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.

People with spina bifida use their MACE on long-term follow-up: A single institutional retrospective cohort study.

OBJECTIVE: While the Malone antegrade continence enema (MACE) facilitates bowel movements in patients with spina bifida (SB) and neuropathic bowel, little is known about its long-term use. We aimed to assess long-term MACE use and potential risk factors for disuse. METHODS: All patients with SB who underwent MACE procedures at our institution were retrospectively reviewed. Main outcome was MACE disuse (no longer catheterizing the MACE for antegrade enemas) based on self-report on a clinic questionnaire, or medical record for patients last seen before introducing the questionnaire 5 years ago. Survival analysis used two timeframes: time after surgery (Analysis 1) and chronological age: accounting for older children reaching adulthood earlier (Analysis 2). RESULTS: Overall, 411 patients (54% female, 78% shunted, 65% augmented) underwent a MACE procedure at median 7.9 years old (median follow-up: 8.4 years). Thirty-three (8%) patients no longer used their MACE. Most common reasons for doing so were channel/stomal stenosis (61%) and excision at colostomy or other abdominal surgery (12%). Bowel management afterwards included oral agents ± enemas (55%), Chait tube (30%), colostomy (12%). After correcting for differential follow-up, 90% of participants used their MACE at 10 years and 87% at 15 years after surgery. Based on chronological age, 97% used their MACE at 15 years old, 92% at 20 and 81% at 30 (Summary Figure). On multivariate analysis, umbilical MACEs were 2.4 times more likely to be disused than right lower quadrant MACEs (p = 0.04). Without correcting for chronological age (Analysis 1), patients undergoing MACE surgery at older ages were more likely to stop MACE use (p = 0.03). However, after accounting for chronological age (Analysis 2), patients undergoing a MACE procedure at older ages were no more likely to stop its use (p = 0.47, Figure). Gender, SB type, shunt status, mobility status, bladder augmentation or a urinary catheterizable channel were not associated with stopping MACE use (p ≥ 0.10). COMMENT: Participants were regularly followed in multi-disciplinary SB clinics. We did not assess continence, satisfaction or long-term urinary channel use, making it premature to recommend optimal stomal locations. CONCLUSIONS: Most patients with SB followed by a multi-disciplinary team continue using their MACE; 1% stopped MACE use annually, particularly after adolescence. This strongly suggests it is an effective bowel management method and transitioning to self-care plays a role in maintaining long-term MACE use. Umbilical MACEs may be at high risk of disuse, but all people with a MACE can benefit from support as they transition to adult care.

Sexual activity and function of adult men with spina bifida.

OBJECTIVE: Data on sexual function of men with spina bifida (SB) is limited. We aimed to assess sexual activity and erectile dysfunction (ED) in a large international sample of men with SB. METHODS: Men with SB (≥18yo) were recruited in an international online survey via clinics and social media. We collected data on demographics, ambulation (Hoffer classification), penile rigidity (Erection Hardness Score), sexual activity and ED (International Index of Erectile Function). Non-parametric tests were used. RESULTS: A total of 162 men (median age 35, 62% shunted, 38% community ambulators) reported sexual desire similar to the general population (p = 0.82), but 55% were dissatisfied with their sex life (Summary Table). Overall, 36% reported full penile rigidity with erections, more commonly with better ambulation (p = 0.01), 69% had ever experienced orgasm and 84% ejaculated. In 44 men (27%) attempting sexual intercourse in the last 4 weeks, 59% had ED (11% severe, 7% moderate, 14% mild-moderate, 27% mild). In this group, 91% of men reporting less than full penile rigidity had ED, compared to 30% with full penile rigidity (p = 0.001). Overall, partnered non-genital contact in the last 3 months was reported by 56%, solo masturbation: 62%, partnered intercourse: 48% (31% vaginal). Of 54 men who used phosphodiesterase type 5 inhibitors (PDE5I), 80% reported improved erections, 56% improved intercourse. Overall, 40% reported non-genital erogenous zones as most pleasurable, especially with poorer ambulation (p = 0.002, chest/nipples: 73%). COMMENT: Strengths of this study include anonymous, voluntary, online participation maximizing participation of a heterogenous, international population. Whenever available, we compared findings to published values for the general population. Since romantic and sexual activity is a complex intersection of interest, opportunity and ability, a more comprehensive assessment was beyond the study's scope. Future work will focus on the interplay with issues like incontinence. CONCLUSIONS: ED was frequent among men with SB, especially in men with poorer ambulation. PDE5 inhibitors may be beneficial. Partnered sexual activity was reported by half of the men, although it may not involve penetrative intercourse.

Age-based risk of end-stage kidney disease in patients with myelomeningocele.

