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Objective To analyze nationwide information on the timing of surgical procedures, cost of surgery, hospital length of stay following surgery, and surgical complications of female genital restoration surgery (FGRS) in females with congenital adrenal hyperplasia (CAH). Materials and Methods We used the Pediatric Health Information System database to identify patients with CAH who underwent their initial FGRS in 2004-2014. These patients were identified by an International Classification of Diseases, Ninth Revision (ICD-9) diagnosis code for adrenogenital disorders (255.2) in addition to a vaginal ICD-9 procedure code (70.x, excluding vaginoscopy only) or perineal ICD-9 procedure code (71.x), which includes clitoral operations (71.4). Results A total of 544 (11.8%) females underwent FGRS between 2004 and 2014. Median age at initial surgery was 9.9 months (interquartile range 6.8-19.1 months). Ninety-two percent underwent a vaginal procedure, 48% underwent a clitoral procedure, and 85% underwent a perineal procedure (non-clitoral). The mean length of stay was 2.5 days (standard deviation 2.5 days). The mean cost of care was $12,258 (median $9,558). Thirty-day readmission rate was 13.8%. Two percent underwent reoperation before discharge, and 1 (0.2%) was readmitted for a reoperation within 30 days. Four percent had a perioperative surgical complication. Conclusion Overall, 12% of girls with CAH underwent FGRS at one of a national collaborative of freestanding children's hospitals. The majority underwent a vaginoplasty as a part of their initial FGRS for CAH. Clitoroplasty was performed on less than half the patients. Overall, FGRS for CAH is performed at a median age of 10 months and has low 30-day complication and immediate reoperation rates.

Purpose Sexuality has received little attention in spina bifida (SB) care. The aim of this study was to assess sexual identity and orientation in adults with SB. Methods An international online survey to adults with SB was administered over 10-months (recruitment: SB clinics, SB organizations via social media). Collected data included demographics, sexual identity and orientation. Non-parametric tests were used for analysis. Results Median age of 77 men and 119 women was 35 years old (52.0% shunted, 48.5% community ambulators, 42.3% outside United States). Most commonly, men identified as male (96.1%), while 1.3% each described themselves as female, transgender and other. All women reporting sexual identity identified as female (99.2%), 0.8% not providing an answer. Most men reported heterosexual orientation (89.6%), followed by gay (7.8%) and bisexual (2.6%). Most women reported heterosexual orientation (84.9%), followed by bisexual (10.4%), gay/lesbian (2.5%), asexual (0.8%) and other (1.7%). Conclusion As in the general population, sexual identity typically coincides with biological gender. Sexual orientation of adults with SB mirrors the general population. Due to self-selection, these findings likely do not reflect exact prevalence in the SB population.

We sought to evaluate health related benefit in children undergoing surgical reconstruction for urinary and fecal incontinence from a parental perspective.A health related benefit instrument was mailed to the parents or guardians of 300 consecutive patients who had undergone reconstruction for urinary and/or fecal incontinence at our institution between 1997 and 2011. We assessed parent reported health related benefit using the validated Glasgow Children's Benefit Inventory and satisfaction with 6 supplemental questions. One-sample t-tests as well as exploratory univariate and multivariate linear regressions were performed for statistical analysis.Response rate was 40.0% at a mean of 5.5 years (range 0.6 to 13.8) after reconstruction. Spina bifida was the most common primary diagnosis (48 patients, 56.5%). Mean total Glasgow Children's Benefit Inventory score and subscores for each domain were positive, indicating an improved health related benefit after reconstruction (all p0.0001). Certain patients, possibly based on diagnosis and gender, may particularly benefit from reconstruction (p ≤0.04). Parents believed that the bladder augmentation and/or urinary continent catheterizable channel most changed the life of their child but that achievement of stool continence was most important to themselves. Only 17 families (16.2%) required more than 1 month to become comfortable with catheterizations, and 69 patients (68.8%) required less care or no assistance with daily activities after reconstruction. Only 2 parents (1.9%) would be unwilling to consent to the procedure again.We observed moderate parental satisfaction and parent reported improvement in health related quality of life for children undergoing surgical reconstruction for urinary and fecal incontinence.

