Zinner syndrome with contralateral hydronephrosis: A rare congenital condition.

Zinner Syndrome (ZS) is a rare congenital genitourinary abnormality defined by seminal vesicle cysts, ejaculatory duct obstruction, and unilateral renal dysplasia or agenesis. Patients can be asymptomatic, while others experience pain, urinary or ejaculatory symptoms and infertility. A patient that presented with painless gross hematuria was found to have a large pelvic cystic structure, an absent left kidney, multiple fluid collections in the region of the left seminal vesicle and right hydronephrosis. Hydronephrosis is atypical in ZS. This patient eventually developed right flank and pelvic pain treated with robotic-assisted laparoscopic excision of the pelvic cystic structure and extravesical ureteral reimplantation.

Clostridium difficile: What is the risk?

INTRODUCTION: Clostridium difficile infections (CDIs) are rising among pediatric patients in the community and hospital setting. Children undergoing transplants and bowel surgery are at a higher risk, while renal surgery has a lower risk. We hypothesize children undergoing pediatric urologic procedures are uncommonly diagnosed with postoperative CDI. OBJECTIVE: To study CDI in pediatric patients undergoing urologic surgery and identify associated perioperative factors. STUDY DESIGN: The American College of Surgeons National Surgical Quality Improvement Program Pediatric data file was queried for children undergoing surgery with pediatric urology or urology between 2015 and 2017. Data points included patient demographics (age, gender, race, ASA classification), surgery performed, and perioperative outcomes (operative time, admission status, length of stay, complications, readmission, and reoperation). Students T-test and Chi-square analyses were applied to detect differences between those with CDI and those without CDI. RESULTS: Of the 27,193 patients undergoing urologic surgery, 36 (0.13%) were diagnosed with CDI. The surgeries are presented in the Summary Figure. Patients with CDI were more likely to be female (50% vs 28%, p = 0.003) than those without. There was no difference in mean age or race. Children with CDI had higher ASA classifications (p < 0.001). Their mean operative times were longer (156.1 ± 19.6 vs 105.2 ± 0.6 min, p < 0.001), as were their mean lengths of stay (4.6 ± 0.8 vs 1.3 ± 0.0 days, p < 0.001). CDI patients were more likely to have other complications (29% vs 6%, p < 0.001). Among patients with CDI, 19.4% experienced concomitant infectious complications. There was no difference in reoperation rate, but more patients with CDI required readmission (56% vs 4%, p < 0.001). A third of children with CDI had undergone vesicoureteral reflux correction, comprising 0.3% of the included procedures. Over 11% of children with CDI had undergone nephrectomy, comprising 1.1% of the included procedures for the highest rate. DISCUSSION: CDI are uncommon following pediatric urologic procedures. No patients undergoing inguinal or scrotal cases developed CDI, while only one patient developed CDI after penile surgery. Our study does have several important limitations: we are unable to provide clinical information about the exact diagnoses, CDI risk factors such as antibiotic usage or comorbid conditions, and the number of patients who were tested for CDI. CONCLUSION: While pediatric urologists are unlikely to encounter postoperative CDI, when they occur, they are associated with longer lengths of stay, increased readmission rates, and an increased rate of non-CDI complications.

Syndromic Wilms tumor: a review of predisposing conditions, surveillance and treatment.

Predisposing syndromes associated with an increased risk of Wilms tumor (WT) are responsible for 9-17% of all cases of the malignancy. Due to an earlier age at WT diagnosis and an increased incidence of bilateral and metachronous disease, management of syndromic WT warrants a distinct approach from that of non-syndromic WT. This review of English-language manuscripts about WT focuses on the most common syndromes, surveillance protocols and current treatment strategies. Highlighted syndromes include those associated with WT1, such as WAGR (Wilms-Aniridia-Genitourinary-mental Retardation), Denys-Drash syndrome (DDS), and Frasier syndrome, 11p15 defects, such as Beckwith-Wiedemann syndrome (BWS), among others. General surveillance guidelines include screening renal or abdominal ultrasound every 3-4 months until the age of 5 or 7, depending on the syndrome. Further, some of the predisposing conditions also increase the risk of other malignancies, such as gonadoblastoma and hepatoblastoma. With promising results for nephron-sparing surgery in bilateral non-syndromic WT, there are increasing reports and recommendations to pursue nephron-sparing for these patients who are at greater risk of bilateral, metachronous lesions. In addition to the loss of renal parenchyma from malignancy, many patients are at risk of developing renal insufficiency as part of their syndrome. Although there may be some increase in the complication rate, recurrence free survival seems equivalent. Some conditions require specialized approaches to adjuvant therapy, as their syndrome may make them especially susceptible to side effects.

