Adverse Birth Experiences and Parent Adjustment Associated With Atypical Genital Appearance Due to Differences of Sex Development.

OBJECTIVE: Differences/disorders of sex development (DSDs) are rare, congenital conditions involving discordance between chromosomes, gonads, and phenotypic sex and are often diagnosed in infancy. A key subset of parents of children newly diagnosed with a DSD experience clinically elevated distress. The present study examines the relationship between perinatal factors (i.e., gestational age, delivery method) and trajectories of parental adjustment. METHODS: Parent participants (mothers = 37; fathers = 27) completed measures at baseline, 6- and 12-month follow-up. Multilevel linear regression controlled for clustering of the data at three levels (i.e., time point, parent, and family) and examined the relationship between perinatal factors and trajectories of depressive and anxious symptoms. Two-way interactions between perinatal factors and parent type were evaluated. RESULTS: Overall depressive and anxious symptoms decreased over time. There were significant interactions between gestational age and parent type for depressive and anxious symptoms, with younger gestational age having a stronger negative effect on mothers vs. fathers. There was a significant interaction between time and gestational age for depressive symptoms, with 36 weeks' gestational age demonstrating a higher overall trajectory of depressive symptoms across time compared to 38 and 40 weeks. Findings for the delivery method were not significant. CONCLUSIONS: Findings uniquely demonstrated younger gestational age was associated with increased depressive symptoms, particularly for mothers compared to fathers. Thus, a more premature birth may predispose parents of infants with DSD to distress. Psychosocial providers should contextualize early diagnosis-related discussions within stressful birth experiences when providing support.

Stigma, Intrusiveness, and Distress in Parents of Children with a Disorder/Difference of Sex Development.

OBJECTIVE: The impact of parent-reported stigma due to their child's disorder/difference of sex development (DSD) on parent psychosocial adjustment is poorly understood. In other pediatric populations, perceived interference of medical conditions into daily activities (i.e., illness intrusiveness ) mediates the relationship of stigma to adjustment. This study assessed relationships between parent-focused and child-focused stigma → illness intrusiveness → depressive and anxious symptoms . Exploratory analyses sought to identify patient characteristics associated with stigma. METHOD: Caregivers (59 women and 43 men) of 63 children diagnosed with a DSD up to age 4 years completed measures of demographics, parent-focused and child-focused stigma, illness intrusiveness, and depressive and anxious symptoms. RESULTS: Increased parent-focused and child-focused stigma were associated with increased illness intrusiveness, which, in turn, was associated with increased depressive and anxious symptoms for parents nested within dyads. Among children with DSD family histories, parents reported greater child-focused stigma. CONCLUSION: Parents who experience DSD-related stigma report greater interference of their child's DSD into their daily activities, which is associated with poorer psychosocial adjustment. Findings support developing clinical interventions related to parents' perceptions of stigma and illness intrusiveness to improve parent adjustment.

Decisional regret about surgical and non-surgical issues after genitoplasty among caregivers of female infants with CAH.

INTRODUCTION: Caregivers of female infants with congenital adrenal hyperplasia (CAH) often confront complex medical decision-making (e.g., early feminizing genitoplasty). OBJECTIVE: This study aimed to evaluate the relevant medical decisions and subsequent decisional regret of caregivers following their child's genitoplasty. STUDY DESIGN: Caregivers (N = 55) were recruited from multidisciplinary treatment programs for participation in a longitudinal study. Qualitative data was collected at 6-12 months following feminizing genitoplasty to evaluate caregiver-reported decision points across their child's treatment. Quantitative exploratory analysis evaluated pre-operative predictors of subsequent decisional regret. DISCUSSION: When prompted about their decision-making and potential regret, most caregivers (n = 32, 80%) reported that their daughter's genital surgery was their primary medical decision. Specific themes regarding genital surgery included the timing and type of surgery. Most caregivers reported no decisional regret (62%), with 38% reporting some level of regret. Greater pre-operative illness uncertainty predicted heightened decisional regret at follow-up, p = .001. CONCLUSION: Two-thirds of caregivers of female infants with CAH reported not regretting their decision-making. Nevertheless, over one-third of caregivers reported some level of regret, suggesting the need for improvements in shared decision-making processes. Many, but not all, families reported that this regret was related to surgical decision-making. Reducing caregiver illness uncertainty (e.g., providing clear information to families) may increase their satisfaction with decision-making. Further research is needed to determine how the evolving care practices surrounding early genitoplasty will impact families.

