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.

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.

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.

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.

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.

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.

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.

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. 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 department 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.

Multi-institutional Study Comparing the Height of Contrast During Performance of Voiding Cystourethrogram in Children.

OBJECTIVE: To assess the effect of contrast height during the voiding cystourethrogram (VCUG). The VCUG is the gold standard diagnostic test for vesicoureteral reflux (VUR). Variation in parameters may affect detection and grade of reflux. MATERIALS AND METHODS: In a multicenter, prospective, nonrandomized, observational study, patients undergoing VCUG were selected. VCUG was performed per study protocol except for a change in contrast height. The initial fill was performed at 50 cm and the second at 100 cm. Data collected included presence and grade of VUR and volume filled. The actual bladder volume filled was normalized to the estimated bladder capacity (EBC) as a percentage. A Cohen's kappa coefficient of agreement was used to test for difference in the incidence of reflux and grade between contrast heights. A Wilcoxon signed-rank test was used for differences in the percent EBC filled between heights. RESULTS: From May 2012 to November 2013, 184 patients were enrolled. Seventy-one patients (39%) exhibited VUR at 50 cm and 80 patients (43%) at 100 cm. The kappa coefficient of agreement between 50 cm and 100 cm showed substantial agreement, with no significant difference in VUR grade. The percent of EBC filled at each height was significantly different: %EBC filled at 50 cm: 101 ± 46 (range 9.2-228.3), and %EBC filled at 100 cm: 130 ± 56 (range 37.8-280.6) (P < .0001). CONCLUSION: No significant difference was noted in the detection of VUR with different contrast heights. A significantly larger bladder volume was instilled at 100 cm.

Characterizing Early Psychosocial Functioning of Parents of Children with Moderate to Severe Genital Ambiguity due to Disorders of Sex Development.

PURPOSE: We examined the psychosocial characteristics of parents of children with disorders of sex development at early presentation to a disorders of sex development clinic. Parental anxiety, depression, quality of life, illness uncertainty and posttraumatic stress symptoms were assessed. Additionally we evaluated the relationship of assigned child gender to parental outcomes. MATERIALS AND METHODS: A total of 51 parents of children with ambiguous or atypical genitalia were recruited from 7 centers specializing in treatment of disorders of sex development. At initial assessment no child had undergone genitoplasty. Parents completed the Cosmetic Appearance Rating Scale, Beck Anxiety Inventory, Beck Depression Inventory, SF-36, Parent Perception of Uncertainty Scale and Impact of Event Scale-Revised. RESULTS: A large percentage of parents (54.5%) were dissatisfied with the genital appearance of their child, and a small but significant percentage reported symptoms of anxiety, depression, diminished quality of life, uncertainty and posttraumatic stress. Few gender differences emerged. CONCLUSIONS: Although many parents function well, a subset experience significant psychological distress around the time of diagnosis of a disorder of sex development in their child. Early screening to assess the need for psychosocial interventions is warranted.

Reliance of preoperative scrotal examination versus final operative findings in the evaluation of non-palpable testes.

OBJECTIVE: The current study evaluates whether the preoperative scrotal examination in children with non-palpable testes is sufficient in all cases or if laparoscopy is necessary to confirm the examination findings. Are the findings different amongst physicians depending on their level of experience? MATERIALS AND METHODS: This retrospective study was done between October 2009 and May 2013. Children with non-palpable testes underwent an examination under anesthesia prior to surgery to feel for a testis or nubbin, followed by a diagnostic laparoscopy to validate the scrotal examination. The test was considered correct if the laparoscopic findings supported the scrotal examination. It was considered incorrect if laparoscopy revealed a different finding. RESULTS: A total of 74 testes were evaluated. All patients underwent examination under anesthesia by the surgeon and senior resident. The findings were recorded separately. Despite careful examination by experienced surgeons under anesthesia, the scrotal examination was incorrect in 14 patients. Four viable testes would have been missed if laparoscopy had not been performed. CONCLUSION: Laparoscopy should be considered in case of palpable scrotal nubbins to ensure no testis is left within the abdominal cavity.

Temporal expression of hyaluronic acid and hyaluronic acid receptors in a porcine small intestinal submucosa-augmented rat bladder regeneration model.