Do current scientific reports of hydronephrosis make the grade?

BACKGROUND: In December 2014, Nguyen et al. introduced the Upper Tract Dilation (UTD) classification scheme, hoping to unify multiple disciplines when describing ultrasound imaging of congenital hydronephrosis. We hypothesized that the academic community has been slow to adopt its use in publications. PRIMARY AIM: To evaluate which hydronephrosis grading systems were currently preferred in publications. STUDY DESIGN: A PubMed® search for hydronephrosis was performed, and abstracts between May 2017 and May 2019 were reviewed. The following data points were collected from the 197 manuscripts meeting inclusion criteria: journal, first and senior author specialty, country, type of article, primary pathology, and classification of hydronephrosis when present. Differences between use of classification system, and author specialty, manuscript type, and pathology were evaluated. RESULTS: First and/or senior author specialties were most commonly pediatric urology, urology, pediatric surgery, and pediatric nephrology. The manuscripts were comprised of retrospective studies (48.2%), prospective studies (25.4%), case reports (15.7%), review articles (9.1%), and systematic reviews (1.5%). The most common pathologies were hydronephrosis (36.5%) and ureteropelvic junction obstruction (21.3%). Over 20% of manuscripts did not categorize hydronephrosis at all. The UTD classification was used by 5.6%, while Society for Fetal Urology (SFU) grading was used by 37.1% and Anterior-Posterior Diameter (APD) measurements by 32.5%. The Summary Table presents grading system by manuscript type, specialty, and pathology. DISCUSSION: There is great variability in hydronephrosis grading. One potential weakness of our study is that sufficient time may not have passed for the UTD system to be adopted. Researchers may need more time to complete and publish their studies, or could be awaiting further validation of UTD utility. They could also be hesitant to change systems when it is unknown if one classification schema is superior to another, either in general or for specific diagnoses. Another weakness is that this study does not quantify what, if any, systems are used clinically. Some attempt to provide objective classification would help clarify the implications of the manuscript for research or clinical applications. Reviewers should ensure that where possible, adequate descriptions of hydronephrosis are included. Education outreach to other specialties may help increase objective grading in research. CONCLUSIONS: The UTD system is not commonly utilized in the literature. SFU grading is applied most commonly, followed by APD measurements. Over one third of manuscripts used no classification system or descriptive terminology.

Using a Decision Tree to Guide Bowel Management in Spina Bifida.

BACKGROUND: When born with spina bifida, there are numerous neurologic disorders that accompany this birth defect, including neurogenic bowel. Proactive, systematic, and rational approaches can lead to continence and a more functional lifestyle [1]. METHODS: Based on the evidence in the literature and expert experience, our approach to bowel management was developed as a step by step, individualized approach. This was converted to a decision tree for easy guidance of treatment decisions. The approach includes teaching patients and families normal bowel function, changes resulting from neurogenic bowel, common pitfalls in bowel management, and techniques that may improve outcomes. The decision tree, starting with dietary management, breaks into a two-fold attack, oral and rectal. Our data as part of the National Spina Bifida Patient Registry (NSBPR) database was compared to public data from the NSBPR. RESULTS: Preliminary data from the NSBPR in 2011 reported bowel continence in 42.1% (n = 898) compared to our clinical outcomes of 72.1% (n = 43). As the variable of bowel continence was further defined and more patients were enrolled, the clinic results were comparable to the national reports. CONCLUSION: Consistency among providers and caregivers is critical to evaluating the management of continence in spina bifida. While this protocol warrants further evaluation, it is offered as an evidence-based, step by step, approach to bowel management in spina bifida with good outcomes for patient management.

Workplace Bullying of Urology Residents: Implications for the Patient and Provider.

OBJECTIVE: To elucidate whether urology residents in the United States feel bullied by nurses, how respected they feel at work, and whether this impacts personal and patient care. METHODS: We distributed an Institutional Review Board-approved online, validated, revised Negative Acts Questionnaire to US urology residents in their first year or above. We evaluated bullying through scoring work (total range 5-25), person (total range 9-45), and physical intimidation (total range 3-15) related bullying domains. We also solicited how respected residents feel by different staff on a Likert scale and the perceived personal and professional impact of bullying. Bullying domains were assessed with descriptive statistics and mean total bullying scores (MTBS) and demographics compared. RESULTS: We received 102 responses (82% MD, 18% DO). One resident reported never experiencing bullying. Overall average MTBS was 28.9 ± 0.9 (17-68). 98.0%, 82.4%, and 77.5% of residents reported at least 1 incident of work, person, and physical intimidation-related bullying, respectively. DO residents reported higher MTBS than MD residents (33.7 ± 2.2vs 27.8 ± 1.0, P = .015). Higher MTBS scores were seen in residents who feared retaliation and considered transferring programs, while lower scores were seen where the resident-nurse relationship was nurtured. CONCLUSION: Ninety percent of residents perceived some degree of bullying and report a negative impact on personal performance and patient outcomes.

