Time to therapy and safety of testicular tissue cryopreservation in children undergoing gonadotoxic treatment or hematopoietic stem cell transplant.

BACKGROUND: With the use of multimodal treatments and hematopoietic stem cell transplant, the majority of children diagnosed with malignancies and hematologic diseases are now surviving into adulthood. Due to the gonadotoxic effects and potential for future infertility associated with many of these treatments, fertility counseling with sperm cryopreservation prior to starting therapy is the standard of care for post-pubertal males. Unfortunately, the options are limited for pre-pubertal patients or those unable to provide a specimen. Testicular tissue cryopreservation (TTC) is an investigational method to surgically obtain germ cells from testicular tissue and potentially restore future spermatogenesis. While TTC has been shown to be safe, little is reported on the time to treatment following the procedure to ensure adequate wound healing and avoid delays in definitive therapy. OBJECTIVES: The primary outcome was the time to initiation of treatment following TTC. Secondary outcomes were complication rates, delays in treatment due to TTC, and presence of germ cells. METHODS: We conducted a single-institution retrospective cohort study of patients undergoing TTC between 2017 and 2023. Patients at significant risk for treatment related infertility based on established criteria were eligible for TTC. Patients were excluded if they received their oncology or hematology care elsewhere. All patients were enrolled in an IRB approved research protocol with 75% of the tissue submitted for cryopreservation and 25% for research purposes. Time to therapy was defined as the first receipt of gonadotoxic treatment following TTC. RESULTS: A total of 122 patients (53 = malignant, 69 = non-malignant) underwent TTC with a median age of 5.9 years (IQR 2.3-9.35). Germ cells were identified in 115 (94%) specimens. A total of 109 (89%) patients underwent concomitant procedures. The median time to initiation of therapy was 5 (IQR 1.0-7.0) and 7 days (IQR 6.0-13.0) for malignant and non-malignant disease, respectively. The 30-day surgical complication rate was 2.5% and was similar between malignant vs non-malignant diagnoses (p = 0.58). All surgical complications were managed non-operatively. No patients had a delay in definitive treatment due to concern for wound healing or complications. DISCUSSION: Our surgical complication rates are similar to previous studies and are not affected by the time to treatment following TTC. Limitations of the study are its retrospective design, single institution, and short-term follow up. CONCLUSION: TTC can be performed safely, efficiently, and in conjunction with other necessary procedures without resulting in delays of definitive treatment. TTC affords the opportunity for fertility preservation in children who have no other options.

Expert Consensus on Pediatric Urodynamics Reporting Using Modified Delphi Technique.

PURPOSE: Urodynamic testing (UDS) is an important tool in the management of pediatric lower urinary tract conditions. There have been notable efforts to standardize pediatric UDS nomenclature and technique, but no formal guidelines exist on essential elements to include in a clinical report. We sought to identify ideal structure and elements of a pediatric UDS assessment based on expert consensus. MATERIALS AND METHODS: Pediatric urologists regularly performing UDS were queried using a Delphi process. Participants were invited representing varied geographic, experience, and societal involvement. Participants underwent 3 rounds of questionnaires between November 2022 and August 2023 focusing on report organization, elements, definitions, and automated electronic health record clinical decision support. Professional billing requirements were also considered. Consensus was defined as 80% agreeing either in favor of or against a topic. Elements without consensus were discussed in subsequent rounds. RESULTS: A diverse sample of 30 providers, representing 27 institutions across 21 US states; Washington, District of Columbia; and Canada completed the study. Participants reported interpreting an average number of 5 UDS reports per week (range 1-22). The finalized consensus report identifies 93 elements that should be included in a pediatric UDS report based on applicable study conditions and findings. CONCLUSIONS: This consensus report details the key elements and structure agreed upon by an expert panel of pediatric urologists. Further standardization of documentation should aid collaboration and research for patients undergoing UDS. Based on this information, development of a standardized UDS report template using electronic health record implementation principles is underway, which will be openly available for pediatric urologists.

Feasibility of Enhanced Recovery After Surgery (ERAS) implementation in Pediatric Urology: Pilot-phase outcomes of a prospective, multi-center study.