OBJECTIVE: We aimed to quantify end-stage kidney disease (ESKD) risk after infancy in individuals with myelomeningocele (MMC) followed by urology in the modern medical era and to assess if ESKD risk was higher after surgery related to a hostile bladder. METHODS: We retrospectively reviewed patients with MMC followed by urology at our institution born ≥ 1972 (when clean intermittent catheterization was introduced) past 1 year of age (when mortality is highest, sometimes before establishing urology care). ESKD was defined as requiring permanent peritoneal/hemodialysis or renal transplantation. Early surgery related to hostile bladder included incontinent vesicostomy, bladder augmentation, detrusor Botulinum A toxin injection, ureteral reimplantation, or nephrectomy for recurrent urinary tract infections. Survival analysis and proportional hazards regression were used. Sensitivity analyses included: risk factor analysis with only vesicostomy, timing of surgery, including the entire population without minimal follow-up (n = 1054) and only patients with ≥ 5 years of follow-up (n = 925). RESULTS: Overall, 1029 patients with MMC were followed for a median of 17.0 years (49% female, 76% shunted). Seven patients (0.7%) developed ESKD at a median 24.3 years old (5 hemodialysis, 1 peritoneal dialysis, 1 transplantation). On survival analysis, the ESKD risk was 0.3% at 20 years old and 2.1% at 30 years old (Figure). This was ∼100 times higher than the general population (0.003% by 21 years old, p < 0.001). Patients who underwent early surgery for hostile bladder had higher ESKD risk (HR 8.3, p = 0.001, 6% vs. 1.5% at 30 years). On exploratory analyses, gender, birth year, shunt status and wheelchair use were not associated with ESKD risk (p ≥ 0.16). Thirty-year ESKD risk was 10% after early vesicostomy vs. 1.4% among children without one (p = 0.001). Children undergoing bladder surgery between 1.5 and 5 years old had a higher risk of ESKD. No other statistically/clinically significant differences were noted. COMMENT: Patients with MMC remain at risk of progressive renal damage throughout life. We relied on the final binary ESKD outcome to quantify this risk, rather than imprecise glomerular filtration rate formulas. Analysis was limited by few people developing ESKD, inconsistent documentation of early urodynamic findings and indications for bladder-related surgery. CONCLUSIONS: While ESKD is relatively uncommon in the MMC population receiving routine urological care, affecting 2.1% of individuals in the first 3 decades, it is significantly higher than the general population. Children with poor bladder function are likely at high risk, underlining the need for routine urological care, particularly in adulthood.

Retrograde stent with external string for pediatric robotic pyeloplasty: does it reduce cost and complications?

Robotic-assisted pyeloplasty (RAP) is a mainstay in the treatment of ureteropelvic junction obstruction (UPJO) in children. At our institution, to limit planned operating rooms visits we have placed a ureteral stent with an external string (SWES) immediately prior to RAP. In this study, we sought to quantify the operative time, complications, and costs associated with this approach compared to the traditional approach, requiring subsequent stent removal in the operating room. We hypothesized the SWES cohort would have decreased cost, yet with similar operative time and complications. We retrospectively collected all RAPs performed at our institution using the SWES approach (Aug 2012-July 2017). We excluded those with a redo pyeloplasty, and/or a percutaneous nephrostomy tube for post-operative drainage. We collected 30-day costs linked to the patients' MRN using the Pediatric Health Information System (PHIS) database. We compared 30-day healthare costs for all patients following RAP. We compared our SWES group to a national cohort of all pediatric RAP during the same time period. Lastly, we sent an anonymous, electronic survey to urologists of all PHIS institutions to identify the predominant postoperative drainage, nationally. Within our institution, we reviewed all those treated with SWES (n = 85) (Table 1). The median 30-day cost was $10,548 among those with SWES (Table 2). This was significantly less than the overall, national cohort of all pediatric RAP during the same period ($14,119, p < 0.001). There was a 15.5 % rate of unplanned return to the hospital in the SWES group. Of those unplanned returns, 8.2 % (7/85) had unplanned return for a procedure (3 for unplanned stent removal, 2 for nephrostomy tube for persistent obstruction, 1 for omental hernia, and 1 for stent replacement). With a 42.5 % (37/87) response rate, our nationwide survey found 84.6 % primarily leave stents WITHOUT a string, 7.7 % left nephrostomy tubes, and 7.7 % stents with strings. During pediatric RAP, placement of a SWES takes little time, carries a risk of unplanned visit to the operating room, saves the patient a certain, second anesthetic for stent removal, and amounts to a cost savings of approximately 25 %.

Urinary and bowel management in cloacal exstrophy: A long-term multi-institutional cross-sectional study.