Differences in early career operative experiences among pediatric urologists.

INTRODUCTION: Previous research suggests that pediatric urologists feel well trained by their fellowship for cases encountered early in their career. We questioned the complexity and diversity of cases new pediatric urologists were actually performing. OBJECTIVE: The aim was to identify the frequency with which newly trained pediatric urologists are performing various procedures, investigate which factors are associated with case complexity and diversity, and evaluate for differences between male and female surgeons. STUDY DESIGN: Case logs of urologists from July 30, 2007, to June 30, 2013, initially applying for the certifying examination who self-identified as pediatric urologists were reviewed. Data points included cases/dates, and surgeon demographics. An in-depth analysis was performed on 51 index cases from the 71 included pediatric urologists, for which a level of complexity was assigned. RESULTS: Compared with the bottom volume quartile, surgeons in the top quartile performed more cases of minimal (115.9 ± 8.7 vs. 51.7 ± 8.7, p < 0.001), moderate (31.1 ± 2.7 vs. 10.1 ± 1.0, p < 0.001) and significant (10.8 ± 1.9 vs. 2.0 ± 0.4, p < 0.001) complexity. More than 90% logged circumcisions, orchiopexies, and inguinal hernia repairs, while less than 1.5% logged open nephroureterectomies or complete male epispadias repair. Surgeons submitted at least one of 17.2 ± 0.5 (range 5-28) unique codes. The figure presents the percentage of current procedural terminology (CPT) codes performed by each urologist. Surgeons with the least case diversity performed a higher percentage of low-complexity cases, and lower percentages of moderate and complex cases (p < 0.001). Males, comprising 60.6% of urologists, performed more cases than females (342.9 ± 30.9 vs. 229.1 ± 18.1, p = 0.007), averaging more cases of minimal (95.0 ± 6.6 vs. 73.3 ± 4.6, p = 0.018) and significant (6.7 ± 1.0 vs. 2.8 ± 0.5, p = 0.005) complexity. There was no difference in cases of moderate complexity (22.0 ± 1.9 vs. 18.1 ± 2.1, p = 0.201). DISCUSSION: In general, pediatric urologists should expect to perform many minor cases when they enter practice. Women are entering urology in increasing numbers. In our study, female urologists performed fewer cases. This could have implications for the workforce, which in urology in general is expected to decrease. CONCLUSIONS: Case diversity and degree of complexity vary among newly trained pediatric urologists. The urologist with the greatest case diversity never performed 45% of the 51 analyzed CPT codes, while the one with the least case diversity never performed 90% of the codes. Male surgeons performed more operations, particularly those of minimal and significant complexity. The variability in operative experience reinforces the importance of continuing education and mentorship after completion of fellowship.

Bladder Management and Continence Outcomes in Adults with Spina Bifida: Results from the National Spina Bifida Patient Registry, 2009 to 2015.

PURPOSE: Most children with spina bifida now survive into adulthood, although most have neuropathic bladder with potential complications of incontinence, infection, renal damage and diminished quality of life. In this study we sought to 1) describe contemporary bladder management and continence outcomes of adults with spina bifida, 2) describe differences from younger individuals and 3) assess for association with socioeconomic factors. MATERIALS AND METHODS: We analyzed data on bladder management and outcomes in adults with spina bifida from the National Spina Bifida Patient Registry. A strict definition of continence was used. Results were compared to young children (age 5 to 11 years) and adolescents (12 to 19). Statistical analysis compared cohorts by gender, ethnicity, spina bifida type, lesion level, insurance status, educational attainment, employment status and continence. RESULTS: A total of 5,250 patients with spina bifida were included, of whom 1,372 (26.1%) were adults. Of the adult patients 45.8% did not take medication, but 76.8% performed clean intermittent catheterization. Continence was decreased in adults with myelomeningocele (45.8%) vs those with nonmyelomeningocele spina bifida (63.1%, p <0.0001). Continence rates were higher in the older cohorts with myelomeningocele (p <0.0001) but not in those with nonmyelomeningocele spina bifida (p = 0.1192). Bladder management and history of urological surgery varied among age groups. On univariate analysis with spina bifida related or socioeconomic variables continence was significantly associated with educational level but on multivariable logistic regression analysis bladder continence was significantly associated with employment status only. CONCLUSIONS: Bladder management techniques differ between adults and children with spina bifida. Bladder continence outcomes were better in adults, with nearly half reporting continence. Continence was significantly associated with employment status in patients age 25 years or older.