INTRODUCTION/BACKGROUND: Enhanced Recovery After Surgery (ERAS) is a fundamental shift in perioperative care that has consistently demonstrated an improved outcome for a wide variety of surgeries in adults but has only limited evidence in the pediatric population. OBJECTIVE: We aimed to assess the success with and barriers to implementation of ERAS in a prospective, multi-center study on patients undergoing complex lower urinary tract reconstruction. STUDY DESIGN: Centers were directed to implement an ERAS protocol using a multidisciplinary team and quality improvement methodologies. Providers completed pre- and post-pilot surveys. An audit committee met after enrolling the first 5 patients at each center. Pilot-phase outcomes included enrollment of ≥2 patients in the first 6 months of enrollment, completion of 90 days of follow-up, identification of barriers to implementation, and protocol adherence. RESULTS: A total of 40 patients were enrolled across 8 centers. The median age at surgery was 10.3 years (IQR 6.4-12.5). Sixty five percent had a diagnosis of myelomeningocele, and 33 % had a ventriculoperitoneal shunt. A bladder augmentation was performed in 70 %, Mitrofanoff appendicovesicostomy in 52 %, Monti ileovesicostomy in 15 %, and antegrade continence enema channel in 38 %. The most commonly perceived barriers to implementation on the pre-pilot survey were "difficulty initiating and maintaining compliance with care pathway" in 51 % followed by a "lack of time, money, or clinical resources" in 36 %. The pre-pilot study experience, implementation, and pilot-phase outcomes are provided in the Table. All primary and secondary outcomes were achieved. DISCUSSION: The findings of the present study were similar to several small comparative studies with regard to the importance of a multidisciplinary team, strong leadership, and continuous audit for successful implementation of ERAS. Similar barriers were also encountered to other studies, which primarily related to a lack of administrative support, leadership, and buy-in from other services. The limitations of the present study included a relatively small heterogeneous cohort and absence of a comparative group, which will be addressed in the larger exploratory phase of the trial. The findings may also not be generaziable due to the need for sustainable processes that were unique to each center as well as an absence of adequate volume or resources at smaller centers. CONCLUSIONS: ERAS was successfully implemented for complex lower urinary tract reconstruction across 8 centers through a multidisciplinary team, structured approach based on the local context, and focus on a continuous audit.

One and done: Feasibility and Safety of Primary Ureteroscopy in a Pediatric Population.

BACKGROUND: Pediatric urolithiasis has been increasing at rate of 4-10 % annually in the United States, most notably within adolescents and females. A significant number of patients will require surgical management of their stones. Primary ureteroscopy (URS) affords the opportunity to treat stones under a single anesthetic with lower re-treatment rates or anatomical and stone characteristic limitations compared to shockwave lithotripsy. Previous studies evaluating primary URS have been largely underpowered, are limited by stone location, and/or are not representative of the stone population in the United States. OBJECTIVES: Primary study outcomes were the success of primary URS and patient characteristics associated with success. Secondary outcomes were the stone-free rate (SFR), 30-day emergency department (ED) visits, 30-day readmissions, and complications. METHODS: We performed a retrospective cohort study of patients less than 18 years of age from 2011 to 2023 who underwent primary URS. Patients were excluded if a ureteral stent was placed prior to URS or diagnostic URS was performed. A successful primary URS was considered if access to the ureter was obtained and treatment of the stone(s) completed. In failed primary URS, a ureteral stent was placed for staged management. RESULTS: A total of 196 patients were included and primary URS was performed or attempted on 224 renal units. The median age was 15.8 (IQR 13.4-16.9) years and median follow up 8.4 (IQR 1.1-24.6) months. The success rate of primary URS was 79 %. No significant characteristics were appreciated for successful primary URS based on: overall age, <14 vs > 14 years of age, sex, body mass index, history of stones, history of endourologic procedures, preoperative alpha blockade, location of stone(s), multiple stones, type of URS, or acute treatment. In successful primary URS, the SFR was 88 % with stone size (p = 0.0001) the only predictor of having residual stones. The 30-day ED rate was 21.4 %, 30-day unplanned readmission rate was 12.5 %, and complication rate was 7.5 %. No long-term complications were appreciated. DISCUSSION: Our success of primary URS compares favorably to previously published literature. Our SFR rate, 30-day ED visits, 30-day unplanned readmission, and complication rates are similar to other studies. Limitations of the study are its retrospective design, selection bias, and intermediate follow-up. CONCLUSIONS: Primary URS can be completed safely in the majority of pediatric patients without any patient characteristics associated with success. We advocate for primary URS when possible due to the excellent SFR and potential of treating stones under a single anesthetic.