We assessed long-term renal function, morbidities and mortality in 50 patients who had undergone primary gastrocystoplasty at our institution.We retrospectively reviewed patients 21 years or younger who had undergone primary gastrocystoplasty between 1984 and 2004. Patients who underwent secondary gastrocystoplasty or primary composite augmentation or had cloacal exstrophy were excluded. Primary outcome was progression to end-stage renal disease. Secondary outcomes included mortality, bladder malignancy, hematuria-dysuria syndrome, electrolyte abnormalities and surgical revisions.Of 50 patients who had undergone gastrocystoplasty 35 met inclusion criteria. Median age was 9.4 years and 60% of the patients were male. Median followup was 19 years (IQR 11 to 25). Of the 35 patients 15 (43%) had normal preoperative estimated glomerular filtration rate and 5 (14%) had stage 2, 10 (29%) stage 3 and 5 (14%) stage 4 chronic kidney disease. Five of the 15 patients with stage 3 or 4 chronic kidney disease improved to normal estimated glomerular filtration rate, 1 remained with stage 3 disease and 9 progressed to end-stage renal disease. In 1 patient with normal estimated glomerular filtration rate end-stage renal disease developed following an episode of septic shock due to osteomyelitis. Seven patients in the cohort (20%) died, with 1 each dying of ventriculoperitoneal shunt infection, pneumonia, end-stage renal disease, complications of pregnancy and unknown cause, and 2 patients dying of septic shock due to urinary tract infection. There were no bladder malignancies. Hematuria-dysuria syndrome developed in 9 patients (24%). Eight patients (23%) underwent surgical revision.The majority of patients had preserved or improved renal function after gastrocystoplasty. There were no deaths attributable to gastric augmentation and no bladder malignancies. Approximately a fourth of patients required surgical revision.

Health-related quality of life (HRQOL) is important in spina bifida (SB) management. No clinically useful, comprehensive instrument incorporating bladder/bowel domains exists. We aimed to develop and validate a self-reported QUAlity of Life Assessment in Spina bifida for Adults (QUALAS-A).We drafted the 53-question pilot instrument using a comprehensive item generation/refinement process. It was administered to an international convenience sample of adults with SB and controls recruited online via social media and in person at outpatient SB clinics (January 2013-September 2014). Final questions were determined by: clinical relevance, high factor loadings and domain psychometrics in an Internal Validation Sample randomly selected from United States participants (n = 250). External validity was evaluated in United States and International External Validation Samples (n = 165 and n = 117, respectively). Adults with SB completed the validated general WHOQOL-BREF and International Consultation on Incontinence Questionnaire (ICIQ).Mean age of 532 participants was 32 years (32.7 % males, 85.0 % Caucasian), similar to 116 controls (p ≥ 0.08). There were 474 online and 58 clinical participants (61.1 % eligible). Face validity and content validity of the 3-domain, 15-question QUALAS-A were established by patients, families and experts. Internal consistency and test-retest reliability were high for all domains (Cronbach's alpha ≥ 0.70, ICC ≥ 0.77). Correlations between QUALAS-A and WHOQOL-BREF were low (r ≤ 0.60), except for high correlations with Health and Relationships domain (0.63 ≤ r ≤ 0.71). Bladder and Bowel domain had a high correlation with ICIQ (r = -0.70). QUALAS-A scores were lower among adults with SB than without (p0.0001). QUALAS-A had good statistical properties in both External Validation Samples (Cronbach's alpha 0.68-0.77).QUALAS-A is a short, valid HRQOL tool for adults with SB.