Distress Trajectories for Parents of Children With DSD: A Growth Mixture Model.

OBJECTIVE: This study identifies trajectories of parent depressive symptoms after having a child born with genital atypia due to a disorder/difference of sex development (DSD) or congenital adrenal hyperplasia (CAH) and across the first year postgenitoplasty (for parents who opted for surgery) or postbaseline (for parents who elected against surgery for their child). Hypotheses for four trajectory classes were guided by parent distress patterns previously identified among other medical conditions. METHODS: Participants included 70 mothers and 50 fathers of 71 children diagnosed with a DSD or CAH with reported moderate to high genital atypia. Parents were recruited from 11 US DSD specialty clinics within 2 years of the child's birth and prior to genitoplasty. A growth mixture model (GMM) was conducted to identify classes of parent depressive symptoms over time. RESULTS: The best fitting model was a five-class linear GMM with freely estimated intercept variance. The classes identified were termed "Resilient," "Recovery," "Chronic," "Escalating," and "Elevated Partial Recovery." Four classes have previously been identified for other pediatric illnesses; however, a fifth class was also identified. The majority of parents were classified in the "Resilient" class (67.6%). CONCLUSIONS: This study provides new knowledge about the trajectories of depressive symptoms for parents of children with DSD. Future studies are needed to identify developmental, medical, or familial predictors of these trajectories.

Illness Uncertainty Longitudinally Predicts Distress Among Caregivers of Children Born With DSD.

OBJECTIVE: A subset of parents of children with disorders/differences of sex development (DSD) including ambiguous genitalia experience clinically elevated levels of anxious and depressive symptoms. Research indicates that uncertainty about their child's DSD is associated with parent psychosocial distress; however, previous studies have been cross-sectional or correlational in nature. The current study is the first to examine the longitudinal trajectory of the relationship between caregiver-perceived uncertainty about their child's DSD and caregiver anxious and depressive symptoms across the first 12 months following genital surgery in young children, or if surgery was not performed, the first 12 months following study entry. METHODS: One hundred and thirteen caregivers (Mage = 32.12; 57.5% mothers; 72.6% Caucasian) of children (N = 70; Mage = 9.81 months; 65.7% female) with DSD were recruited from 12 DSD specialty clinics in the United States. Caregivers completed psychosocial measures at baseline, 6 and 12 months following genitoplasty, or study entry if parents elected not to have surgery for their child. RESULTS: Caregiver illness uncertainty and both anxious and depressive symptoms were highest at baseline and decreased over time (ps < .05). Caregiver illness uncertainty predicted symptoms of anxious and depressive symptoms across all time points (ps < .05). CONCLUSIONS: Caregivers' perceptions of uncertainty about their child's DSD are highest soon after diagnosis, and uncertainty continues to predict both anxious and depressive symptoms across time. Thus, the initial diagnostic period is a critical time for psychological assessment and intervention, with parent illness uncertainty being an important clinical target.

Who, where, and why are patients lost to follow-up? A 20-year study of bladder exstrophy patients at a single institution.

INTRODUCTION: Individuals with bladder and cloacal exstrophy are at increased risk for kidney disease, renal failure, and bladder complications. Given the social implications and sensitive nature of the disease, these patients are also at risk for psychosocial problems. Lack of regular medical follow-up visits may pose serious risks to their long-term health status. The aim of this study is determine what factors place an affected individual at risk for limited long term follow up. MATERIALS AND METHODS: We identified all patients with bladder or cloacal exstrophy seen by the pediatric urology department at the Oklahoma University Health Sciences Center (OUHSC) between January 1996 and August 2016. Patient data included demographics, insurance coverage, distance from patient's home to the clinic, medical and surgical history, and the date of their last clinic visit. Two groups for comparison were (1) those that had been seen within the last 2 years, and (2) those that were counted as failing to maintain follow-up because 2 or more years had passed since their last clinic visit. These groups were compared using the Student t-test, the chi-square test, or the Fisher exact test and p < 0.05 was treated as significant for the purposes of discussion. RESULTS AND DISCUSSION: Ninety-one patients with bladder or cloacal exstrophy were seen by the pediatric urology department between January 1996 and August 2016. Of the 91 patients, 24 left the clinic for known reasons thus leaving 67 patients that were considered for analyses: 51 had been seen within the last 2 years while 16 were counted as lost to follow-up. These two groups (active and lost to follow-up) did not differ significantly for age at last clinic visit, distance between family's home and clinic, history of bladder reconstruction, sex, or insurance status. There was a significant difference between the two groups in the medical complexity of their condition. The group active in urological care had more patients with cloacal exstrophy and additional anomalies than the group lost to follow-up. CONCLUSIONS: Patients with bladder exstrophy and cloacal exstrophy are less likely to maintain annual follow-up visits with their urologist if they have a simpler diagnosis requiring fewer surgical interventions. For patients with exstrophy, regular clinic visits prioritizing education and psychosocial support may prevent hospitalizations, emergency interventions, and poor overall health outcomes. To maintain contact with the medical team and promote optimal health outcomes, a social worker or care coordinator/educator may play an integral part in addressing the unique needs of this population.