INTRODUCTION: Hyaluronic acid (HA), a non-sulfated glycosaminoglycan, is an essential component of the extracellular matrix (ECM). Since HA is involved in many phases of wound healing and may play a key role in tissue repair and regeneration, this study was intended to understand temporal and spatial expression of HA and HA receptors (HARs) during the course of bladder regeneration in rats. MATERIALS AND METHODS: Sprague-Dawley rats were subjected to partial cystectomy followed by augmentation with porcine small intestinal submucosal (SIS) prepared from distal sections of the small intestine. SIS-augmented bladders were harvested between postoperative days 2 and 56. RESULTS: Bladder regeneration proceeded without complications. All augmented bladders had complete urothelial lining and smooth muscle bundles by day 56 post-augmentation. Temporal and spatial distributions of HA and HARs were studied by immunohistochemistry in regenerating bladders. The strongest HA immunoreactivity was observed in the ECM on postoperative days 28 and 56. Cluster of differentiation 44 (CD44) immunoreactivity was detected in the cytoplasm of urothelial cells on day 56; and LYVE-1 immunoreactivity was exclusively limited to lymphatic vessels on days 28 and 56. CONCLUSIONS: We demonstrated that HA was synthesized throughout the course of bladder wound healing and regeneration; and HA deposition coincided with urothelial differentiation. Expression of CD44 and LYVE-1 followed the same temporal pattern as HA deposition. Therapeutic modalities through local delivery of exogenous HA to improve the outcome of SIS-mediated bladder regeneration might need to be coordinated with HAR expression in order to achieve maximal regenerative responses as opposed to fibrosis.

Biomatrices for bladder reconstruction.

There is a demand for tissue engineering of the bladder needed by patients who experience a neurogenic bladder or idiopathic detrusor overactivity. To avoid complications from augmentation cystoplasty, the field of tissue engineering seeks optimal scaffolds for bladder reconstruction. Naturally derived biomaterials as well as synthetic and natural polymers have been explored as bladder substitutes. To improve regenerative properties, these biomaterials have been conjugated with functional molecules, combined with nanotechology, or seeded with exogenous cells. Although most studies reported complete and functional bladder regeneration in small-animal models, results from large-animal models and human clinical trials varied. For functional bladder regeneration, procedures for biomaterial fabrication, incorporation of biologically active agents, introduction of nanotechnology, and application of stem-cell technology need to be standardized. Advanced molecular and medical technologies such as next generation sequencing and magnetic resonance imaging can be introduced for mechanistic understanding and non-invasive monitoring of regeneration processes, respectively.

Understanding roles of porcine small intestinal submucosa in urinary bladder regeneration: identification of variable regenerative characteristics of small intestinal submucosa.

Neuropathic bladders are the result from damages to the central or peripheral nervous system, and ultimately may require surgical reconstruction to increase bladder volumes and to reduce the risk of damages to the kidneys. Surgical reconstruction through bladder augmentation has traditionally been practiced using a segment of the ileum, colon, or stomach from the patient through enterocystoplasty. However, the use of gastrointestinal segments can lead to serious adverse consequences. Porcine small intestinal submucosa (SIS), a xenogeneic, acellular, biocompatable, biodegradable, and collagen-based bioscaffold is best known to encourage bladder regeneration without ex vivo cell seeding before implantation in various experimental and preclinical animal models. Although it has been demonstrated that SIS supports bladder cell growth in vitro, and SIS-regenerated bladders are histologically and functionally indistinguishable from normal functional tissues, clinical utilization of SIS for bladder augmentation has been hampered by inconsistent preclinical results. Several variables in SIS, such as the age of pigs, the region of the small intestine, and method of sterilization, can have different physical properties, biochemical characteristics, inflammatory cell infiltration, and regenerative capacity due to cellular responses in vitro and in vivo. These parameters are particularly important for bladder regeneration due to its specific biological function in urine storage. Clinical application of SIS for surgical bladder reconstruction may require graft materials to be prepared from a specific region of the small intestine, or to be further formulated or processed to provide uniform physical and biochemical properties for consistent, complete, and functional bladder regeneration.