Renal failure has been a leading cause of death for children with spina bifida. Although improvements in management have increased survival, current data on mortality are sparse. Bladder augmentation, a modern intervention to preserve renal function, carries risks of morbidity and mortality. We determined long-term mortality and causes of death in patients with spina bifida treated with bladder augmentation.We retrospectively reviewed the records of patients with spina bifida who underwent bladder augmentation between 1979 and 2013. Those born before 1972 or older than 21 years at augmentation were excluded. Demographic and surgical data were collected. Outcomes were obtained from medical records, death records and the Social Security Death Index. Fisher exact and Wilcoxon rank-sum tests and Kaplan-Meier plots were used for analysis.Of 888 patients in our bladder reconstruction database 369 with spina bifida met inclusion criteria. Median followup was 10.8 years. A total of 28 deaths (7.6%) occurred. The leading causes of mortality were nonurological infections (ventriculoperitoneal shunt related, decubitus ulcer fasciitis, etc) and pulmonary disease. Two patients (0.5%) died of renal failure. No patient died of malignancy or bladder perforation. Patients with a ventriculoperitoneal shunt had a higher mortality rate than those without a shunt (8.9% vs 1.5%, p = 0.04).Previously reported mortality rates of 50% to 60% in patients with spina bifida do not appear to apply in children who have undergone bladder augmentation. On long-term followup leading causes of death in patients with spina bifida after bladder augmentation were nonurological infections rather than complications associated with augmentation or renal failure.

Objective It is critical to differentiate between a multicystic dysplastic kidney (MCDK) and a kidney with severe hydronephrosis as the treatment varies significantly. We designed a study to compare renal ultrasound (RUS) to nuclear medicine (NM) scan in the diagnosis of MCDK, in order to determine if RUS can be used for the definitive diagnosis of MCKD without use of NM scan. Materials and methods We performed a retrospective review of children with MCDK, who underwent both a RUS and Tc-99m MAG3 or DMSA scan. We planned to calculate the positive predictive value of an RUS diagnosis of MCDK, using NM scan diagnosis of a nonfunctioning kidney as the gold standard. Results The diagnosis of MCDK was made by RUS in 91 patients, 84 of whom had a normal bladder US. NM confirmed the diagnosis of MCDK in all 84 of these patients (100%). Conclusion We have demonstrated a high predictive value for RUS in the diagnosis of MCDK. Our data support that in healthy infants with RUS diagnosis of unilateral MCDK and normal bladder US, NM scan may be unnecessary to confirm the diagnosis.

Dietary antigen acquisition by lamina propria (LP) dendritic cells (DCs) is crucial to induce oral tolerance and maintain homeostasis. However, encountering innocuous antigens during infection can lead to inflammatory responses, suggesting processes may limit steady-state luminal antigen capture during infection. We observed that goblet cell (GC) associated antigen passages (GAPs), a steady-state pathway delivering luminal antigens to LP-DCs, are inhibited during Salmonella infection. GAP inhibition was mediated by IL-1β. Infection abrogated luminal antigen delivery and antigen-specific T cell proliferation in the mesenteric lymph node (MLN). Antigen-specific T cell proliferation to dietary antigen was restored by overriding GAP suppression; however, this did not restore regulatory T cell induction, but induced inflammatory T cell responses. Salmonella translocation to the MLN required GCs and correlated with GAPs. Genetic manipulations overriding GAP suppression, or antibiotics inducing colonic GAPs, but not antibiotics that do not, increased dissemination and worsened outcomes independent of luminal pathogen burden. Thus, steady-state sampling pathways are suppressed during infection to prevent responses to dietary antigens, limit pathogen entry, and lessen the disease. Moreover, antibiotics may worsen Salmonella infection by means beyond blunting gut microbiota colonization resistance, providing new insight into how precedent antibiotic use aggravates enteric infection.