Clinical considerations in adults with history of posterior urethral valves.

INTRODUCTION: Management of patients with posterior urethral valves (PUV) is commonplace for many pediatric urologists, however adult providers may be far less familiar with this diagnosis, its management and long-term ramifications. As urologic management of these patients has evolved, clinical outcomes have substantially improved with many more patients now surviving into adulthood. These patients remain at increased risk of morbidity due to their condition and therefore are likely to benefit from long term follow-up with adult providers. OBJECTIVE: In this review we analyze the psychosocial impacts of PUV on adults, evaluate long term transplant outcomes in PUV patients and discuss effective clinical management strategies of bladder dysfunction in adult PUV patients. STUDY DESIGN: A retrospective literature review was performed using the MEDLINE (Pubmed) electronic database using key words such as "posterior urethral valve", "quality of life", "sexual function", "transplant outcomes", "bladder dysfunction", "mitrofanoff" etc. to identify relevant studies. RESULTS: Generally, the quality of life of PUV patients is good, those suffering from renal insufficiency or lower urinary tract symptoms, specifically incontinence, appear to be a group that may benefit from more intensive follow-up. Good long-term kidney transplant (KT) function and survival can be achieved in patients with PUV. Rigorous management to optimize bladder function and close follow-up, are key for long term graft survival after KT. DISCUSSION: The chronicity of PUV warrants adult providers to be not only well versed in the pathophysiology of the disease, but well prepared to care for these patients as they transition into adulthood. CONCLUSION: Additional studies addressing psychosocial, clinical and transplant outcomes of adults with PUV are necessary to develop optimal long-term follow-up regimens for these patients.

Decisional Support Needed when Facing Tough Decisions: Survey of Parents with Children having Differences of Sex Development.

Introduction: Parents of infants and young children newly diagnosed with differences of sex development (DSD) commonly face medical and psychosocial management decisions at a time when they are first learning about the condition and cannot consult their child for input. The aim of this study was to identify areas of greatest need for parental decisional support. Methods: 34 parents of children receiving care for DSD at one of three US children's hospitals participated in a survey to learn what clinical and psychosocial decisions needed to be made on behalf of their child. Parents were then asked to identify and focus on a "tough" decision and respond to questions assessing factors affecting decision-making, decision-making preferences, decisional conflict, and decision regret. Descriptive analyses were conducted. Results: Decisions about surgery and aspects of sharing information about their child's condition with others were the two most frequently reported decisions overall, experienced by 97% and 88% of parents, as well as most frequently nominated as tough decisions. Many parents reported mild to moderate levels of decisional conflict (59%) and decision regret (74%). Almost all parents (94%) reported experiencing at least one factor as interfering with decision-making (e.g., "worried too much about choosing the 'wrong' option"). Parents universally reported a desire to be involved in decision-making - preferably making the final decision primarily on their own (79%), or together with their child's healthcare providers (21%). The majority of parents judged healthcare providers (82%) and patient/family organizations (58%) as trustworthy sources of information. Discussion: Parents of children with DSD encounter medical, surgical, and psychosocial management decisions. Despite difficulties including emotional distress and informational concerns (including gaps and overload), parents express strong desires to play key roles in decision-making on behalf of their children. Healthcare providers can help identify family-specific needs through observation and inquiry in the clinical context. Together with families, providers should focus on specific clinical management decisions and support parental involvement in making decisions on behalf of young children with DSD.

Co-creating a suite of patient decision aids for parents of an infant or young child with differences of sex development: A methods roadmap.