BACKGROUND: We sought to evaluate long-term surgical urinary and bowel management in cloacal exstrophy (CE) in a multi-institutional study. METHODS: We performed a cross-sectional study of people with CE and covered variants managed at five participating institutions. Those with <1 year follow-up or born with variants without hindgut involvement were excluded. Primary outcomes were methods of urinary and bowel management. Urinary management included: voiding via urethra, clean intermittent catheterizations (CIC), incontinent diversion and incontinent in diaper. Bowel management included: intestinal diversion (colostomy/ileostomy) and pull-through (with/without MACE). We evaluated three age groups: children (<10 years), older children (10 to <18) and adults (≥18). We assessed if management varied by age, institution or time (born≤2000 vs. >2000). RESULTS: A total of 160 patients were included (40% male). Median follow-up was 15.2 years (36% children, 22% older children, 43% adults). While 42% of children were incontinent in diapers, 73% of older children and adults managed their bladder with CIC, followed by incontinent urinary diversion (21%) (p < 0.001, Table). CIC typically occurred after augmentation (88%) via a catheterizable channel (89%). Among older children and adults, 86% did not evacuate urine per urethra and 28% of adults had an incontinent urinary diversion. No child or adult voided per urethra. Age-adjusted odds of undergoing incontinent diversion was no different between institutions (p = 0.31) or based on birthyear (p = 0.08). Most patients (79%) had an intestinal diversion, irrespective of age (p = 0.99). Remaining patients had a pull-through, half with a MACE. The probability of undergoing bowel diversion varied significantly between institutions (range: 55-91%, p = 0.001), but not birth year (p = 0.85). SUMMARY: We believe this large long-term data presents a sobering but realistic view of outcomes in CE. A limitation is our data does not assess comorbidities or patient-reported outcomes. Rarity of volitional urethral voiding in CE forces the question of whether is a potentially unachievable goal. We advocate thoughtful surgical decision making and thorough counseling about appropriate expectations, distinguishing between volitional voiding and urinary and fecal dryness. CONCLUSIONS: In this long-term, multi-institutional study of patients with CE, 94% of older children and adults manage their bladder with incontinent diversion or CIC. Nearly 80% of patients, regardless of age, have an intestinal diversion. Given that no patients were dry and voided via urethra and 86% of older patients do not evacuate urine per urethra, these data bring into question what functional goals are achievable when performing reconstructive surgery for these patients.

Intraoperative laser angiography in bladder exstrophy closure: A simple technique to monitor penile perfusion.

BACKGROUND: The successful repair of Bladder Exstrophy remains one of the biggest challenges in Pediatric Urology. The primary focus has long been on the achievement of urinary continence. Historically there has been less focus on early penile outcomes. To this end we have incorporated penile perfusion testing using intraoperative laser angiography in to our operative approach. OBJECTIVE: We hypothesize that assessment of penile perfusion at various points in the procedure is a feasible technique that may assist in decision making during the repair of this complex condition. This will reduce the risk of tissue compression and potential loss of penile tissue that has been reported to occur as a complication of the procedure. STUDY DESIGN: Consecutive patients presenting with bladder exstrophy were evaluated at four stages of their operation (i.e. following induction of anesthesia, after bladder mobilization, following internal rotation of the pubis and at the end of the procedure) by infusing indocyanine green (ICG) at a dose of 1 mg per 10 kg body weight. Measurements were taken at 80 s post infusion and the medial thigh served as the reference control. Postoperative penile viability was evaluated by visual inspection and palpation three months following the procedure. RESULTS: Eight consecutive patients were included in this study. Perfusion was easy to measure and posed no significant technical difficulties. Penile perfusion increased slightly following bladder dissection. Internal rotation of the hips with apposition of the symphysis pubis resulted in an average 50% reduction in penile blood flow. Patients undergoing CPRE experienced an additional mean 33% drop in blood flow. In all eight cases the penis was symmetric and healthy with no sign of tissue loss at three months follow up. CONCLUSIONS: This pilot study demonstrates that the measurement of penile perfusion utilizing intraoperative laser angiography is easy to employ and should be considered a reasonable adjunct to tissue assessment in this complex condition. Marked reduction in penile blood flow may occur without any outward clinical signs. Penile perfusion is markedly reduced by apposition of the symphysis pubis and, in the immediate postoperative period, there may be further reduction in penile blood flow with CPRE as opposed to a staged repair. Future correlation with measures of penile viability and function are needed to define the clinical utility of this modality.

Solo and Partnered Sexual Behavior Among an International Sample of Adults With Spina Bifida.

BACKGROUND: Spina bifida (SB) may differentially impact adults' participation in solo and partnered sexual behaviors, but little research investigates this topic. AIM: Describe solo and partnered sexual behaviors among an international sample of adult men and women with SB. MAIN OUTCOME MEASURES: Ever participated (no/yes) and recent participation (>1 year ago/within last year) in solo masturbation, cuddled with a partner, held hands with a partner, kissed a partner, touched a partner's genital, had genitals touched by a partner, gave a partner oral sex, received oral sex from a partner, vaginal sex, anal sex, and sex toy use. METHODS: Data were drawn from a larger cross-sectional, internet-based survey assessing the sexual behaviors of an international sample of men and women with SB. We used logistic regression to examine the impact of background (gender, age, independent living, and relationship status) and health (shunt status, ambulation, and genital sensation) factors on each outcome. RESULTS: The sample consisted of 345 respondents aged 18-73 years from 26 nations. Very few (<3%) had no lifetime experience with any solo or partnered behaviors; 25.0% reported participating in all behaviors at some point in their lives. The median number of past year sexual behaviors (of 16 total) was 7. Lifetime and recent participation were associated with demographic and health factors. CLINICAL IMPLICATIONS: Despite impairment, adults with spina bifida do participate in solo and partnered sexual behaviors. Medical personnel who work with this population should include discussions about sexuality as part of routine care. STRENGTHS & LIMITATIONS: Although this research measured solo and partnered sexual behavior in large international sample of adults with spina bifida, it is limited by its cross-sectional retrospective design and non-clinical convenience sample. CONCLUSION: Despite disability, many adults with SB participate in solo and partnered sexual behavior. Medical and psychosocial supports are needed to help adults in this population enjoy sexuality in a healthy and safe manner. Hensel DJ, Misseri R, Wiener JS, et al. Solo and Partnered Sexual Behavior Among an International Sample of Adults With Spina Bifida. J Sex Med 2022;19:1766-1777.

