Blake W. Palmer

Regional variations in small intestinal submucosa evoke differences in inflammation with subsequent impact on tissue regeneration in the rat bladder augmentation model.

To examine the histological differences in the inflammatory response and regenerative outcomes of distal vs proximal porcine small intestinal submucosa (SIS) grafts in the rat bladder, as SIS from distal small intestine yields reliable and reproducible bladder regeneration, while SIS from proximal portions of small intestine does not provide similar results.

Total and Partial Urogenital Mobilization: Focus on Urinary Continence

PURPOSE Total and partial urogenital mobilization procedures are the most common contemporary vaginoplasty surgeries for patients with congenital adrenal hyperplasia, urogenital sinus and cloacal anomalies. There is controversy regarding the urinary continence outcomes of these procedures. We reviewed the urinary continence outcomes of children who underwent total or partial urogenital mobilization at our institution and reviewed the literature to determine the continence rates of these procedures. MATERIALS AND METHODS We retrospectively reviewed 25 patients who underwent total or partial urogenital mobilization with a focus on postoperative continence status. Continence was defined as parental report of full toilet training with no accidents during the day and rare accidents (fewer than 2 per month) at night after age 3 years. RESULTS A total of 14 congenital adrenal hyperplasia, 5 urogenital sinus and 6 cloacal anomaly cases were managed by total (18) or partial (7) urogenital mobilization procedures with a mean followup of 4.41 years (range 0.21 to 12.1). In our cohort 21 of 22 patients (95.5%) were continent by age 3 years and there were no urinary complications. A total of 111 patients were identified in the literature with congenital adrenal hyperplasia or urogenital sinus, with 107 in 7 studies being continent (96.4%) by age 3 to 4 years. In 4 studies 32 patients were identified with cloacal anomalies who underwent total or partial urogenital mobilization, of whom 28 (87.5%) were continent by age 3 to 4 years. CONCLUSIONS There was no significant difference between total and partial urogenital mobilization procedures regarding postoperative urinary continence in our cohort and the literature. The urinary continence rate was 96% in the congenital adrenal hyperplasia/urogenital sinus group and 89.5% in the cloacal group.

A model of delivering multi-disciplinary care to people with 46 XY DSD

In 2006, a consensus statement was jointly produced by the Lawson Wilkins Pediatric Endocrine Society (LWPES) and the European Society of Paediatric Endocrinology (ESPE) concerning the management of disorders of sex development (DSD) [1]. A recommendation provided by this consensus was that evaluation and long-term care for people affected by DSD should be performed at medical centers with multi-disciplinary teams experienced in such conditions. Here we provide our teams interpretation of the 2006 consensus statement recommendations and its translation into a clinical protocol for individuals affected by 46 XY DSD with either female, or ambiguous, genitalia at birth. Options for medical and surgical management, transitioning of care, and the use of mental health services and peer support groups are discussed. Finally, we provide preliminary data to support the application of our model for delivering multi-disciplinary care and support to patients and their families.

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.

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.

Voiding cystourethrogram--are our protocols the same?

PURPOSE Voiding cystourethrogram is the gold standard for evaluating and diagnosing vesicoureteral reflux. Reflux detection can potentially be affected by many parameters during voiding cystourethrogram. MATERIALS AND METHODS A 29-item survey was sent via e-mail through SurveyMonkey® to the chairperson of pediatric radiology at 65 national pediatric hospitals. This survey included questions on institutional protocols for performing voiding cystourethrogram. RESULTS Responses were received from 41 institutions from across North America, including 17 of 19 Randomized Intervention for Children with Vesicoureteral Reflux study sites. Many aspects of the reports of voiding cystourethrogram protocols were similar with 90% or greater agreement in allowing parents in the room, contrast infusion by gravity, catheter or feeding tube use without balloons, no contrast dilution and voiding without a catheter in place. The height at which contrast medium was raised for infusion was 40, 60, 80, 100 and greater than 100 cm at 2.4%, 17.1%, 17.1%, 39.0% and 12.2% of sites, respectively, while the height was not measured or it varied at 12.2%. The infilling phase stopped when the bladder appeared full at 2.4% of sites, infusion stopped itself at 12.2%, patient voided at 61.0%, volume attained age expected capacity at 12.2%, the patient was uncomfortable at 4.9% and results varied at 7.3%. CONCLUSIONS Data reveal that voiding cystourethrogram is performed differently across North America and no standard protocol exists for the procedure. These differences could significantly impact voiding cystourethrogram results among institutions and taint our ability to compare results in the literature.