Introduction: Parents and guardians of infants and young children with differences of sex development (DSD) often face numerous health and social decisions about their child's condition. While proxy health decisions can be stressful in any circumstance, they are further exacerbated in this clinical context by significant variations in clinical presentation, parental lack of knowledge about DSD, irreversibility of some options (e.g., gonadectomy), a paucity of research available about long-term outcomes, and anticipated decisional regret. This study aimed to engage clinicians, parents, and an adult living with DSD to collaboratively develop a suite of patient decision aids (PDAs) to respond to the decisional needs of parents and guardians of infants and young children diagnosed with DSD. Methods: We used a systematic co-development process guided by the Ottawa Decision Support Framework and the International Patient Decision Aids Standards (IPDAS). The five steps were: literature selection, establish the team, decisional needs assessment, create the PDAs, and alpha testing. Results: We developed four PDAs to support parents/guardians of infants or young children diagnosed with DSD about four priority decisions identified through our decisional needs assessment: genetic testing, gender of rearing, genital surgery and gonadal surgery. All four PDAs include information for parents about DSD, the options, reasons to choose or avoid each option, and opportunities for parents/guardians to rate the importance of features of each option to clarify their values for these features. Qualitative feedback was positive from clinicians, parents and an adult living with DSD. Conclusions: These PDAs are clinical tools designed to support parents/guardians and to promote making an informed and shared DSD-related decision. While these tools are specific to DSD, they contain themes and elements translatable to other pediatric populations.

Long-Term Male Sexual Function and Fecal Incontinence Outcomes for Adult Patients with Hirschsprung Disease or Anorectal Malformation.

BACKGROUND: This study sought to characterize sexual function and fecal incontinence related quality of life (QOL) outcomes for adult males with anorectal malformation (ARM) or Hirschsprung's Disease (HD). METHODS: We conducted a cross-sectional survey study of male patients ≥18 years with ARM or HD. Patients were identified from our institutional database, contacted and consented by telephone, and sent a REDCap survey via email. The International Index of Erectile Function (IIEF-5) and Male Sexual Health Questionnaire (MSHQ) evaluated erectile dysfunction (ED) and ejaculatory dysfunction (EjD), respectively. The Cleveland Clinic Incontinence Score (CCIS) and the Fecal Incontinence Quality of Life Scale (FIQLS) assessed fecal incontinence-related outcomes. A linear regression analysis of IIEF-5 scores compared to CCIS scores was used to evaluate for an association between ED and incontinence. RESULTS: Of 63 patients contacted, 48 completed the survey. The median age for respondents was 22.5 years (IQR 20-25). There were 19 patients with HD and 29 patients with ARM. On the IIEF-5 survey, 35.3% report some level of ED. On the MSHQ-EjD survey, the median score was 14 out of 15 (IQR 10.75-15), indicating few EjD concerns. The median CCIS was 5 (IQR 2.25-7.75) and the median FIQL scores ranged from 2.7 to 3.5 depending on the domain assessed, demonstrating some QOL challenges secondary to fecal incontinence. On linear regression analysis, IIEF-5 and CCIS scores were weakly associated (B = -0.55, p = 0.045). CONCLUSIONS: Male adult patients with ARM or HD may have ongoing concerns with sexual function and fecal incontinence. LEVEL OF EVIDENCE: Level 4. TYPE OF STUDY: Cross-Sectional Survey Study.

Reoperative Anorectal Procedures in Patients with Anorectal Malformations - Is Bladder Function Affected?

BACKGROUND: Anorectal malformations (ARM) are associated with neurogenic bladder. The traditional surgical ARM repair is a posterior sagittal anorectoplasty (PSARP), which is believed to have a minimal effect on bladder dynamics. However, little is known about the effects of reoperative PSARP (rPSARP) on bladder function. We hypothesized that a high rate of bladder dysfunction existed in this cohort. METHODS: We performed a retrospective review of ARM patients undergoing rPSARP at a single institution from 2008 to 2015. Only patients with Urology follow-up were included in our analysis. Data was collected regarding original level of ARM, coexisting spinal anomalies and indications for reoperation. We assessed urodynamic variables and bladder management (voiding, CIC or diverted) before and after rPSARP. RESULTS: A total of 172 patients were identified, of which 85 met inclusion criteria with a median follow-up of 23.9 months (IQR, 5.9-43.8 months). Thirty-six patients had spinal cord anomalies. Indications for rPSARP included mislocation (n = 42), posterior urethral diverticulum (PUD; n = 16), stricture (n = 19) and rectal prolapse (n = 8). Within 1 year following rPSARP, 11 patients (12.9%) had a negative change in bladder management, defined as need for beginning intermittent catheterization or undergoing urinary diversion, which increased to 16 patients (18.8%) at last follow-up. Postoperative bladder management changed in rPSARP patients with mislocation (p < 0.0001) and stricture (p 0.005) but not for rectal prolapse (p 0.143). CONCLUSIONS: Patients who undergo rPSARP warrant especially close attention for bladder dysfunction as we observed a negative postoperative change in bladder management in 18.8% of our series. LEVEL OF EVIDENCE: Level IV.