Summary Purpose The need for mechanical inpatient bowel preparation (IBP) in reconstructive pediatric urology has come under scrutiny, secondary to literature demonstrating little benefit regarding outcomes. Starting in 2013, a majority of patients undergoing reconstructive procedures at our institution no longer underwent IBP. We hypothesized that outpatient bowel preparation (OBP) would reduce length of stay (LOS) without increasing postoperative complications after appendicovesicostomy surgery. Materials and methods An institutional database of patients undergoing lower urinary tract reconstruction between May 2010 and December 2014 was reviewed. Starting in 2013, a departmental decision was made to replace IBP with OBP. Patients undergoing an augmentation cystoplasty or continent ileovesicostomy were excluded because of insufficient numbers undergoing OBP. Patients undergoing IBP were admitted 1 day prior to surgery and received polyethylene glycol/electrolyte solution. A personalized preoperative OBP was introduced in 2013. Cost data were obtained from the Pediatric Health Information System. Results Sixty-seven patients met the inclusion criteria, with 30 (44.8%) undergoing IBP. There were no differences with respect to gender, age, presence of ventriculoperitoneal shunt, body mass index, glomerular filtration rate, preoperative diagnosis, operative time, and prior or simultaneous associated surgeries (p ≥ 0.07). Patients undergoing an IBP had a longer median LOS (7 vs. 5 days, p = 0.0002) and a higher median cost (US$4,288, p = 0.01). Postoperative complications in both groups were uncommon and were classified as Clavien–Dindo grade 1–2, with no statistical difference (IBP 20.0% vs. OBP 5.4%, p = 0.13). No serious postoperative complication occurred, such as a dehiscence, bowel obstruction, or shunt infection. Discussion This is the first analysis of hospitalization costs and IBP, showing a higher median cost of US$4288 compared with OBP. The LOS was shorter with an OBP (figure), similar to a previous report. Similar complication rates between the groups add to the growing body of literature that avoidance of IBP is safe in pediatric lower urinary tract reconstruction. Being a retrospective review of a practice change, differences in care that influenced cost and LOS may be missing. Also, as the surgeons do not know if a usable appendix is initially present, our data may not extrapolate to all patients. Despite these potential limitations, our data support the safety of utilizing OBP in patients with a high likelihood of a usable appendix, including those undergoing a synchronous Malone antegrade continence enema via a split-appendix technique. Conclusion In patients undergoing an appendicovesicostomy, preoperative IBP led to longer LOS and higher costs of hospitalization. OBP was not associated with increased risks of postoperative complications. Download : Download high-res image (84KB) Download : Download full-size image Summary Figure. . Length of hospital admission for children and adolescents undergoing appendicovesicostomy.

Summary Objective Appendicovesicostomy (APV) and Monti ileovesicostomy (Monti) are durable catheterizable channels. While subfascial revision rates vary by channel type, a channel implanted in the anterior (vs posterior) aspect of the bladder may have a lower subfascial revision risk, due to decreased channel mobility and better fascial fixation. The present study aimed to compare long-term durability of anteriorly compared to posteriorly implanted APV and Monti channels in a large international cohort. Materials and methods A retrospective cohort study was conducted on patients aged ≤21 years and who underwent APV or Monti surgery with an open technique at three high-volume centers (1990–2015). The following were noted: patient demographics, stomal and subfascial revisions, stomal location, channel placement (anterior/posterior), and channel type – APV, spiral Monti to umbilicus (SMU), other Monti channels. Survival analysis and Cox proportional hazards regression were used to separately examine the three channel groups. Results Of the 675 patients who met inclusion criteria, 387 had an APV (71.3% anterior), 53 had an SMU (13.2% anterior) and 235 had other Monti channels (42.1% anterior). Median age at surgery was 8.8 years for APV (median follow-up: 5.5 years), 9.2 years for other Monti (follow-up: 6.6 years) and 7.9 years for SMU (follow-up: 9.0 years). Patients originated from the USA (67.9%), Argentina (26.4%) and Chile (5.8%). Overall, 76 stomal and 77 subfascial revisions occurred. Risk of stomal revision was 9.3–12.0% at 5 years of follow-up, and was similar between channel types or location (P = 0.57). Risk of subfascial revision at 5 years was 7.4% for APV, 12.7% for all other Monti channels and 25.9% for SMU (P = 0.001). On survival analysis, stomal and subfascial revision rates were similar between anterior and posterior channels for APV (P ≥ 0.16), other Monti channels (P ≥ 0.62) and SMU (P ≥ 0.43) (Summary Fig.). On multivariate regression, channel configuration was not associated with stomal or subfascial revision for APV (P ≥ 0.18) or other Monti channels (P ≥ 0.64). Sex, age, diagnosis, country and stomal location were not associated with revision risk (P ≥ 0.06). Discussion Contrary to the hypothesis, subfascial revision rates were no different between anterior and posterior channels. Given that many reported outcomes related to genitourinary reconstruction occur rarely and require prolonged follow-up, collaborative research in this area should be encouraged. Conclusions The study demonstrated durable long-term results with the APV and Monti techniques in an international cohort. Risks of stomal and subfascial complications were not significantly different between anteriorly and posteriorly implanted channels. As previously reported, Monti channels, particularly SMU, were more prone to undergoing subfascial revisions. Download : Download high-res image (398KB) Download : Download full-size image Summary Fig . Probability of remaining without subfascial revision, between anterior and posterior channels, for appendicovesicostomy (APV), other Monti channels, and spiral Monti to the umbilicus.

