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.

Causes of death among people with myelomeningocele: A multi-institutional 47-year retrospective study.

PURPOSE: This study aimed to analyze organ system-based causes and non-organ system-based mechanisms of death (COD, MOD) in people with myelomeningocele (MMC), comparing urological to other COD. METHODS: A retrospective review was performed of 16 institutions in Canada/United States of non-random convenience sample of people with MMC (born > = 1972) using non-parametric statistics. RESULTS: Of 293 deaths (89% shunted hydrocephalus), 12% occurred in infancy, 35% in childhood, and 53% in adulthood (documented COD: 74%). For 261 shunted individuals, leading COD were neurological (21%) and pulmonary (17%), and leading MOD were infections (34%, including shunt infections: 4%) and non-infectious shunt malfunctions (14%). For 32 unshunted individuals, leading COD were pulmonary (34%) and cardiovascular (13%), and leading MOD were infections (38%) and non-infectious pulmonary (16%). COD and MOD varied by shunt status and age (p < = 0.04), not ambulation or birthyear (p > = 0.16). Urology-related deaths (urosepsis, renal failure, hematuria, bladder perforation/cancer: 10%) were more likely in females (p = 0.01), independent of age, shunt, or ambulatory status (p > = 0.40). COD/MOD were independent of bladder augmentation (p = >0.11). Unexplained deaths while asleep (4%) were independent of age, shunt status, and epilepsy (p >= 0.47). CONCLUSION: COD varied by shunt status. Leading MOD were infectious. Urology-related deaths (10%) were independent of shunt status; 26% of COD were unknown. Life-long multidisciplinary care and accurate mortality documentation are needed.

9-Year Evaluation of a Transitional Care Program for Congenital Neurogenic Bladder Patients.

OBJECTIVE: To evaluate short and long-term adherence and nonadherence patterns within a staged transition clinic developed in 2014 to assist congenital neurogenic bladder patients transfer from pediatric to adult urologic care. METHODS: We performed a retrospective chart review of all congenital neurogenic bladder patients who had been prospectively identified in the transition clinic and adult urology clinic since 2014. Demographic and clinical data were collected and analyzed to determine patient adherence with transfer of care, to study long-term compliance with follow-up, and to identify factors that positively and negatively influenced transition clinic success. RESULTS: 157 patients participated in the transition clinic. 76/79 (96.2%) patients ready to transition successfully transferred to adult care. 55/76 (72.4%) remain active. Greater number of visits between transition stages was significant among patients who maintained active follow-up after transition (P = .006). 171 patients initiated care in our adult urology clinic without transition. Irrespective of nontransitioned patients longer follow-up, formally transitioned adult patients had higher yearly participation rates, with 55 (72.4%) transitioned patients compared to 63 (36.8%) nontransitioned patients remaining active (P < .001). CONCLUSION: Congenital neurogenic bladder patients who participated in a transition clinic were successful transferring from pediatric to adult care, with improved clinic adherence in adulthood, compared to patients who initiated adult care without structured transition. Early introduction to adult providers and increased number of visits positively impacted adherence upon transition to adult care. Noncompliance with pediatric provider visits negatively impacted transition adherence.

Referrals for Cryptorchidism: Analyzing for Quality from a Pre-COVID Dataset.

OBJECTIVE: To identify risk factors associated with an inaccurate diagnosis, delayed referral, and/or inappropriate ultrasound in consecutive referrals for an undescended testicle (UDT). METHODS: This study was approved by the institutional review board. Data was prospectively collected for all children referred to University of Oklahoma's pediatric urology service from August 2019 to February 2020. The data was imported into SAS vs 9.4 for analysis. We categorized the "quality" of referral by a point system using 3 factors (no previous ultrasound, ≤18 months, correct location). An optimal referral had an accurate testicular examination, was referred by 18 months of age, and no diagnostic ultrasound. RESULTS: We collected 75 referrals. Thirty-nine (52.0%) had confirmed UDT, requiring surgery. Twenty-seven (69.2%) had an accurate initial examination. Forty-one (54.7%) were outside the optimal age. Thirty-four were considered low quality (0-1 qualities) and 41 were high quality (2-3 qualities). Seven (9.3%) were optimal (3/3 qualities). Referrals that needed intervention were statistically more likely to be higher quality. CONCLUSION: Increased quality of UDT referrals directly correlated with the likelihood for treatment. This study reveals a need for more education on UDT referral in the community. By improving referring provider knowledge and skills, we can minimize unnecessary referrals.