Bowel management and continence in adults with spina bifida: Results from the National Spina Bifida Patient Registry 2009-15.

INTRODUCTION: Advances in care have allowed most children with spina bifida (SB) to live to adulthood. The majority have neuropathic bowel dysfunction (NBD), resulting in constipation, incontinence, and diminished quality of life. We sought to 1) describe contemporary NBD management and continence outcomes of adults with SB; 2) describe differences from younger patients; and 3) assess for association with socio-economic factors. METHODS: We analyzed data on NBD management and continence from the National Spina Bifida Patient Registry (NSBPR). Patients were segregated into young children (5-11 years), adolescents (12-19 years), and adults (20 years and older). A strict definition of continence was utilized. Statistical analysis compared cohorts by gender, ethnicity, SB type, lesion level, insurance status, educational attainment, employment status, and continence. RESULTS: A total of 5209 SB patients were included, of whom 1370 (26.3%) were adults. Management and continence varied by age and SB type. Oral medication use did not differ between groups (5.2-6.6%). Suppositories and rectal enemas were used only by 11.5% of adults, which was significantly less than among school-aged children. Antegrade enemas were used by 17.7% of adults which was significantly less than among adolescents (27.2%). Adults were more likely to use digital stimulation or disimpaction or have undergone a colostomy. Bowel continence was reported by 58.3% of overall adult cohort: 55.6% of adults with myelomeningocele and 74.9% with non-myelomeningocele. Bowel continence was significantly associated with employment (p= 0.0002), private insurance (p= 0.0098), non-myelomeningocele type of SB (p= 0.0216) and educational attainment (p= 0.0324) on univariate analysis but only with employment on multivariable logistic regression (p= 0.0027). CONCLUSIONS: Bowel management techniques differed between adults and younger patients with SB. Bowel continence was reported by over half of SB adults and was associated with socio-economic factors.

Transitional Urology for Male Adolescents: What Adult Urologists Should Know.

In recent years, there has been increasing interest in transitional urology, or how to best prepare patients with major congenital urologic diseases, such as bladder exstrophy and neuropathic bladder, to manage their own health care with adult urologists. However, common pediatric urologic conditions may be encountered by the adult urologist with more regularity. This review focuses on three relatively common conditions which may be identified in childhood, the consequences from which a patient may seek help from an adult urologist: cryptorchidism, varicocele, and Klinefelter syndrome.

Treatment patterns, testicular loss and disparities in inpatient surgical management of testicular torsion in boys: a population-based study 1998-2010.

OBJECTIVES: To examine temporal trends in inpatient testicular torsion (TT) treatment and testicular loss (TL), and to identify risk factors for TL using a large nationally representative paediatric cohort, stratified to established high prevalence TT cohorts (neonatal TT [NTT]; age <1 years) and adolescent TT (ATT; age 12-17 years). METHODS: Boys (age ≤17 years, n = 17 478) undergoing surgical exploration for TT were identified within the Nationwide Inpatient Sample (1998-2010). Temporal trends in inpatient TT management (salvage surgery vs orchiectomy) and TL were examined using estimated annual percent change methodology. Multivariable logistic regression models were used to identify risk factors for TL. RESULTS: Teaching hospitals treated 90% of boys with NTT, compared with 55% with ATT (P < 0.001). Of boys with NTT, 85% lost their testis, compared with 35% with ATT (P < 0.001). Inpatient management of NTT declined during the study period, from 7.5/100 000 children in 1998 to 3/100 000 in 2010 (estimated annual percent change -4.95%; P < 0.001). The decrease was similar but less dramatic in ATT. TL patterns did not improve. In adjusted analyses, for NTT, orchiectomy was more likely at teaching hospitals. For ATT, orchiectomy was more likely in children with comorbidities (odds ratio 5.42; P = 0.045), Medicaid coverage or self-pay (P < 0.05) and weekday presentation (P = 0.001). Regional or racial disposition was not associated with TL. CONCLUSIONS: There has been a gradual decrease in inpatient surgical treatment for both NTT and ATT, presumably as a result of increased outpatient and/or non-operative management of these children. Concerningly, TL patterns have not improved; targeted interventions such as parental and adolescent male health education may lead to timely recognition/intervention in children at-risk for ATT. We noted no regional/racial disparities in contrast to earlier studies.