A survey of healthcare professionals' perceptions of the decisional needs of parents with an infant born with a disorder/difference of sex development.

INTRODUCTION: Parents of infants born with differences in sex development (DSD) face many difficult decisions. As part of a larger project designed to develop educational interventions to promote shared decision making, this study assessed healthcare professionals' (HCPs) perceptions of parental decision-making needs when an infant is born with a DSD. METHODS: A cross-sectional web-based survey following the Ottawa Decision Support Framework was conducted in two waves, between October 2020 and June 2022. Survey domains included: common DSD decisions, indicators of parents' decisional needs, and resources and approaches to support parental decision making. Eligible participants were HCPs working within interprofessional pediatric DSD centers in the USA. Up to three reminders were sent. Descriptive analysis was conducted. RESULTS: 71 HCPs participated; most (>90%) reported parents experience signs of decisional conflict including feeling unsure, worrying about what could go wrong, and fear of choosing a "wrong," irreversible option. The majority (90%) reported parents experience strong emotions interfering with their receptivity to information or deliberation. The majority (>70%) identified inadequate parental knowledge of the DSD as a barrier to decision making, coupled with information overload (>90%). HCPs rated several factors as "very" important, including: parents having information on benefits, harms, and other features of options (93%), having information about all the available options (87%), and having access to providers to discuss the options (84%). Providers endorsed using a variety of approaches to support parents' decision making; however, access to decision aids was not universally rated as highly important (very, 44%; somewhat, 46%; a little, 10%). IMPLICATIONS: Overall, HCPs expressed favorable attitudes toward supporting active parental participation in medical decision making. Opportunities for enhanced support of shared decision making included: a) recognizing and addressing parental emotional distress and informational overload at a time when parents need to consider complex options for their infant or young child; and b) the need for HCPs to encourage values clarification in decision-making encounters with parents.

Outpatient Robotic Surgery in Pediatric Urology: Assessment of Feasibility and Short-Term Safety.

PURPOSE: Robotic reconstructive and extirpative procedures have been widely utilized for surgical management of various conditions in pediatric urology. Outpatient laparoscopic surgery has become the standard in cases of orchidopexy, inguinal hernia repair and varicocelectomy. There is a growing body of evidence that robotic surgery for more complex conditions can also be performed in an outpatient setting. The aim of the study was to assess the short-term safety and feasibility of robotic reconstructive and extirpative procedures for various pediatric urological conditions as scheduled outpatient procedures. MATERIALS AND METHODS: Demographic information and perioperative data were prospectively collected in an institutional database for all pediatric patients undergoing outpatient robotic surgery from June 2012 to December 2019. Primary outcomes included rates of 30-day complications, emergency room visits and readmissions. RESULTS: A total of 135 pediatric patients underwent robotic procedures in an outpatient setting. The majority underwent pyeloplasty (62) or extravesical ureteral reimplantation (55). Ten patients underwent ureteroureterostomy and 8 patients underwent extirpative procedures (nephrectomy, hemi-nephrectomy). Median age at surgery was 62 months (IQR, 27-99), median weight was 20 kg (IQR, 12-30) and median body mass index was 17 (IQR, 15-18). During the 30-day followup period there were 9 complications (6.7%), of which only 1 (0.7%) was high grade (Clavien-Dindo 3). There were 9 emergency room visits (6.7%) including 5 cases of readmission (3.7%). CONCLUSIONS: Robotic reconstructive and extirpative procedures in pediatric urology can be safely performed as scheduled outpatient procedures in the majority of patients, obviating the need for routine inpatient care.