Supernumerary Testis Discovered Upon Workup of a Paratesticular Mass.

A supernumerary testis is a rare finding with limited description in the literature. Presentations of supernumerary testes vary and often involve initial discovery intraoperatively or in association with a separate condition. In this case report, we describe our experience of a patient with ultrasound imaging concerning for a left paratesticular mass. The patient was taken to the operating room where the mass was found to be an atrophic, supernumerary testis. We also address the specific anatomy encountered in our case and discuss both the intraoperative and post-operative management that resulted, in addition to reviewing relevant literature.

Pediatric Robotic Adrenalectomy for Virilizing Adrenal Tumor in a 4-year-old Female.

Adrenocortical tumors in children are rare, with approximately 90% of children presenting with endocrine syndromes, most commonly virilization. The standard treatment remains an open resection, although there is increasing use of the minimally invasive approach. With the advent of robotic surgery, its advantages over open and conventional laparoscopy make it a viable option in select patients. Despite the adoption of robot-assisted surgery in pediatric urology, limited literature regarding robotic pediatric adrenal surgery exists, with only a few small case series. We present a female child with virilizing features due to a large functional adrenal tumor managed by robot-assisted adrenalectomy.

Urologic guidelines for the care and management of people with spina bifida.

PURPOSE: The life expectancy for people with spina bifida has increased, thus resulting in greater need for guidelines in urologic care in order to protect normal renal function, to develop strategies for urinary continence, and to advance independence through adult years. METHODS: The English literature was assessed from 2002-2015; greater than 300 publications identified. Case reports and opinion pieces were eliminated leaving 100 for in depth review. Clinical questions were then established for each age group that allowed for focused assessment. RESULTS: There was no Level 1 evidence for any of the defined clinical questions. This resulted in group consensus for all questions throughout all age groups. Guidelines were provided for identifying a symptomatic urinary infection, the role of urodynamic bladder testing and identification of bladder hostility, determining methods of renal function assessment and surveillance, the initiation of continence control, and transitioning to self-care through the teen and adult years. CONCLUSION: Urologic guidelines continue to be based on clinical consensus due to the lack of high level evidence-based research. Further research is required in all aspects of urologic management. While not the "Standard of Care," these guidelines should be considered "Best Practice".

A Rare Case of T-Cell Lymphoma Presenting With Bilateral Nephrolithiasis and Acute Renal Failure.

We present the rare case of a young boy who was found to have T-cell lymphoma after presenting with flank pain and bilateral nephrolithiasis. He initially underwent bilateral ureteral stent placement but returned with oliguria and acute renal failure. His subsequent workup revealed lymphoma involving both kidneys. He was started on chemotherapy for his lymphoma and dialysis for his renal failure. His stones ultimately dissolved with aggressive hydration and correction of serum uric acid levels. In this report, we discuss the identification and management of this rare condition.

Pediatric Ureteral Stent Placement to Manage Right Kidney Hemorrhage Following Coarctation Angioplasty.

This is the first case report of a catastrophic renal bleeding into the renal collecting system causing hemorrhagic shock in a child after a cardiac catheterization. In this case report, we discuss how we used nontraditional means of endourologic intervention in treating our hemodynamically unstable patient resulting in saving the patient's life and kidney.

Spina Bifida Health-care Guidelines for Men's Health.

Spina bifida has traditionally been regarded as a pediatric health issue with little regard to adult consequences of the disorder. The congenital neurologic and urologic anomalies, as well as sequelae of bladder management, can have a profound impact on adult male sexual function. Abnormalities in testicular descent, development, and function; fertility; penile sensation; erectile function; ejaculatory function; and orgasmic function are common. Prostate cancer has been diagnosed in men with spina bifida, but little data are available to guide screening, diagnosis, and treatment efforts. The Spina Bifida Association has supported development of guidelines for health care providers to address male health issues in individuals with spina bifida throughout their lives.

A cross sectional single institution study of quality of life in adult patients with spina bifida.