Growing up with clitoromegaly: experiences of North American women with congenital adrenal hyperplasia.

INTRODUCTION: To describe experiences of clitoromegaly in women with congenital adrenal hyperplasia (CAH). METHODS: CAH females (46XX, ≥16 years old) from the United States and Canada were eligible for a cross-sectional online survey (2019-2020) if reporting clitoromegaly (life-long: "growing up with a larger than average clitoris," secondary: "clitoris grew over weeks or months"). A multidisciplinary team and women with CAH drafted questions assessing net effects of clitoromegaly on 10 activities and 10 life domains. Fisher's exact test was used to compare net effect (positive-negative) vs. no effect (Bonferroni p = 0.05/10 = 0.005). RESULTS: Of 97 women with CAH enrolled, 53 women (55%, median age: 36 years, advocacy group recruitment: 81%) reported recognizing clitoromegaly at median 11-13 years old, with 21% identifying it in adulthood. There was no difference in self-reported timing or clitoral shape between life-long or secondary clitoromegaly (p ≥ 0.06). There were no net positive effects of clitoromegaly. Rather, clitoromegaly had net negative effects on 7/10 activities (p ≤ 0.003) and no net effect (neutral) on 3 (Table). Women were less likely to wear tight clothing, change clothes in public locker rooms and play group sports. Women reported net negative effects for most romantic activities (dating, any sexual activity, pain-free sexual activity, having a partner see their genitalia, p=<0.003), but did not report a net effect on pleasurable sexual activity (p = 0.12). Clitoromegaly had net negative effects in 9/10 life domains (p < 0.001) and neutral on job self-perception (p = 0.25). Few women reported any positive impact (2-6%). However, 49-59% of women experienced poor self-esteem, anxiety, gender self-perception and body image, while 36% felt "down or depressed." Also, 21-23% experienced negative self-perception as friends and parents, 42-47% reported negative effects on plans for romantic and sexual relationships. Responses did not differ with advocacy group membership (p ≥ 0.02). DISCUSSION: Our findings support qualitative and case series evidence that clitoromegaly has a negative psychological outcome on women with CAH. Clitoromegaly may add to the burden of living with a chronic endocrine disease. Women with positive and negative experiences had the same opportunity to participate. Since we could not assess objective clitoral size, baseline virilization and exact nature of any childhood clitoral procedures, these data cannot be used to estimate the impact of specific clitoral size or effectiveness of early clitoral treatments. CONCLUSIONS: Clitoromegaly appears to be common among women with CAH. While experiences of clitoromegaly vary between women, the overall experience is negative in multiple social, romantic, and emotional activities and domains.

Sacral nerve stimulator success after filum section for refractory dysfunctional voiding.

INTRODUCTION: Filum section (FS) has been used to treat tethered cord syndrome (on MRI or occult) in pediatric patients with refractory dysfunctional voiding (DV). While controversial, some groups have previously reported significant improvement in patients' symptoms after FS, even in the presence of a normal preoperative spinal MRI. Until recently, it was our practice to refer patients with DV to pediatric neurosurgery for evaluation, MRI, and possible FS. OBJECTIVES: We report our experience with sacral nerve stimulator (SNS) placement for pediatric patients with refractory DV after failed FS with the primary outcome being complications and explantations. The secondary outcome being change in quality of life. STUDY DESIGN: We retrospectively reviewed all consecutive patients <18 years old who underwent second stage SNS placement after FS at our institution between November 2012 and December 2019. We abstracted rate of 2nd stage implantation, complication, and explantation. We also collected age-appropriate preoperative and postoperative 15-question quality of life questionnaires (PedsQL™). The PedsQL scores ranged 0-100, higher numbers correlating with higher quality of life. A paired t-test was used for statistical analysis. RESULTS: Overall, 23 children proceeded to the second stage SNS due to persistent symptoms following FS. Median age at SNS implantation was 10.3 years (IQR 7.5-11.8 years), and 13 were female (56.5%). There were no intraoperative complications. Median follow-up was 2.8 years (IQR 1.1-3.7 years). One patient had the SNS removed due to a need for MRI, one for resolution of symptoms, and 4 patients underwent lead revision for lead fracture and return of symptoms (17.4%). All 15 patients who completed both preoperative and postoperative PedsQL reported significantly improved overall scores after SNS (Figure). Median scores improved from 61.7 to 86.7 (p < 0.0001). DISCUSSION: Symptom relief in the child with refractory DV using SNS is not new. However, we sought to review our results in those with previous FS for tethered cord syndrome. While our study is limited by its small size, we assessed both surgical and patient-reported outcomes. Our patients' marked improvement in quality of life is similar to that of other reported pediatric SNS cohorts. CONCLUSION: SNS placement after FS is feasible, safe, and can result in significant improvement in overall quality of life. Given these findings, we have changed our practice to offer SNS in patients with refractory DV with a normal lumbosacral spinal MRI. For those with MRI abnormalities, we seek neurosurgical evaluation for FS (if deemed necessary) prior to considering SNS.