Mental health and parenting characteristics of caregivers of children with spina bifida

INTRODUCTION Within the chronic medical illness literature, associations exist between caring for an affected child and parent mental health. The few studies examining both mothers and fathers provide mixed results. The purpose of this study is to examine associations between caregiver anxiety, depression, and parenting variables in caregivers of youth with SB as these relate to marital status, age, education, household income, work status, and childs severity of SB. OBJECTIVE The aim of this study is to examine associations between anxiety, depression, and parenting variables in caregivers of youth with spina bifida and how they relate to demographic and disease variables. Exploratory analyses examined the relationship between participation in support activities and depressive and anxious symptomatology and parenting characteristics. STUDY DESIGN Eighty-four primary caregivers (49 mothers) of 51 youth with spina bifida completed measures of depressive and anxious symptomology, parenting stress, parent overprotection, and perceived child vulnerability. RESULTS There were differences between mothers and fathers on several parenting characteristics; however, these were related more to marital status and employment than to gender of the caretaker per se. In the 33 married/remarried couples for whom both spouses participated, stress for the mothers was correlated with stress for the fathers. This correlation was strongest in the 12 married couples in which the mother works. Higher perceived vulnerability scores were reported in parents of SB patients in the younger age group, especially preschoolers (0-4 years). Parents of children with shunts reported more anxiety, depression and perceived child vulnerability. Both male and female caregivers of younger children reported significantly higher protectiveness scores. Involvement in recreational activities with other families affected by SB was associated with more positive parenting characteristics for mothers. DISCUSSION Stress and protectiveness were found to be positively correlated (r > 0.6); depression, anxiety, and perceived vulnerability were not (0.3 < r < -0.3). Overall, mothers reported more stress and anxiety than fathers. Higher perceived vulnerability scores were reported in parents of SB patients in the younger age group, especially preschoolers (0-4 years). Parents of children with shunts reported more anxiety, depression and perceived child vulnerability. Both male and female caregivers of younger children reported significantly higher protectiveness scores compared to caregivers of older children. Involvement in recreational activities with other families affected by SB was associated with more positive parenting characteristics for mothers. There were differences between mothers and fathers on several parenting characteristics; however, these were related more to marital status and employment than to gender of the caretaker per se. Limitations to the current study qualify our results and conclusions. Associations do not prove causation. Our measure of parent protection had a lower Cronbachs alpha score for male caregivers (0.68) than female caregivers (0.83), consistent with an examination of the factor structure of the PPS that found the measure to have a poor factor structure and limited reliability in samples with a chronic medical condition. CONCLUSION Anxiety, depression, and parenting characteristics were differentially impacted by variables such as caregiver and child age, shunt status, and employment status/income for parents of youth with SB. Interventions to improve parenting skills and mental health of these caregivers can be designed to target specific needs of parents. Groups such as the Greater Oklahoma Disabled Sports Association (GODSA) offer real-world support to improve the lives of caregivers of SB children, and should be studied further to optimize outcomes for children.

Temporal differentiation and maturation of regenerated rat urothelium

To determine if porcine small intestinal submucosa (SIS)‐regenerated urothelium expresses markers of urothelial differentiation, uroplakin and zona occludens‐1 (ZO‐1), and whether their expression correlates with the histological appearance of the urothelium.

Multi-Institutional Review of Outcomes and Complications of Robot-Assisted Laparoscopic Extravesical Ureteral Reimplantation for Treatment of Primary Vesicoureteral Reflux in Children

Purpose: Robot‐assisted laparoscopic extravesical ureteral reimplantation has been proposed as a minimally invasive alternative to open ureteral reimplantation for correcting primary vesicoureteral reflux in children. However, in the current literature there are conflicting data regarding the safety and efficacy of this approach. Amid ongoing debate we analyzed outcomes and complications from this procedure in a large multi‐institutional cohort. Materials and Methods: We reviewed the records of children who underwent robot‐assisted laparoscopic extravesical ureteral reimplantation at 9 academic centers from 2005 to 2014. Radiographic failure was defined as persistent vesicoureteral reflux on postoperative voiding cystourethrogram or radionuclide cystogram. Complications were graded using the Clavien‐Dindo scale. Results: A total of 260 patients (363 ureters) underwent robot‐assisted laparoscopic extravesical ureteral reimplantation for primary vesicoureteral reflux during the study period. The cohort included 90 patients with either duplex ureter (42), failed endoscopic treatment (40) or concomitant diverticulectomy (8). Of the 280 ureters with postoperative voiding cystourethrogram or radionuclide cystogram available radiographic resolution was seen in 246 (87.9%). There were 25 complications overall (9.6%), with 7 grade 3 complications (2.7%) and no grade 4 or 5 complications. Four patients (3.9%) had transient urinary retention following bilateral reimplantation. Conclusions: Robot‐assisted laparoscopic extravesical ureteral reimplantation has a low complication rate consistent with published series of open ureteral reimplantation. Radiographic success rates are approaching those of the open procedure but continue to fall short of the gold standard approach. These findings necessitate critical thinking regarding potential technique improvements and further prospective investigation into the efficacy of this procedure.

Update on Tissue Engineering in Pediatric Urology

Pediatric urology patients often present with congenital or acquired tissue and organ dysfunction that requires surgical reconstruction to recreate the normal genitourinary systems functions. The traditional methods have varying degrees of donor site morbidity or inherent side effects. Tissue engineering is a developing field that aims to replace or regenerate these dysfunctional tissues and organs with cells, biomaterials, or a combination thereof. A tremendous amount of work has been done to these ends in terms of preclinical work, and some clinical trials have resulted. This review highlights the status of these studies in pediatric urology for the use of tissue engineering and reconstruction of the corporal bodies, urethra, and bladder.