Diagnosis, management and recurrence rates of Mitrofanoff polyps.

BACKGROUND: Intraluminal polyps are a known complication following creation of a urinary continent catheterizable channel (Mitrofanoff). These polyps can lead to difficult catheterizations in addition to symptomatic bleeding. However, there is limited data available regarding management and outcomes of these polyps. We aim to describe clinical presentation and management of a large series of polyps occurring in a Mitrofanoff channel. METHODS: We performed a retrospective review of all patients that were treated for polyps in a Mitrofanoff at our institution. Information was collected regarding presenting symptoms, management and recurrence rates of the polyps. RESULTS: A total of 24 patients were identified that fulfilled inclusion criteria. The majority of these polyps developed in channels composed of appendix (87%), while only 3 patients (13%) had polyps develop in an ileal composed channel. Thirteen (54%) of these polyps were incidentally diagnosed while 11 patients presented with a variety of symptoms such as difficulty in catheterization, bleeding with catheterization or both difficulty catheterizing and bleeding. For management of the polyps, a cystoscopy was performed and snaring the polyp with stone basket was performed in 37%, energy was applied to base to remove polyps in 33%, 16% were fulgurated and only 13% were left in situ. All procedures were performed under general anesthesia and all of the pathology was benign showing chronic inflammatory tissue. Eight polyps (33%) recurred after initial treatment. DISCUSSION: We did not observe an asymptomatic channel polyp convert to a symptomatic during our follow up period. Our experience has led us to not intervene on all asymptomatic Mitrofanoff polyps encountered during cystoscopy under assumption they will inevitably become symptomatic. Although we admit our follow up period may not be long enough to make this a universal declaration of best practice when any Mitrofanoff polyp is diagnosed. Endoscopic treatment was effective minimally invasive method to address the symptomatic polyp rather than excision and construction of new channel. CONCLUSIONS: This is the largest series to date of polyps developing in urinary continent catheterizable channels. The majority of these polyps were encountered incidentally however symptomatic polyps presented with difficulty with catheterizations. Symptomatic polyps can be managed endoscopically but recurrence of the polyp can occur.

Comparison of risk of anticholinergic utilization for treatment of neurogenic bladder between in utero or postnatal myelomeningocele repair.

INTRODUCTION: In utero myelomeningocele (MMC) repair has resulted in significant decrease in need for shunt-dependent hydrocephalus, however its impact on bladder function remains less clear. Neurogenic detrusor overactivity (NDO) due to MMC can be addressed with combination of clean intermittent catheterization (CIC) and anticholinergic (AC) therapy to minimize its effect on bladder function and upper urinary tract. The aim of this study was to compare the risk of postnatal AC utilization for in patients that underwent either postnatal or in utero MMC repair related to neurogenic bladder (NGB) management. We hypothesized that postnatal MMC repair would be associated with increased risk of postnatal AC utilization compared to in utero MMC repair. MATERIAL AND METHODS: All newborns with MMC in our hospital undergo prospective surveillance radiographic and urodynamic testing as part of institutional MMC protocol. Those MMC patients born between 2013 and 2018 at our institution, who underwent in utero or postnatal MMC repair were retrospectively analyzed. We identified postnatal AC utilization from electronic medical records and recorded indications for AC therapy according to the urodynamic, radiographic and clinical findings related to NGB management. RESULTS: 97 patients fulfilled the inclusion criteria. 56 patients underwent a postnatal and 41 an in utero repair. Median follow-up for the in utero and postnatal MMC repair groups was the same at 37 months (p = 0.53). More newborns from the postnatal group were discharged from birth hospital performing CIC (91.1% vs. 58.5%, p < 0.0001), however upon last follow up no difference existed between the groups on use of CIC (83.9% postnatal group % vs. 82.9% in utero group, p = 0.78). At last follow up, postnatal AC utilization was observed in 75% of postnatal MMC group compared to 78.1% of the in utero MMC repair group, p = 0.81 (Figure). The median time to AC utilization was 6.9 months and 8.8 months in the in utero and postnatal groups, respectively (p = 0.28). DISCUSSION: We observed no reduction in risk of AC utilization with in utero repair which refuted the hypothesis of our study. Indications for AC utilization were urodynamic abnormalities such as NDO (81.3% in utero vs. 81% postnatal) or impaired bladder compliance (53.1% in utero vs. 64.3% postnatal). CONCLUSIONS: We found no difference in risk of postnatal AC utilization between in utero or postnatal MMC repair. CIC rates were higher at birth hospital discharge in the postnatal repair group, however at last follow up CIC rates did not differ between groups.