Split appendix catheterizable urinary channels are at no higher risk of undergoing revision compared to channels made with the intact appendix.

OBJECTIVE: To assess long-term APV and split-appendix MACE durability and to compare split and intact appendix APVs in a large patient cohort. METHODS: This retrospective cohort study included consecutive patients ≤21 years old undergoing an APV at our institution (1990-2019). Main outcomes were stomal and subfascial revisions. Kaplan Meier survival and Cox proportional hazards analysis were used. RESULTS: A total of 339 patients underwent APV creation at a median 7.4 years old (41% female vs. 59% male; 37% umbilical stoma vs. 63% other). In total, 36 patients underwent a stomal revision and 19 a subfascial revision (median channel follow-up 6.3 years). On survival analysis, the risk of stomal revision of the APV was 9.1% at 5 years, 12.6% at 10 years and 16.5% at 15 years. Risk of subfascial revision of the APV was 5.1% at 5 years, 7.0% at 10 years and 8.2% at 15 years. A split-appendix APV was performed in 118 (34.8%) of 339 patients. They had a shorter follow-up compared to those with an intact APV (5.1 vs. 7.0 years, p = 0.03). After correcting for differential follow-up time, there was no significant difference between groups for stomal revisions (HR 1.11, p = 0.76) or subfascial revisions (HR 0.80, p = 0.67, Figure). Risk of APV stomal revision was independent of stomal location and age at surgery (p ≥ 0.37). Similarly, risk of subfascial APV revision was independent of stomal location and age at surgery (p ≥ 0.18). Risk of stomal revision for split-appendix MACE channels was 16.2% at 5, 10 and 15 years (similar to split-appendix APV and all APVs, p ≥ 0.26). Risk of MACE subfascial revision was 5.5% at 5 years, 5.5% at 10 years and 14.7% at 15 years (similar to split-appendix APV and all APVs, p ≥ 0.36). COMMENT: We focused on surgical complications, as these entail the highest morbidity, however, we did not assess non-surgical, percutaneous or endoscopic management which also impact long-term outcome and patient quality of life. We did not compare the outcomes of the split-appendix MACE to an intact-appendix MACE cohort, as this patient population was not captured in this review. CONCLUSIONS: The split-appendix technique has durable long-term results for both the APV and MACE channels, which are comparable to the technique utilizing the intact appendix. Channel complications occur over the channel's lifetime, as 1 in 8 APVs in the entire cohort underwent a stomal revision and 1 in 14 APVs underwent a subfascial revision at 10 years after surgery.

Intrasacral Meningocele Complicating Caudal Analgesia in a 6-Year-Old Child After 3 Previous Uneventful Caudals: A Case Report.

A 6-year-old male undergoing bilateral hydrocelectomy was to receive caudal analgesia after induction of general anesthesia. After insertion of the caudal needle, cerebrospinal fluid was unexpectedly aspirated and the caudal was abandoned. The surgeon performed bilateral ilioinguinal nerve blocks just before incision. Surgery was uneventful. The patient had difficulty ambulating postoperatively, and a detailed neurologic examination revealed quadriceps weakness. A lumbosacral magnetic resonance imaging (MRI) revealed a sacral meningocele. By the next morning, quadriceps function had returned, and he was ambulating normally. The ilioinguinal block was most likely deep to the internal oblique muscle and produced femoral nerve dysfunction.

Incidence of nephrolithiasis after bladder augmentation in people with spina bifida.