The most extensive public debate on the moral dilemmas of life in the Japanese-occupied Philippines (1941–1945) took place between the first elected legislators of an independent Philippine republic in 1948 over whether to approve a partial presidential amnesty for wartime collaborators. The eventual approval of the amnesty brought an end to a struggling People’s Court system of trying treason cases, abandoned prosecutions for all accused political and economic collaborators, and transferred other cases of military collaboration and informers to regular criminal courts. The chapter explores some of the objections to the amnesty on the grounds of class discrimination that ultimately failed to persuade the majority but argues that, on all sides, participants saw the Philippine experience as deeply integrated in a broader global process of confronting the legacies of brutal foreign occupations.

Bladder stones are common after bladder augmentation, often resulting in numerous procedures for recurrence. We sought to determine whether surgical technique and stone fragmentation are significant predictors of bladder stone recurrence after bladder augmentation.We retrospectively reviewed 107 patients treated for first bladder stones at our institution. Patient demographics, details of surgeries, stone therapy and recurrence were reviewed. Kaplan-Meier survival and Cox proportional hazards analysis were used to determine predictors of time to first stone recurrence.Of 107 patients 55.1% were female and 79.4% had neuropathic bladder. Patients underwent augmentation at a median age of 8.0 years (range 2.4 to 22.8) and were followed for a median of 12.4 years (1.8 to 34). Segments used for augmentation included ileum (72.9% of cases), sigmoid (16.8%), cecum/ileocecum (9.4%) and other (ureter, stomach/ileum, 1.8%). Bladder neck procedures were performed in 63.6% of patients and catheterizable channels in 75.7%. First stone surgery occurred at a median of 3.1 years after augmentation (range 5 months to 21.8 years). Endoscopy was used in 66.4% of cases and open cystolithotomy in 33.6%. Overall 47.7% of stones were fragmented. Bladder stones recurred in 47.7% of patients (median recurrence time 9.5 years, range 3 months to 14.7 years). Recurrence risk was greatest in the first 2 years postoperatively (12.1% per patient per year, p = 0.03). Recurrence risk did not change with technique (endoscopic vs open) or fragmentation, even after controlling for surgical and clinical variables.Bladder stones recurred in almost half of the patients at 9 years postoperatively independent of treatment technique and patient characteristics. As a high risk group, yearly x-ray of the kidneys, ureters and bladder, and ultrasound of the kidneys and bladder are recommended in these patients.