OBJECTIVE: To describe and compare differences in perception of independence, urinary continence, and quality of life in an adult spina bifida (SB) population. METHODS: We collected data on adult neurogenic bladder patients which included demographics, relevant procedures, and quality of life (QoL) questionnaires. QoL and functional outcomes were assessed using spinal cord independence measure (SCIM) and SF-8 health questionnaire. International consultation of incontinence questionnaire (ICIQ) was used to assess incontinence. Comparisons were drawn between patients who underwent surgical reconstruction and those who did not. Student t-tests were used for comparisons and a P-value <0.05 was considered statistically significant. RESULTS: Fifty-four patients with SB were included. A total of 43% underwent bladder augmentation (BA) and 30% underwent antegrade continence enema (ACE). Patients with BA scored 49 ± 25 on the SCIM survey while those without had higher scores of 68 ± 19 with a P-value of 0.016. This difference remained evident when patients with ACE were excluded. When comparing ICIQ and SF-8, no statistically significant differences were found between those who underwent surgical procedures and those who did not. CONCLUSIONS: Assessing QoL in congenital NGB patients is a complex task. In our cohort, patients who underwent BA and ACE were shown to have decreased SCIM scores. SCIM scores for BA patients were significantly higher in patients who did not receive a BA independent of ACE status. SF-8 and ICIQ scores did not show any statistically significant difference in quality of life survey scores in those who underwent procedures versus those who did not.

Analysis of Factors Associated with Patient or Caregiver Regret following Surgery for Fecal Incontinence.

PURPOSE: Malone antegrade continence enema has been a successful and widely used procedure for achieving fecal continence in children. We present data on the previously uninvestigated issue of patient and caregiver regret following surgery for intractable constipation and fecal incontinence. MATERIALS AND METHODS: We reviewed all patients undergoing antegrade continence enema or cecostomy creation at a single institution between 2006 and 2016. Patients and caregivers were assessed for decisional regret using the Decisional Regret Scale. Results were correlated with demographics, surgical outcomes and complications. RESULTS: A total of 81 responses (49 caregivers and 32 patients) were obtained. Mean followup was 49 months. Decisional regret was noted in 43 subjects (53%), including mild regret in 38 (47%) and moderate to severe regret in 5 (6%). No statistical difference in regret was noted based on gender, complications or performance of concomitant procedures. On regression analysis incontinence was strongly associated with decisional regret (OR 4.4, 95% CI 1.1-18.1, p <0.001) and regret increased as age at surgery increased, particularly when patients were operated on at age 13 to 15 years (OR 2.6, 95% CI 1.0-6.4 for age 13 years; OR 2.9, 95% CI 1.1-7.8 for age 14 years; OR 3.1, 95% CI 1.1-8.8 for age 15 years). CONCLUSIONS: This is the first known study describing decisional regret following surgery for fecal incontinence. Surgical factors aimed at achieving continence may be effective in decreasing postoperative regret. The finding of increased regret in teenage patients compared to younger children should be shared with families since it may impact the age at which surgery is pursued.

"Watering Can" Ureterocele Puncture Technique Leads to Decreased Rates of De Novo Vesicoureteral Reflux and Subsequent Surgery With Durable Results.

OBJECTIVE: To assess the outcomes of "watering can" ureterocele puncture (WCP), a technique previously associated with decreased incidence of de novo vesicoureteral reflux (VUR), as a durable option for management of ureteroceles and to determine the need for subsequent surgery for VUR following watering can puncture. MATERIALS AND METHODS: We retrospectively reviewed records of 55 consecutive endoscopic ureterocele procedures performed at our institution from 1999 to 2015. The WCP was performed using a holmium laser fiber to make 10-20 puncture holes through the ureterocele. Follow-up data were collected on infection, de novo VUR in the affected renal moiety and the need for further treatment and surgery. RESULTS: Of 55 patients who underwent endoscopic ureterocele management, 34 underwent WCP and 21 patients underwent either incision or puncture. Median follow-up was 3.4 and 2.8 years in the incision and puncture groups, respectively. Both groups had similar rates of ureterocele decompression (88% vs 90%; P >.05) and improvement in hydronephrosis (82% vs 81%; P >.05). The WCP group had a significantly decreased rate of de novo VUR (32% vs 67%; P <.05) and of subsequent surgery due to de novo VUR (38% vs 71%; P <.05). The average grade of de novo VUR was lower in the WCP group (1.4 vs 2.8; P <.05). CONCLUSION: Our study shows that the endoscopic WCP successfully decompresses the obstructing ureterocele and results in a decreased incidence of de novo VUR and ultimately in fewer invasive procedures for the patient. This update demonstrates the durable outcomes of this novel technique.