INTRODUCTION: Risks of nephrolithiasis after bladder augmentation in people with spina bifida (SB) remain unclear. Annual incidence of nephrolithiasis in the general population is 0.01% for 10-14 years old, 0.07% for 15-19 years old and 0.2% for 20-24 years old. Our aim was to assess the incidence and risk factors of nephrolithiasis in SB patients after augmentation. METHODS: Patients with SB and augmentation followed at our institution were retrospectively reviewed (born ≥1972, surgery 1979-2019). Patients were screened annually with renal bladder ultrasound and abdominal radiograph. Main outcome was nephrolithiasis treatment. Kaplan-Meier survival and Cox proportional hazards analysis were used. Possible predictors were assessed using stepwise forward selection (variables with p < 0.1 on univariate analysis included in multivariate analysis). RESULTS: 427 patients with SB and augmentation were included (51.8% female, 74.9% shunted). Median age at augmentation was 8.5 years (median follow-up: 12.4 years, ileum segment: 81.0%, bladder neck procedure: 60.7%, urinary channel: 74.2%) and 28.8% developed bladder stones. Overall, 47 (11.0%) patients were treated for nephrolithiasis. After correction for differential follow-up, nephrolithiasis was treated in 7.3% at 10 years, 13.2% at 15 years, and 18.0% at 20 years (Figure). Patients presented with either a urinary tract infection (46.8%), on screening (44.7%), or pain (8.5%). Stones were treated percutaneously, endoscopically or by ESWL (63.8%/34.0%/10.7%, respectively). Most were calcium stones (58.3%). On multivariate analysis, compared to younger patients, patients augmented at ≥10 years of age had 1.84 times the risk of nephrolithiasis (p = 0.01). Nephrolithiasis was more common in those who developed bladder stones (HR = 3.00, p < 0.0001). Among those with both renal and bladder stones, bladder stones typically preceded nephrolithiasis (55.2%), were treated concurrently (31.0%) and 13.8% occurred after nephrolithiasis. Gender, wheelchair use, bowel segment used, MACE and skeletal fractures were not associated with higher nephrolithiasis risk (p ≥ 0.11). DISCUSSION: This study of a large cohort of SB patients with long-term follow-up highlights that the risk of nephrolithiasis is cumulative and related to bladder stone formation, age at augmentation and time since augmentation. An association with bladder stones suggests potential shared metabolic causes. The study's retrospective design likely led to underestimating the risk of nephrolithiasis by not capturing spontaneously passed stones. CONCLUSION: Approximately 1% of patients with SB develop nephrolithiasis annually after augmentation. Close long-term surveillance after augmentation is strongly indicated, as nephrolithiasis incidence in augmented patient with SB is at least 10 times higher than general population. Patients with bladder stones are especially at risk.

Troubleshooting problems with antegrade continent enema flushes: The Indiana university algorithm.

INTRODUCTION: Antegrade continence enemas have transformed treatment and improved the quality of life in children with neuropathic bowel, refractory constipation and fecal incontinence. However, it can often be difficult to manage problems that arise with ACE flushes. OBJECTIVE: We report the use of an online tool designed for nurses to help troubleshoot calls for problems associated with antegrade continence enema (ACE) flushes as well as update our algorithm for managing refractory constipation/fecal incontinence in a large single institution experience. STUDY DESIGN: We developed an online tool based on our management protocol for managing refractory constipation/fecal incontinence (Summary Figure). Patient frequency and bother was assessed prior to the intervention and at one month after the intervention using 5- and 4-point Likert scales respectively. Patient demographics, MACE/Chait information, type of difficulty, volume of flush, and use of additives were recorded. Nurses were also interviewed prior to using the tool and 14 months after its development with regards to taking these phone calls and the helpfulness of the tool. RESULTS: Over 14 months, the nurses received 22 patients calls via the nursing triage line regarding ACE flush problems and prospectively collected data. Half reported multiple episodes of fecal incontinence. Other complaints included no response to flush (8, 36.4%), occasional episodes of liquid fecal incontinence (2, 9.1%) and time of flush exceeding 60 min (1, 4.5%). While patients did not report decreased frequency of problems as a result of nurse troubleshooting using the ACE algorithm (2.5 vs. 2, p = 0.55), patients did report a significant improvement in their bother scores (4 vs. 2, p = 0.02). All but one patient reported that the recommendation was "some" or "a lot" helpful on follow up interview. The nurses all indicated that the tool helped "some" or "a lot." DISCUSSION: The antegrade continence enema is valuable in managing neurogenic bowel, refractory constipation, and fecal incontinence, however, some patients experience problems with flushes that can often be difficult to manage. CONCLUSION: Patients reported less bother with their bowel issues after using our algorithm for managing refractory constipation/fecal incontinence and nurses reported that the tool was helpful.

What Do Patients and Parents Say About Parents' Ability to Choose Early Genital Surgery for Girls with Congenital Adrenal Hyperplasia?

PURPOSE: We assessed opinions of females with congenital adrenal hyperplasia and their parents about the parent's ability to choose early genital surgery for these patients. MATERIALS AND METHODS: We conducted an online survey of females with congenital adrenal hyperplasia (46XX,16+ years old) and independently recruited parents (2019-2020) diagnosed in first year of life in the United States. A multidisciplinary medical team, women with congenital adrenal hyperplasia and parents drafted the survey. Fisher exact test was used. RESULTS: Of 57 females with congenital adrenal hyperplasia (median age 39 years), 93.0% underwent genital surgery (median 1-2 years old). Most females (79.0%) believed legislation prohibiting surgery in childhood would cause harm. Most (64.9%) believed a ban "would have been harmful to me" (24.6% not harmful, 10.5% neutral). Most females (70.2%) believed a ban undermined parental rights to make medical decisions in their child's best interest. While 75.4% did not believe a ban was in the best interest of females with congenital adrenal hyperplasia, 14.0% did (10.5% neutral). For 132 parents of females with congenital adrenal hyperplasia (parent/child median age 40/11 years), 78.8% of children underwent surgery (median <1 year old). Most parents (93.9%) believed legislation prohibiting surgery in childhood would cause harm. Most (77.3%) believed a ban "would have harmed my daughter" (12.1% no harm, 5.3% neutral, 5.3% no answer). Parents were more likely than females with congenital adrenal hyperplasia to oppose a ban (p ≤0.02). Most parents (90.9%) believed a ban undermined parental rights. While 93.9% did not believe a ban was in the best interest of females with congenital adrenal hyperplasia, 3.8% did (2.3% neutral). CONCLUSIONS: The majority of females with congenital adrenal hyperplasia and their parents support the parents' ability to decide about potential genital surgery, opposing moratoria on surgery in childhood.