The use of vibrational spectroscopy, FTIR and Raman, for cytology and cellular research has the potential to revolutionise the approach to cellular analysis. Vibrational spectroscopy is non-destructive, simple to operate and provides direct information. Importantly it does not require expensive exogenous labels that may affect the chemistry of the cell under analysis. In addition, the advent of spectroscopic microscopes provides the ability to image cells and acquire spectra with a subcellular resolution. This introductory review focuses on recent developments within this fast paced field and highlights potential for the future use of FTIR and Raman spectroscopy. We particularly focus on the development of live cell research and the new technologies and methodologies that have enabled this.

To assess long-term renal preservation and surgical outcomes in patients undergoing ureteric substitution with ileum. This has been a mainstay of reconstruction options for lengthy ureteral defects.Consecutive patients aged 18 or older undergoing ileal ureters at our institution were retrospectively reviewed (from 1989 to June 2013). Patients with6 months of follow-up were excluded. Demographic, surgical, and renal functional outcomes were reviewed. Renal function was assessed by the Chronic Kidney Disease Epidemiology Collaboration and Modification of Diet in Renal Disease Study equations.Of the 108 patients meeting the inclusion criteria, 86 (79.6%) had single-renal unit reconstruction and 22 had bilateral reconstruction. Eighty-four (77.8%) had radiation-induced stricture and 24 had iatrogenic or trauma-induced strictures. The median follow-up was 51 months (interquartile range: 22-112). Short-term complications included Clavien grade I (16 [14.8%]), Clavien grade II (3 [2.8%]), Clavien grade III (9 [8.3%]), and Clavien grade IV (3 [2.8%]). Long-term complications included fistula in 6 patients (5.6%), renal failure requiring dialysis in 2 patients (1.9%), hyperchloremic metabolic acidosis in 4 patients (3.7%), and incisional hernia in 11 patients (10.2%). Nine patients (8.3%) had small-bowel obstructions; 3 (2.8%) required adhesiolysis. Four patients (3.7%) had an anastamotic stricture. Nineteen patients (17.6%) had worsening renal function. Cox proportional hazards regression found that those with bilateral repair were at 3.7 times increased risk of worsening renal function (P = .02).Ureteral substitution with ileum provides an effective and versatile long-term reconstructive option with minimal renal function compromise in properly selected patients. Bilateral reconstruction may contribute to worse long-term renal function.

Subglottic stenosis (SGS) is a challenging disease to diagnose in neonates. Long-range optical coherence tomography (OCT) is an optical imaging modality that has been described to image the subglottis in intubated neonates. A major challenge associated with OCT imaging is the lack of an automated method for image analysis and micrometry of large volumes of data that are acquired with each airway scan (1 to 2 Gb). We developed a tissue segmentation algorithm that identifies, measures, and conducts image analysis on tissue layers within the mucosa and submucosa and compared these automated tissue measurements with manual tracings. We noted small but statistically significant differences in thickness measurements of the mucosa and submucosa layers in the larynx (p 0.001), subglottis (p = 0.015), and trachea (p = 0.012). The automated algorithm was also shown to be over 8 times faster than the manual approach. Moderate Pearson correlations were found between different tissue texture parameters and the patient’s gestational age at birth, age in days, duration of intubation, and differences with age (mean age 17 days). Automated OCT data analysis is necessary in the diagnosis and monitoring of SGS, as it can provide vital information about the airway in real time and aid clinicians in making management decisions for intubated neonates.