"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.

Testicular torsion: A retrospective investigation of predictors of surgical outcomes and of remaining controversies.

INTRODUCTION: Testicular torsion (TT), a common surgical emergency worldwide, is typically treated with orchiectomy or orchiopexy. It is widely accepted that the chance of salvaging the testicle declines with time and degree of torsion. The impact of ethnicity on outcome is less well understood, and the association between weather and onset of TT remains a controversy. OBJECTIVES: It is important to know the signs of TT so that appropriate treatment can be given quickly. The purpose of this study was to provide a detailed analysis of registered cases of TT in adolescent patients diagnosed at a single institution to better understand the association between clinical indicators and surgical outcomes and to examine some remaining controversies in the literature on TT. STUDY DESIGN: A retrospective chart review was conducted, using medical records from the present institution. Data were collected for 165 patients who met the following inclusion criteria: 1) adolescent males between 10 and 18 years of age at the time of diagnosis, and 2) TT between January 2001 and June 2013. RESULTS: Of the 165 patients, 38% had orchiectomies. Patients with orchiectomies had longer wait times for surgery (p < 0.0001)-but not greater driving times, driving distances, or degrees of torsion-than those with orchiopexies (Table). Yet, among patients who waited less than the median wait time to surgery (197 min), patients with orchiectomies had greater degrees of torsion than did those with orchiopexies (p = 0.02). Assuming that patients without reference to presence of bell clapper deformity in their medical notes did not have the deformity, those with orchiectomies were less likely to have bell clapper deformity than were those with orchiopexies (p < 0.01). Although mean atmospheric temperature was unassociated with onset of TT and with surgical outcome in general, patients without bell clapper deformity had TT on relatively colder days (p = 0.02). DISCUSSION AND CONCLUSION: Wait time to surgery positively correlates with orchiectomy. Early identification and intervention is vital to testicular salvage. As the degree of torsion increases, the blood supply to the affected testis decreases and the time required to inflict testicular vascular damage decreases. Our results showed the presence of the bell clapper deformity moderated the relationship between temperature and TT: Those without the deformity had torsions on colder days than did those with the deformity. A comprehensive multi-centered study could help draw further conclusions regarding temperature correlation and the bell clapper deformity.

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.

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 child's 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 Cronbach's 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 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.

Pediatric robot-assisted laparoscopic radical adrenalectomy and lymph-node dissection for neuroblastoma in a 15-month-old.

Neuroblastoma (NB) is the most common extra-cranial solid tumor in children and the most common malignancy in infants, with complete resection being curative in low-stage disease. The previous standard of treatment for many abdominal NBs involving the adrenal gland had been open surgery; however, there have been numerous descriptions of the safety and feasibility of a laparoscopic approach to resect adrenal masses in the pediatric population in benign and malignant disease, including improved cosmetic results, decreased length of stay, decreased surgical morbidity, and comparable oncological outcomes to open surgery. Despite these reported advantages over open surgery, the newer robot-assisted laparoscopy (RAL) offers benefits over the conventional laparoscopic approach that could further improve outcomes and expand the use of minimally invasive surgical approaches for pediatric adrenal masses. RAL offers many additional advantages over conventional laparoscopy, such as 3D visualization, increased range of motion of surgical instruments, tremor control, and a shorter learning curve compared with traditional laparoscopic surgery, while still maintaining the advantages of minimally invasive surgery. The body of literature concerning robot-assisted oncological surgery involving the adrenal gland in children is quite small, and to our knowledge no case reports have been published describing robot-assisted removal of an adrenal NB in a pediatric patient. We present our experience and technique of an RAL approach for lymph-node dissection and radical resection of a low-stage NB involving the adrenal gland with no image-defined risk factors in a 15-month-old infant.