Psychological Adjustment of Parents of Children Born with Atypical Genitalia 1 Year after Genitoplasty.

PURPOSE: We examined the psychological adjustment of parents of children born with moderate to severe genital atypia 12 months after their child underwent genitoplasty. MATERIALS AND METHODS: Parents were recruited longitudinally from a multicenter collaboration of 10 pediatric hospitals with specialty care for children with disorders/differences of sex development and/or congenital adrenal hyperplasia. Parents completed measures of depressive and anxious symptoms, illness uncertainty, quality of life, posttraumatic stress and decisional regret. RESULTS: Compared to levels of distress at baseline (before genitoplasty) and 6 months after genitoplasty, data from 25 mothers and 20 fathers indicated significant improvements in all psychological distress variables. However, a subset of parents continued endorsing clinically relevant distress. Some level of decisional regret was endorsed by 28% of parents, although the specific decision that caused regret was not specified. CONCLUSIONS: Overall the majority of parents were coping well 1 year after their child underwent genitoplasty. Level of decisional regret was related to having a bachelor's level of education, increased levels of illness uncertainty preoperatively and persistent illness uncertainty at 12 months after genitoplasty but was unrelated to postoperative complications.

Penile Fracture Following a Fall in a 7-Year-Old Male.

We report a rare case of isolated penile fracture in a prepubescent male caused by an accidental fall. The patient presented with swelling and ecchymosis on the base of the penis and along the penile shaft. A flexible cystoscopy was performed, which did not reveal any concomitant urethral injury. The penis was degloved and the corporal tear was closed with absorbable suture. Due to the location of the injury, ventral mobilization of the urethra was required. The pathophysiology of penile fracture as well as the diagnosis and management of this rare injury are discussed in this report.

Changes in levels of parental distress after their child with atypical genitalia undergoes genitoplasty.

BACKGROUND: The birth of a child with a disorder of sex development (DSD) and atypical genitalia can be traumatizing and isolating for families. Parents of children with DSD are at risk for increased levels of psychological distress, including depression, anxiety, illness uncertainty (IU), post-traumatic stress symptoms (PTSS), and impairments in quality of life (QOL). Our previous report indicated that although the majority of parents of children with atypical genitalia were coping well prior to the child's genitoplasty, approximately 25% of them reported experiencing some type of psychological distress. OBJECTIVE: The current study sought to examine the trajectory of parental psychological distress prior to, and 6 months after their child underwent genitoplasty. METHODS: Parents were recruited as part of an ongoing, prospective, multi-site study involving 10 pediatric hospitals with specialized care for children with atypical genitalia. Results from 49 parents (27 mothers, 22 fathers) of 28 children (17 female sex of rearing, 11 male sex of rearing) born with atypical genitalia (Prader rating of 3-5 in 46,XX DSD or by a Quigley rating of 3-6 in 46,XY DSD or 45,XO/46,XY) were included in the study. RESULTS: There were no significant changes in level of depressive or anxious symptoms or quality of life between baseline and 6-month post-operative follow-up, although mothers continued to report significantly higher levels of depressive symptoms than fathers, and as a group, these parents reported lower QOL than published norms. The level of PTSS significantly decreased for all parents, suggesting that parents may have come through the acute stress phase associated with their child's diagnosis. Finally, while there were no significant changes in IU over the time period, the level of IU for parents of boys actually increased, while parents of girls reported no change (Figure). CONCLUSION: Six months after their child has undergone genitoplasty, the majority of parents report minimal levels of psychological distress. However, a subset of these parents continue to experience significant distress related to their child's diagnosis. Specifically, parents of boys appear to be at increased risk for difficulties, which may be related to either the lack of clinical diagnosis for almost half of these children or the necessity of two-stage surgeries for the majority of them. We will continue collecting data on these families to better understand the trajectory of these adjustment variables.