Historically, there have been few treatment options for children with severe refractory bladder and bowel dysfunction (BBD). Sacral neuromodulation (SNM) continues to show promising results in this challenging pediatric population with recalcitrant lower urinary tract symptoms. At the authors institution, they have begun offering explantation to those with persistent improvement after6 months of having device turned off. The authors hypothesized that (1) SNM explantation for cure increases with extended follow-up and (2) those explanted for cure would have improved symptoms and quality of life when compared to those explanted for complication.The authors retrospectively reviewed all consecutive patients aged18 years who underwent SNM placements at their institution (2012-2017). They excluded those without the second stage procedure. Reasons for device explantation were categorized as cure (resolution of symptoms with the device turned off for at least 6 months) or a complication (e.g. infection, need for magnetic resonance imaging, or pain). Non-parametric tests and survival analysis were used for analysis to account for differential follow-up time. Of those explanted, surveys were electronically sent to assess BBD severity and overall quality of life.Of 67 children who underwent a first stage procedure, 62 (92.5%) underwent a second stage procedure. 61 met inclusion criteria (68.9% female, 29.5% with previous filum section, median age at implantation 10.3 years). During follow-up (median 2.3 years), 12 patients (19.7%) had the SNM exchanged/revised because of lead fracture/breakage and return of urinary symptoms. To date, 50 patients remain with their SNM implanted, and 11 have been explanted. Adjusting for follow-up time, the risk of explantation was 6.5% at 2 years (2.2% for cure, 4.3% for complications) (Figure 1). Explantation increased to 24.5% at 3 years (16.5% for cure, 8.0% for complications) and 40.4% at 4 years (32.4% for cure, 8.0% for complications). Questionnaires were collected on patients after explant (median 2.2 years), with improvement in those explanted for cure compared to complication (Figure 2).Sacral neuromodulation explantation for cure is a novel concept previously not described in the literature. Limitations of this study include the relatively small numbers and lack of objective data in the cohort that remains with SNM device implanted.Sacral neuromodulation is a safe, viable option for the pediatric patient with refractory bladder dysfunction. Furthermore, SNM explantation for cure is an option with increasing likelihood after 2 years.

Summary Introduction It has been established that patients with congenital diseases, including spina bifida (SB) are often lost to routine medical care in young adulthood. While the reasons for this observation are not entirely defined, many believe it is multifactorial. Objective This study sought to characterize self-reported barriers to transition among adults with SB who failed to transition to adult urological care. Study design This study, conducted at two tertiary centers with established adult myelomeningocele multidisciplinary clinics, enrolled patients with SB who had not been seen in >18 months. A visit was scheduled and upon arrival patients were provided a questionnaire with 22 yes/no questions about barriers to arranging a visit and eight questions regarding their health issues. Questions were categorized as relating to patient factors, provider factors, and system factors. To facilitate targeted areas of improvement, patient factors were further subdivided into self-management/support (SMS), preferences, and education, and provider factors into knowledge, communication, and location. System factors included only questions related to insurance issues. The questionnaire was uncomplicated, which eliminated the need for assistance when answering the questions. Results There were 27 questionnaires collected. Overall, a total of seven patients (26%) identified no barriers to follow-up, eight identified one barrier (30%), and 12 (44%) identified more than one barrier. Patient factors made up approximately 52% (28 of 54 total “Yes” responses) of all barriers, with Preference being the largest contributor (30%, 16/54) (Fig.). Provider factors accounted for about 37% (20/54) of all barriers, with the Communication (26%, 14/54) subcategory making up the vast majority. System factors contributed only 11% (6/54) of all barriers. The majority of patients (18/27, 67%) reported having a new health issue since their last visit to their pediatric urologist, with only five of 18 (28%) seeking urological care. Discussion This study demonstrated that the decision to pursue adult urologic care is multifactorial. The questionnaire may not have captured all of the reasons SB patients are lost to follow-up, as a quarter of patients did not identify a barrier. Limitations of this study include the small number of participants, use of only two study locations, and the closed-ended nature of the questionnaire. Conclusion Multiple factors contribute to the SB patient's decision to pursue adult urologic care. Targeted areas of improvement include provider communication at both the pediatric and adult level, as well as education regarding patient preferences and SMS. Download : Download high-res image (118KB) Download : Download full-size image Figure . Overall proportion of barriers to establishing follow-up among adults with spina bifida. SMS = self-management/support.

