancer in men with spina bifida.

BACKGROUND: Advances in medical care have increased the long-term survival of patients with spina bifida. Despite this growing population, limited knowledge is available on age-related illnesses in adults with spina bifida, particularly prostate cancer for which there is no published data. OBJECTIVE: Our aim was to describe inpatient care for prostate cancer in men with spina bifida in the United States. METHODS: We performed a descriptive, retrospective study utilizing the 1998 to 2014 National Inpatient Sample from the Healthcare Cost and Utilization Project. Weights were applied to the sample to make national level inferences. We identified all adult encounters (≥18 years old) with prostate cancer and spina bifida. RESULTS: We identified 253 encounters (mean age 64.9 years). Most were Caucasian (67.5%) and had public insurance (61.6%). 44% of encounters included a major urologic procedure. 38.4% of encounters included prostatectomies, 28.3% included lymph node dissections, and 7.8% included cystectomies. Robotic surgery was performed in 9.4%. Mean length of stay was 5.6 days (95% CI: 3.7, 7.5). The average total cost was $14,074 (95% CI: $8990.3, $19,158.6). CONCLUSIONS: In this first-ever exploration of inpatient care for prostate cancer in men with spina bifida, we found that length of stay and total costs were higher in men with spina bifida. Almost half of encounters included a prostatectomy, cystectomy, and/or lymph node dissection. More detailed investigations are necessary to assess comparative treatment outcomes and complications, including prevalence and mortality rates of prostate cancer among adult men with SB.

Bone scan positivity in non-metastatic, castrate-resistant prostate cancer: external validation study.

INTRODUCTION: Tables predicting the probability of a positive bone scan in men with non-metastatic, castrate-resistant prostate cancer have recently been reported. We performed an external validation study of these bone scan positivity tables. MATERIALS AND METHODS: We performed a retrospective cohort study of patients seen at a tertiary care medical center (1996-2012) to select patients with non-metastatic, castrate-resistant prostate cancer. Abstracted data included demographic, anthropometric, and disease-specific data such as patient race, BMI, PSA kinetics, and primary treatment. Primary outcome was metastasis on bone scan. Multivariable logistic regression was performed using generalized estimating equations to adjust for repeated measures. Risk table performance was assessed using ROC curves. RESULTS: We identified 6.509 patients with prostate cancer who had received hormonal therapy with a post-hormonal therapy PSA ≥2ng/mL, 363 of whom had non-metastatic, castrate-resistant prostate cancer. Of these, 187 patients (356 bone scans) had calculable PSA kinetics and ≥1 bone scan. Median follow-up after castrate-resistant prostate cancer diagnosis was 32 months (IQR: 19-48). There were 227 (64%) negative and 129 (36%) positive bone scans. On multivariable analysis, higher PSA at castrate-resistant prostate cancer (4.67 vs. 4.4ng/mL, OR=0.57, P=0.02), shorter time from castrate-resistant prostate cancer to scan (7.9 vs. 14.6 months, OR=0.97, P=0.006) and higher PSA at scan (OR=2.91, P<0.0001) were significantly predictive of bone scan positivity. The AUC of the previously published risk tables for predicting scan positivity was 0.72. CONCLUSION: Previously published risk tables predicted bone scan positivity in men with non-metastatic, castrate-resistant prostate cancer with reasonable accuracy.

Nationwide Readmission Rates Following Percutaneous Nephrolithotomy: Does Age Matter?

Introduction/Objectives: Despite minimal evidence that evaluates the effect of age on percutaneous nephrolithotomy (PCNL) morbidity, pediatric and elderly patients are considered high-risk groups. Our objective was to assess the effect of the extremes of ages on PCNL readmission and postoperative complication rates. Methods: We identified all PCNL encounters in the 2013 and 2014 Nationwide Readmission Database. Encounters were divided into five age groups: pediatric (<18 years old), young adult (18-25 years old), adult (26-64 years old), geriatric (65-74 years old), and elderly (≥75 years old). Weighted descriptive statistics were used to describe population demographics. We fit an adjusted weighted logistic regression model for 30-day readmission and complication rates. Results: We identified 23,357 encounters. Testing average effect of pediatric and elderly encounters to all other age groups did not reveal a difference in odds for 30-day readmissions, but did result in increased odds for 30-day GU readmissions (odds ratio: 17.7 [95% confidence interval (CI): 2.65-118.9]; p = 0.003). Compared to all other age groups, elderly encounters had 7.5 (95% CI: 2.5-22.7; p = 0.0004) times the odds of a 30-day readmission and 68.3 (95% CI: 29.1-160.4; p < 0.0001) times the odds of a postoperative complication. Conclusions: When comparing the average effect of the extremes of ages to all other age groups, we did not find evidence to suggest a difference in odds for 30-day GU readmissions, but did find increased odds for complications. Further examination revealed that PCNL encounters of elderly patients had significantly increased odds for both readmission and complications, whereas PCNL encounters of pediatric patients did not.