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.

Proximal hypospadias repair outcomes in patients with a specific disorder of sexual development diagnosis.

Boys with undermasculinized external genital and/or 46,XY disorders of sex development (DSD) often receive masculinizing genitoplasty. Such procedures are done to correct ventral curvature of the phallus, reposition a proximally located urethral meatus, and cosmetically correct the appearance of labioscrotal folds. No studies to date have assessed if patients with a specific DSD diagnosis have worse outcomes for severe proximal hypospadias procedures or whether or not these patients require more extensive surgical maneuvers than severe proximal hypospadias patients without a specific DSD diagnosis. We retrospectively reviewed consecutive proximal hypospadias repairs performed at our institution from 1998 to 2010 and compared the anatomy, surgical technique required for repair, and outcomes in patients with and without a definitive DSD diagnosis. Boys with a specific DSD diagnosis do have significantly more atypical anatomy when undergoing proximal hypospadias masculinizing genitoplasties. They are more likely to require associated gonad procedures but do not have an increased risk of complications or number of surgeries when compared to other proximal hypospadias patients without a specific DSD diagnosis. The risk of complications is consistent with reports in the literature, and the mean number of procedures in this contemporary study is fewer than in historic reports.

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 team's 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.

Wilms' tumor metastatic to bilateral testes at presentation: case and review of the literature.

A 7-year-old male with bilateral palpable testicular masses was found to have metastatic stage IV Wilms' tumors associated with both left renal and lung lesions. The patient was treated successfully with testicular-sparing bilateral partial orchiectomies, radical nephrectomy, chemotherapy, and radiation, with 8 years free of recurrence. This is the only reported case of bilateral testicular tumors from metastatic Wilms' tumor that were present at the initial presentation. A review of the literature of all previous reported cases of metachronous or related testicular metastatic Wilms' tumors is reported.

Hand-assisted laparoscopic nephroureterectomy with cystoscopic en-bloc excision of the distal ureter and bladder cuff and isthmusectomy in a horseshoe kidney for invasive urothelial carcinoma of the renal pelvis.

Upper tract invasive urothelial carcinoma and horseshoe kidneys are familiar to the practicing urologist but relatively rare individual entities. The complication of managing them when they coexist in the same patient can be challenging. Herein, we present the first reported case in which an upper tract invasive urothelial carcinoma in a horseshoe kidney was successfully managed with a combined hand-assisted laparoscopic nephroureterectomy and isthmusectomy with cystoscopic en-bloc excision of the distal ureter and bladder cuff. This highlights the fact that complex anatomy can be managed in a completely minimally invasive fashion, and sound oncologic principles can still be maintained.

Comparison of endoscopic ureterocele decompression techniques. Preliminary experience--is the watering can puncture superior?

PURPOSE: The optimal endoscopic technique to manage an obstructing ureterocele would reliably relieve obstruction without creating de novo vesicoureteral reflux. The classic incision achieves decompression but invariably creates new vesicoureteral reflux. We compared a new endoscopic puncture technique to assess its superiority to endoscopic incision. MATERIALS AND METHODS: We retrospectively reviewed 27 consecutive endoscopic ureterocele procedures at our institution. Patients who underwent an endoscopic incision or watering can puncture procedure had their records reviewed for preoperative radiological and clinical data, operative description, and postoperative radiological and clinical outcomes. RESULTS: Of the 15 patients with endoscopic ureterocele incision 11 underwent a watering can puncture. All ureteroceles were associated with grade 3 or 4 hydronephrosis. Incision successfully decompressed the ureterocele in 14 of 16 cases (87.5%) and hydronephrosis in 14 (87.5%) on postoperative renal ultrasound. De novo reflux was detected in 12 of 16 patients (75.0%) on postoperative voiding cystourethrogram. Puncture successfully decompressed the ureterocele in 10 of 11 cases (90.9%) and hydronephrosis in 9 (81.8%). De novo vesicoureteral reflux was detected in 4 of 11 patients (36.4%). CONCLUSIONS: Our initial review shows that the endoscopic watering can procedure successfully decompresses the obstructing ureterocele and its associated hydronephrosis. This technique also results in a decreased incidence of de novo vesicoureteral reflux and ultimately in fewer invasive procedures for the patient.

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.