Summary Purpose The parental decision-making process regarding female genital restoration surgery (FGRS) for girls with congenital adrenal hyperplasia (CAH) is controversial and poorly understood. The aim of the study aim was to evaluate parental concerns related to their child's future and parental plans about disclosure prior to FGRS. Materials and methods The authors performed an online survey of consecutive parents presenting at a tertiary referral center for consultation regarding FGRS for their daughter with CAH before 3 years of age (2016–2018). Twenty issues initially identified by three families and six clinicians were rated on a 6-point Likert scale of importance (‘not at all’ to ‘extremely’). Results Sixteen consecutive families participated (Prader 3/4/5: 43.8%/43.8%/12.5%). Fourteen girls (87.5%) subsequently underwent FGRS at a median age of 8 months. Most issues (19/20, 95.0%) were ranked ‘quite a bit’ to ‘extremely’ important ( Table ). Top issues were not surgical: Normal physical/mental development, adrenal crisis and side-effects of medications. Surgery-related and self-image concerns followed in importance. Least prioritized issues were multiple genital exams (‘quite a bit’ important) and the child not being involved in the decision to proceed with FGRS (‘somewhat’ important). On average, no issues were considered ‘not at all’ or ‘a little’ important. Disclosure of FGRS to their daughter was the 15th prioritized issues. Almost all families (93.8%, 1 unsure) planned to disclose the surgery to their daughter, although many were unsure when and how to do it (33.3% and 37.5%, respectively). Comment Initial efforts to understand the complex process of parental decision-making regarding FGRS in the context of CAH, a complex, multifactorial disease, are presented. Parents of infant girls with CAH simultaneously weigh multiple life-threatening concerns with a decision about FGRS. While issues of genital ambiguity and surgery are important, they are not overriding concerns for parents of girls with CAH. Parents report significant uncertainty about appropriate timing and approach to disclosing FGRS to their daughters. Unfortunately, best practice guidelines for this process are lacking. The findings are not based on actual history of disclosure but on parents' anticipated behavior. Further data are need from parents, children, and women with CAH about successful disclosure. Being a single-center series, these data may not correspond to the wider CAH community. Conclusions Parental decision-making regarding FGRS is multifactorial. Even when considering FGRS, parents' largest concerns remain focused on the life-threatening and developmental effects of CAH and side-effects of its medical treatment. The disclosure process deserves further attention. Table . Parent-reported importance of issues prior to female genital restoration surgery (no issue had a mean important score below ‘somewhat’ important, n = 16). Rank Category Issue Importance score Importance category 1 Developmental Normal physical and mental development 92.5 Extremely (90.0–100.0) 2 Medical My child having an adrenal crisis 88.8 Very much (70.0–89.9) 3 Medical Side-effects from medications 88.8 4 Surgery/genital My child's future ability to have children 85.0 5 Surgery/genital My child's future ability for sexual intercourse 80.0 6 Developmental My child having problems with her self-image 80.0 7 Surgery/genital My child having a complication after surgery 78.8 8 Developmental Not letting CAH define her life 77.5 9 Surgery/genital General appearance of my child's genitalia 76.7 10 Surgery/genital My child needing possible future surgery 76.7 11 Surgery/genital Decreased sensation of my child's clitoris 72.5 12 Developmental My child's future gender identity 67.5 Quite a bit (50.0–69.9) 13 Medical Privacy about my child's medical history 65.0 14 Surgery/genital Size of my child's clitoris 65.0 15 Surgery/genital Disclosing the surgery to my child 63.8 16 Family stress Stress on our family 61.3 17 Developmental How other people will view my child 58.8 18 Developmental My child finding love and acceptance in the future 57.5 19 Surgery/genital My child having multiple genital exams by doctors in the future 50.0 20 Surgery/genital My child not having had a voice in choosing surgery 28.8 Somewhat (30.0–49.9) n/a n/a n/a n/a A little (10.0–29.9) n/a n/a n/a n/a Not at all (0.0–9.9) CAH, congenital adrenal hyperplasia.

Advanced technology and economics of scale are the principal determinants in EUROVISION's structure of international cooperation. (Author/STS)