Complexities of Müllerian Anatomy in 46XX Cloacal Exstrophy Patients.

STUDY OBJECTIVE: To characterize Müllerian anatomy in 46,XX cloacal exstrophy patients. DESIGN: Retrospective review of prospectively maintained, institutionally approved exstrophy-epispadias-cloacal exstrophy database. SETTING: Tertiary care, high-volume exstrophy center (Division of Pediatric Urology, The Johns Hopkins Hospital, Baltimore, Maryland). PARTICIPANTS: We included 31 patients who were genetically female with cloacal exstrophy for whom records included detailed evaluation of Müllerian anatomy. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Müllerian structures, method of evaluation, management, and sexual activity. RESULTS: Of our patients, 12.9% (3/31) had no identified abnormalities. Vaginal anatomy was described for 30 patients; 3/30 had vaginal agenesis, 14/30 had a single vagina, and 13/30 had vaginal duplication. Of 14 patients with 1 vagina, 5 had atresia/hypoplasia, and 1 had a lateral displacement. One patient with 2 vaginas also had distal atresia. Of the cervices evaluated, 9/14 were duplicated (2/9 with a solitary vagina), and 19/27 of the uteri were duplicated (6/22 with 1 vagina, 1/22 with no vagina). Five patients required imaging to fully characterize their anatomy, and 7 patients had studies that failed to identify Müllerian structures seen in the operating room or on physical examination. Common reconstructive surgeries included vaginoplasties, incisions of vaginal septa, colporrhaphies, and hysterectomies. Sexual activity was confirmed for 3 patients, 2 of whom had conceived. CONCLUSION: Most female cloacal exstrophy patients exhibit abnormalities of the Müllerian system. Axial imaging and ultrasound are helpful diagnostic adjuncts but do not replace careful physical examination and assessment in the operating room. Further studies of sexual activity and fertility are warranted.

Presentation of a Clitoral Mass in a Prepubescent Female: A Case Report and Discussion of the Evaluation.

Clitoral masses are rare. We present a case report of a prepubescent female with a periclitoral mass.

Practice patterns and outcomes of pediatric partial nephrectomy in the United States: Comparison between pediatric urology and general pediatric surgery.

BACKGROUND: In the United States, both pediatric urologists (PUROs) and general pediatric surgeons (GPSs) perform nephrectomies in children, with PUROs performing more nephrectomies overall, most commonly for benign causes. GPSs perform more nephrectomies for malignant causes. We questioned whether the same trends persisted for partial nephrectomy. OBJECTIVE: We hypothesized that PUROs performed more partial nephrectomies for all causes, including malignancy. Our primary aim was to characterize the number of partial nephrectomies performed by PUROs and GPSs. We also compared short-term outcomes between subspecialties. STUDY DESIGN: We analyzed the Pediatric Health Information System (PHIS), a database encompassing data from 44 children's hospitals. Patients were ≤18 years old and had a partial nephrectomy (ICD-9 procedure code 554) carried out by PUROs or GPSs between 1 January, 2004 and June 30, 2013. Queried data points included surgeon subspecialty, age, gender, 3M™ All Patient Refined Diagnosis Related Groups (3M™ APR DRG) code, severity level, mortality risk, length of stay (LOS), and medical/surgical complication flags. Data points were compared in patients on whom PUROs and GPSs had operated. Statistical analysis included the Student t test, chi-square test, analysis of covariance, and logistic regression. RESULTS: Results are presented in the table. While PUROs performed the majority of partial nephrectomies, GPSs operated more commonly for malignancy. For surgeries performed for non-malignant indications, PURO patients had a shorter LOS and lower complication rate after controlling for statistically identified covariates. There was no difference in LOS or complication rate for patients with malignancy. DISCUSSION: A Pediatric Health Information System study of pediatric nephrectomy demonstrated PUROs performed more nephrectomies overall, but GPSs performed more surgeries for malignancy. The difference was less dramatic for partial nephrectomies (63% GPS, 37% PURO) than for radical nephrectomies (90% GPS, 10% PURO). PUROs performed more partial nephrectomies for benign indications (94% PURO, 6% GPS) at an even greater rate than nephrectomies (88% PURO, 12% GPS). As a national database study, there are a number of inherent limitations: applicability of results to non-participating hospitals, possibility of inaccurate data entry/coding, and lack of data points that would be relevant to the study. CONCLUSIONS: While most partial nephrectomies in the United States are performed by PUROs, GPSs perform the majority of surgeries for malignancy. There is no difference in LOS or complication rate undergoing nephron-sparing surgery for malignant disease; however, PUROs had a shorter LOS and lower complication rate when operating for benign diseases.