Majority of females with a life-long experience of CAH and parents do not consider females with CAH to be intersex.

INTRODUCTION: To assess opinions of females with CAH, and parents of females with CAH, about designating this population "intersex," particularly in legislation about genital surgery during childhood. METHODS: We conducted a mixed-methods (quantitative and qualitative) anonymous cross-sectional online survey of females with CAH (46XX, 16+years old) and independently recruited parents of girls with CAH (2019-2020) diagnosed in first year of life from the United States. A multidisciplinary CAH team drafted the survey in collaboration with women with CAH and parents. Fisher's exact test was used to compare female and parent responses. A qualitative thematic approach was used to analyze open-ended answers for emergent categories of reasons why CAH females should or should not be considered as intersex. RESULTS: Of 57 females with CAH participating (median age: 39 years, 75.5% of ≥25year olds had post-secondary degree), all had classical CAH and 93.0% underwent genital surgery at median 1-2 years old. While 89.5% did not endorse the intersex designation for CAH, the remaining 5.3% did (5.3% provided no answer, Summary Figure). Most CAH females (63.2%) believed CAH females should be considered separately in "any laws banning or allowing surgery of children's genitals" (19.3% disagreed, 17.5% neutral, 0.0% no answer). Most common themes identified by females with CAH not endorsing an intersex designation were: normal female internal organs, sex chromosomes, personal identity, genital appearance, issues with language, hormones, and those endorsing it: genital appearance, community/group experiences, topic complexity. Overall, 132 parents of females with CAH participated (parent/child median ages: 40/11 years, 81.7% of ≥25year olds had post-secondary degree). All children had classical CAH and 78.8% underwent surgery at median <1 year old. While 95.5% of parents did not endorse the intersex designation for CAH, 2.3% did (2.3% no answer), similar to females (p = 0.29). Most parents (81.1%) believed CAH females should be considered separately in legislation (9.1% disagreed, 6.1% neutral, 3.8% no answer), a slightly higher percentage than females (p = 0.01). DISCUSSION: Echoing previously published disagreement with clinically designating CAH females as intersex, majority of CAH females and parents oppose a legal intersex designation. Differing opinions among females and parents strengthen concern about a one-size-fits-all approach to legislation about childhood genital surgery. Differences in opinions between female and parent responses, while statistically significant, were relatively small. CONCLUSION: Majority of females with CAH and parents believe CAH should be excluded from the intersex designation, and should be considered separately in legislation pertaining to childhood genital surgery.

Provider perspectives on shared decision-making regarding hypospadias surgery.

INTRODUCTION: Many parents experience decisional conflict and decisional regret around hypospadias surgery. The utilization of a shared decision-making (SDM) process may mitigate these issues, however addressing the principal components of the SDM process is a complex task that requires the investment of providers. OBJECTIVE: The purpose of this study was to facilitate a discussion about SDM anchored on hypospadias with pediatric urology and general pediatric providers to explore perspectives, clinical applications and barriers to adopting SDM in clinical practice. STUDY DESIGN: We conducted two focus groups in order to engage pediatric urology and general pediatric providers in guided discussions about SDM anchored on hypospadias. All activities were audio recorded and professionally transcribed. The transcripts were analyzed by three coders using directed qualitative content analysis techniques to identify themes and relationships between themes to inform the development of an affinity diagram (Extended Summary Figure). RESULTS: Two focus groups were held; one with seven pediatric urology providers in November 2018 and one with ten general pediatric providers in January 2019 (median age 51 years, 88.2% Caucasian, 58.8% female, 70.6% physicians and 29.4% nurse practitioners). Both groups identified some of the key components of SDM including engaging families in decision-making, informing them about treatment options and clarifying values/preferences (Extended Summary Figure). They thought that SDM was useful for discussing preference-sensitive conditions (e.g. hypospadias) and addressing parental compliance. General pediatric providers also suggested that SDM helped them avoid unnecessary referrals to specialists. Both groups identified parental, provider and systemic barriers to the adoption of SDM: a) desire for paternalism, b) misperceptions about medical evidence, c) completion of parental decision-making prior to the clinical visit, d) provider bias/lack of interest and e) time constraints/productivity pressures. DISCUSSION: Providers who care for hypospadias patients are knowledgeable about SDM and its potential clinical applications. They identified several potentially modifiable barriers to the adoption of a SDM process about hypospadias surgery in a pediatric clinical setting. CONCLUSIONS: Based on feedback from providers, we plan to implement a hypospadias decision aid early in the parental decision-making process about hypospadias such as in the postpartum unit and at well-child visits in the newborn period and provide a provider training session about SDM to address the identified knowledge gaps.

Endoscopic-assisted robotic pyelolithotomy: a viable treatment option for complex pediatric nephrolithiasis.