Post-operative complications following masculinizing genitoplasty in moderate to severe genital atypia: results from a multicenter, observational prospective cohort study.

INTRODUCTION: Differences of sex development (DSD) are congenital conditions in which there is atypical chromosomal, gonadal and/or phenotypic sex. A phenotype of severe genital atypia in patients raised as male is a relatively rare occurrence and standards for management are lacking. Decision making for early surgical planning in these rare cases includes, but is not limited to, degree of atypia, location of testes, and presence of Mϋllerian remnants. In this study we describe surgical approaches and short-term outcomes for masculinizing genitoplasty in moderate to severe genital atypia in young patients raised male, for whom parents opted for early surgery. METHODS: This NIH-sponsored study is an ongoing, observational, multicenter investigation assessing medical, surgical and psychological outcomes in children and their parents affected by atypical genitalia due to DSD. Participants were prospectively enrolled from 12 children's hospitals across the United States that specialize in DSD care. Criteria for child enrollment were a Quigley score of 3-6 in those with a 46, XY or 45,X/46, XY chromosome complement, age <3 years with no previous genitoplasty; patients were included independent of whether genitoplasty was performed. Cosmesis was graded according to a 4-point Likert scale and complications per the Clavian-Dindo classification. RESULTS: Of the 31 participants, 30 underwent hypospadias repair and 1 patient did not undergo a genitoplasty procedure. The majority of participants (22) received a staged hypospadias repair. Seventeen complications were identified in 12 of the 31 children (41%) at 12 months of follow up. Glans dehiscence and urethrocutaneous fistula were the most common complications. Orchiopexy was performed in 14 (44%) and streak gonads were removed in 4 (13%) participants. Both parents and surgeons reported improved cosmesis after surgery when compared to baseline. CONCLUSION: Genitoplasty was chosen by parents for the majority of children eligible for study. No single surgical approach for masculinizing moderate to severe genital ambiguity in young patients with 46, XY or 45,X/46, XY DSD was adopted by all surgeons. Complications occurred in 41% of those who underwent genitoplasty for severe hypospadias. Overall, appearance of the genitals, as determined by parents and surgeons, improved following genitoplasty. Outcomes of early genitoplasty are needed to guide families when making decisions about such procedures for their young children.

Outcomes according to channel type for continent catheterizable channels in patients undergoing simultaneous urinary and fecal reconstruction.

INTRODUCTION: When creating a continent catheterizable channel (CCC) the choice of bowel segment used as the conduit should be tempered with the morbidity associated with it. The split-appendix technique allows the creation of both a urinary and fecal CCC without the need for a bowel anastomosis. However, there is concern that by splitting the appendix there is compromise to its blood supply and may affect outcomes. We aim to compare what affect the bowel segment used for urinary and fecal CCCs has on perioperative and long-term outcomes in patients undergoing simultaneous urinary and fecal reconstruction. METHODS: A retrospective review was performed analyzing all patients that underwent simultaneous continent catheterizable urinary and fecal CCC between the years 2010-2016. Patient demographics, channel characteristics, perioperative complications and clinical success rate were analyzed. RESULTS: A total of 106 patients were identified that had simultaneous fecal and urinary CCC created at time of reconstruction. For urinary CCC, there were 64 patients (60.4%) that underwent a split-appendix technique, 27 patients (25.4%) underwent a Monti, and 15 patients (14.2%) had the appendix used only for the urinary channel. Those patients undergoing a split-appendix technique had median operative time of 447 min compared to 619 min when a Monti channel was created. The median length of hospital stay was 9 days for the split-appendix technique compared to 12 and 13 day median hospital stay when the appendix was used only for the urinary channel or a Monti was created, respectively. There was no difference seen in revision free survival of the channel following surgery of any of the channels with median follow-up of 44.5 months. However, there were more subfascial revisions of urinary CCC in those that underwent a Monti (5 patients, 18.5%) compared to other bowel segments (0 patients). CONCLUSIONS: Utilizing a split-appendix approach for creation of urinary and fecal CCCs does not affect 30-day complications or long-term revision rates compared to other established techniques. This technique minimizes the potential surgical morbidity of a bowel anastomosis and provide shorter operative times, when feasible, at time of simultaneous creation of fecal and urinary CCC.