A framework for transitioning patients from pediatric to adult health settings for patients with neurogenic bladder.

BACKGROUND: Adolescents with neurogenic bladder are a vulnerable population that severely lacks consistent transitional care from pediatric to adult urology settings. AIMS: Our practice determined that 100 patients with spina bifida and other neurogenic bladder conditions were not appropriately transferred to the adult setting once reaching adulthood. METHODS: We initiated a transitional program to establish a dedicated and formal process for adolescent patients to transition to adult urology. The REACH clinic implements a formalized staging framework to facilitate migration of adolescents and young adults to the adult health setting. A social worker was incorporated to act as a patient advocate, behavioral health consultant, and resource specialist. RESULTS: To date 45 patients have been enrolled in the transition program. We have identified and categorized according to the appropriate stage. The REACH clinic has appropriately outlined the goals and mission of the program and resources utilized are financially practical and feasible by conducting a monthly combined clinic. The program has been instrumental in improving tracking and monitoring of these patients through their transition period. CONCLUSION: Through the efforts of the pediatric and adult urology teams, the REACH program is a dedicated framework that provides structure for transition of the adolescent patient. The addition of a social worker has resulted in enriched rapport and will likely result in improved compliance. This program allows for surveillance and evaluation of patient outcome indicators in this patient population. We believe that early introduction and frequent encounters with the adult urologic team is crucial to successful transitions. Neurourol. Urodynam. 36:973-978, 2017. © 2016 Wiley Periodicals, Inc.

The Effect of Handcycle Ergometer Exercise on Glucose Tolerance in Ambulatory and Non-Ambulatory Adolescents.

PURPOSE: Whole body or leg exercise before a meal can increase insulin sensitivity, but it is unclear whether the same can occur with upper body exercise since a smaller muscle mass is activated. We measured the impact of a single session of handcycle exercise on glucose tolerance and insulin sensitivity. METHODS: Nonambulatory (Non-Amb) adolescents with spina bifida or cerebral palsy (4F/3M), or ambulatory peers (Control, 4F/7M) completed 2 glucose tolerance tests on separate days, preceded by either rest or a 35-min bout of moderate-to-vigorous intermittent handcycle exercise. RESULTS: The Non-Amb group had higher body fat (mean ± SD: 38 ± 12%, Control: 24 ± 9, p = .041) but similar VO2peak (17.7 ± 6.1 ml/kg/min, Control: 21.1 ± 7.9). Fasting glucose and insulin were normal for all participants. Compared with the rest trial, exercise resulted in a reduction in glucose area under the curve (11%, p = .008) without a significant group x trial interaction and no difference in the magnitude of change between groups. Insulin sensitivity was increased 16% (p = .028) by exercise in the Control group but was not significantly changed in the Non-Amb group. CONCLUSION: A single bout of handcycle exercise improves glucose tolerance in adolescents with and without mobility limitations and could therefore help maintain or improve metabolic health.

Establishing a standard protocol for the voiding cystourethrography.

The voiding cystourethrogram (VCUG) is a frequently performed test to diagnose a variety of urologic conditions, such as vesicoureteral reflux (VUR). The test results determine whether continued observation or an interventional procedure is indicated. VCUGs are ordered by many specialists and primary care providers, including pediatricians, family practitioners, nephrologists, hospitalists, emergency room physicians, and urologists. Current protocols for performing and interpreting a VCUG are based on the International Reflux Study in 1985. However, more recent information provided by many national and international institutions suggests a need to refine those recommendations. The lead author of the 1985 study, R.L. Lebowitz, agreed to and participated in the current protocol. In addition, a recent survey directed to the chairpersons of pediatric radiology of 65 children's hospitals throughout the United States and Canada showed that VCUG protocols vary substantially. Recent guidelines from the American Academy of Pediatrics (AAP) recommend a VCUG for children between 2 and 24 months of age with urinary tract infections but did not specify how this test should be performed. To improve patient safety and to standardize the data obtained when a VCUG is performed, the AAP Section on Radiology and the AAP Section on Urology initiated the current VCUG protocol to create a consensus on how to perform this test.