INTRODUCTION AND OBJECTIVE: Endourological and percutaneous approaches are the standard of care for treatment of pediatric urolithiasis. However, in certain situations, an endoscopic-assisted robotic pyelolithotomy (EARP) can be an acceptable alternative. Limited data exist on pediatric EARP; thus, the authors describe their experience. METHODS: Patient selection: The authors retrospectively analyzed the records of all robotic procedures performed at five institutions from 7/09-10/17 to identify patients who underwent EARP. The authors collected demographics data, indications, operative time, and postoperative complications. Stone composition was reported as the majority composition (≥50%), unless any uric acid or struvite was noted, and those stones were classified as such. TECHNIQUE: Through a traditional or hidden incision endoscopic surgery (HIdES) robot pyeloplasty approach, the authors are able to easily pass a flexible endoscope through a robotic trocar and into the renal collecting system to perform pyeloscopy or ureteroscopy. Stones were primarily retrieved via the pyelolotomy and, if indicated, treated with laser lithotripsy. RESULTS: The authors identified 26 patients who underwent EARP in 27 renal units. Median patient age was 12.2 years (interquartile range [IQR] 6.1-14.5 years), and body mass index was 17.5 kg/m2 (IQR 16.5-25.4 kg/m2). The median pre-operative dimension of the largest stone was 9.0 mm (IQR 5.8 mm-15.0 mm). Reasons for EARP: 21 (77.8%) concomitant pyeloplasty, four (14.8%) altered anatomy precluding other techniques, and two (7.4%) multiple large stones. Multiple stones were present in 20 renal units (74.1%). Stones were located in the renal pelvis in nine (33.3%), lower pole in 10 (37.0%), ureter in one (3.7%), and multiple locations in seven (25.9%). Hidden incision endoscopic surgery approach was used in 14 (51.9%), and the median operative time was 237.5 min (IQR 189.8-357.8 min) with a median length of stay 1.0 day (IQR 1.0-2.0 days). Stone composition included calcium oxalate in 14 (51.9%), calcium phosphate in five (18.5%), cysteine in two (7.4%), struvite in two (7.4%), and unknown in four (14.8%). Overall stone free status was 19 (70.4%); of the eight (29.6%) renal units with residual stones, four underwent ureteroscopy, two extracorporeal shockwave lithotripsy (ESWL), one spontaneously passed, and one underwent percutaneous nephrolithotomy (PCNL). After secondary treatment, final stone free rate was 96.3%. Complications included stent migration and admission for urosepsis. At a median follow-up of 12 months (IQR 6.2-19.2 months), five (18.5%) had stone recurrence. CONCLUSIONS: Endoscopic-assisted robotic pyelolithotomy is a reasonable treatment option for select pediatric patients with concomitant ureteropelvic junction obstruction and nephrolithiasis or pediatric patients with stones inaccessible by standard methods.

Additional Surgeries after Bladder Augmentation in Patients with Spina Bifida in the 21st Century.

PURPOSE: We determined the long-term risks of additional surgery after bladder augmentation in a modern spina bifida cohort accounting for differential followup. MATERIALS AND METHODS: We retrospectively reviewed patients with spina bifida who were born after 1972 and were followed at our institution after augmentation surgery performed between 1979 and 2018. Outcomes included diversion, bladder stones, perforation, reaugmentation, laparotomy for bowel obstruction, and benign and malignant bladder tumors. Survival analysis was used for the entire cohort and the modern cohort (detubularized and reconfigured ileocystoplasty beginning in 2000). RESULTS: A total of 413 patients were included in the study. At a median followup of 11.2 years 80.9% of the patients had undergone ileocystoplasty and 44.1% had undergone 370 additional surgeries. Ten-year risk of any reoperation was 43.9%, with 17.4% of patients undergoing 2 or more and 9.9% undergoing 3 or more additional surgeries. Outcomes included conversion to a diversion (2.7% at 10-year followup) and bladder stones (28.2% with recurrence in 52.4%) irrespective of detubularized reconfigured status (p ≥0.20). Bladder perforation risk was 9.6% for patients undergoing vs 23.7% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.01). Similarly reaugmentation rate was 5.3% for patients undergoing vs 15.2% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.001). Finally, 10-year reperforation risk was 32.1% for patients undergoing vs 73.8% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.053). Other risks included bowel obstruction (4.5% with recurrence in 15.8%), nephrogenic adenoma (2.2% with regrowth in 48.2%) and malignancy (0.0% at 20 years). For 222 patients in the modern cohort (median followup 9.1 years) 10-year risk of any reoperation was 46.0%, which consisted of diversion in 4.0%, stones in 32.9% (recurrence in 44.5%), perforation in 8.8% (recurrence in 42.2%), reaugmentation in 4.3%, obstruction in 4.9% (recurrence in 10.0%), adenoma in 4.7% (regrowth in 40.0%) and cancer in 0.0%. CONCLUSIONS: Bladder augmentation is long-lasting. While benefiting continence and renal outcomes, this operation frequently requires additional surgeries, necessitating close followup. Since survival analysis based risks of alternative management options such as incontinent diversion are unavailable, comparisons with augmentation are unfeasible.