Design and development of the Pediatric Urology Recovery After Surgery Endeavor (PURSUE) multicentre pilot and exploratory study.

INTRODUCTION: Lower urinary tract reconstruction in paediatric urology represents a physiologically stressful event that is associated with high complication rates, including readmissions and emergency room visits. Enhanced recovery after surgery (ERAS) protocol is a set of multidisciplinary, perioperative strategies designed to expedite surgical recovery without adversely impacting readmission or reoperation rates. Early paediatric urology data demonstrated ERAS reduced complications in this population. METHODS AND ANALYSIS: In 2016, a working group of paediatric urologists and anaesthesiologists convened to develop an ERAS protocol suitable for patients undergoing lower urinary tract reconstruction and define study process measures, patient-reported outcomes and clinically relevant outcomes in paediatric and adolescent/young adult patients. A multicentre, prospective, propensity-matched, case-control study design was chosen. Each centre will enrol five pilot patients to verify implementation. Subsequent enrolled patients will be propensity matched to historical controls. Eligible patients must be aged 4-25 years and undergoing planned operations (bladder augmentation, continent ileovesicostomy or appendicovesicostomy, or urinary diversion). 64 ERAS patients and 128 controls will be needed to detect a decrease in mean length of stay by 2 days. Pilot phase outcomes include attainment of ≥70% mean protocol adherence per patient and reasons for protocol deviations. Exploratory phase primary outcome is ERAS protocol adherence, with secondary outcomes including length of stay, readmissions, reoperations, emergency room visits, 90-day complications, pain scores, opioid usage and differences in Quality of Recovery 9 scores. ETHICS AND DISSEMINATION: This study has been registered with authors' respective institution review boards and will be published in peer-reviewed journals. It will provide robust insight into the feasibility of ERAS in paediatric urology, determine patient outcomes and allow for iteration of ERAS implementations as new best practices and evidence for paediatric surgical care arise. We anticipate this study will take 4 years to fully accrue with completed follow-up. TRIAL REGISTRATION NUMBER: NCT03245242; Pre-results.

Post-operative complications following feminizing genitoplasty in moderate to severe genital atypia: Results from a multicenter, observational prospective cohort study.

Disorders/differences of sex development (DSD) are congenital conditions in which there is atypical chromosomal, gonadal and/or phenotypic sex. While there remains controversy around the traditionally binary concept of sex, most patients with DSD are reared either male or female depending on their genetic sex, gonadal sex, genital phenotype and status of their internal genital tract. This study uses prospective data from 12 institutions across the United States that specialize in DSD care. We focused on patients raised female. Eligible patients had moderate to severe genital atypia (defined as Prader score >2), were ≤2 years of age at entry, and had no prior genitoplasty. The aim of this study is to describe early post operative complications for young patients undergoing modern approaches to feminizing genitoplasty. Of the 91 participants in the cohort, 57 (62%) were reared female. The majority had congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (n = 52), 1 had ovo-testicular syndrome, 2 had mixed gonadal dysgenesis and 2 had partial androgen insensitivity syndrome (PAIS). Of the 50 participants who received early genitoplasty, 43 (86%) had follow-up at 6-12 months post-surgery. Thirty-two participants (64%) received a clitoroplasty, 31 (62%) partial urogenital mobilization and 4 (8%) total urogenital sinus mobilization. Eighteen percent (9/50) experienced post-surgical complications with 7 (14%) being rated as Clavien-Dindo grade III. Both parents and surgeons reported improved satisfaction with genital appearance of participants following surgery compared to baseline. This information on post-operative complications associated with contemporary approaches to feminizing genitoplasty performed in young children will help guide families when making decisions about whether or not to proceed with surgery for female patients with moderate to severe genital atypia.