Implementation and Validation of a High-Fidelity Simulation Model for Surgical Resident Training: Management of Acute Intraoperative Hemorrhage during Robot-assisted Surgery.

OBJECTIVES: To improve our previous simulation-based training module by using sustainable material to mold an anatomically accurate terrain and reproducing major vascular injuries encountered during robot-assisted nephrectomy. METHODS: The simulator was built with a pump, gauge, and valve linked via silicone tubing. Artificial blood was made from cornstarch, water, and red dye, and pumped through 3D-Med artificial vessels with the dimensions of an average renal artery. Silicone was used to emulate the pliability of organic tissue and mold an anatomically accurate terrain. Eight urologic residents participated in the pilot simulation. We employed validated assessment tools including Non-Technical Skills for Surgeons and Objective Structured Assessment of Technical Skills forms to guide debrief sessions moderated by an expert physician after individual performance evaluations. RESULTS: The apparatus demonstrated high reproducibility across all simulation scenarios, enhancing resident problem-solving skills. Residents' pre-simulation surveys revealed significant concern regarding their acute hemorrhage management. Residents' post-simulation survey demonstrated average realism scores increased from 4.375 to 4.75. Residents also felt the simulator enhanced learning, offering valuable practice and knowledge applicable to their surgical specialty. CONCLUSIONS: The management of acute hemorrhage during robot-assisted surgery remains a space for additional surgical education and training. Our simulation successfully provided a reliable, reproducible training for residents to practice their technical and non-technical skills in managing acute hemorrhage.

Variation in content discussed by specialty in consultations for clinically localized prostate cancer.

INTRODUCTION: Multidisciplinary consultations improve decisional conflict and guideline-concordant treatment for men with prostate cancer (PC), but differences in the content discussed by specialty during consultations are unknown. METHODS: We audiorecorded and transcribed 50 treatment consultations for localized PC across a multidisciplinary sample of urologists, radiation oncologists, and medical oncologists. Conversation was coded for narrative content using an open coding approach, grouping similar topics into major content areas. The number of words devoted to each content area per consult was used as a proxy for time spent. Multivariable Poisson regression calculated incidence rate ratios (IRR) for content-specific word count across specialties after adjustment for tumor risk and patient demographics. RESULTS: Coders identified 8 narrative content areas: overview of PC; medical history; baseline risk; cancer prognosis; competing risks; treatment options; physician recommendations; and shared decision making (SDM). In multivariable models, specialties significantly differed in proportion of time spent on treatment options, SDM, competing risks, and cancer prognosis. Urologists spent 1.8-fold more time discussing cancer prognosis than medical oncologists (IRR1.80, 95%CI:1.14-2.83) and radiation oncologists (IRR1.84, 95%CI:1.10-3.07). Urologists (IRR11.38, 95%CI:6.62-19.56) and medical oncologists (IRR10.60, 95%CI:6.01-18.72) spent over 10-fold more time discussing competing risks than radiation oncologists. Medical oncologists (IRR2.60, 95%CI:1.65-4.10) and radiation oncologists (IRR1.77, 95%CI:1.06-2.95) spent 2.6- and 1.8-fold more time on SDM than urologists, respectively. CONCLUSIONS: Specialists focus on different content in PC consultations. Our results suggest that urologists should spend more time on SDM and radiation oncologists on competing risks. Our results also highlight the importance of medical oncologists in facilitating SDM.

The impact of life expectancy on cost-effectiveness of treatment options for clinically localized prostate cancer.

BACKGROUND: Life expectancy (LE) impacts effectiveness and morbidity of prostate cancer (CaP) treatment, but its impact on cost-effectiveness is unknown. We sought to evaluate the impact of LE on the cost-effectiveness of radical prostatectomy (RP), radiation therapy (RT), and active surveillance (AS) for clinically localized disease. METHODS: We created a Markov model to calculate incremental cost-effectiveness ratios (ICERs) for RP, RT, and AS over a 20-year time horizon from a Medicare payer perspective for low- and intermediate-risk CaP. Mortality outcomes varied by tumor risk and PCCI score, a validated proxy for LE. We performed 1,000 Monte Carlo simulations with 1-way sensitivity analyses of PCCI within each tumor risk subgroup to compare cost/quality-adjusted life years (QALYs) between treatments. RESULTS: AS dominated RP and RT for low- and intermediate-risk disease in men with LE ≤10 years (PCCI ≥7 and ≥9, respectively). However, AS failed to dominate RP and RT for men with longer LE. For men with low-risk cancer and LE>10 years (PCCI 0-6), AS had the greatest effectiveness, but failed to dominate due to higher cost relative to RP. For men with intermediate-risk cancer with LE>10 years, AS failed to dominate due to higher cost relative to RP (PCCI 0-8) and lower effectiveness relative to RT (PCCI 0-3). The range of QALYs between RP, RT, and AS varied <13% (range: 0%-12.9%) while costs varied up to 521% (range 0.5%-521%) across PCCI scores. CONCLUSIONS: LE strongly modulates the cost of CaP treatments. This results in AS dominating RP and RT in men with LE ≤10 years. However, in men with longer LE, AS fails to dominate primarily due to its high cumulative costs, underscoring the need for risk-adjusted AS protocols.

Patient and Parent Perspectives on Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia.

BACKGROUND: Testicular adrenal rest tumors (TARTs) increase the risk of infertility in males with classic congenital adrenal hyperplasia (CAH). There is no consensus regarding at what age screening testicular ultrasounds should begin and how often they should be repeated. Furthermore, it is unknown whether patients and parents are aware of the significance of TARTs. OBJECTIVE: The objective of the study was to investigate awareness, concern, and screening rates for TARTs in males with classic CAH. METHODS: Males with CAH and parents completed an online questionnaire from 2019 to 2020. Responses to questions about TARTs were analyzed. Fisher's exact test was used to determine statistical significance. RESULTS: Of 123 responders, 14 were males with CAH (range 16-54 years) and 109 were parents of males with CAH (son's age range infancy to 37 years). Of all responders, 74% were concerned about the possibility of TARTs, 48% had discussions about TARTs with their endocrinologist, and 42% were aware of possible infertility in males with CAH. There was no difference between responses provided by affected males and parents for these topics (p ≥ 0.08). Among male responders with CAH, 93% had at least one testicular ultrasound, and 77% had undergone more than one. Among parent responders, 30% of their sons had at least one testicular ultrasound, and 61% had more than one. The frequency, total number, and age when the first testicular ultrasound was obtained were inconsistent in both groups. Fifty percent of male responders with CAH and 11% of sons were referred to a urologist for evaluation. CONCLUSIONS: Although most responders were concerned about TARTs, less than half recalled discussing this issue with their endocrinologist, and less than half were aware of the possibility of infertility. Although TARTs are most often treated medically, several responders were referred to a urologist. Standardized patient education and consensus guidelines are needed for the surveillance and management of TARTs in males with classic CAH.

Sociodemographic differences in opioid use and recovery following ambulatory pediatric urologic procedures.

Our aim was to examine associations between sociodemographic factors and postoperative opioid use and recovery among pediatric patients undergoing outpatient urologic procedures. We retrospectively evaluated 831 patients undergoing ambulatory urologic procedures from 2013 to 2017 at an urban pediatric hospital. Patients were evaluated for days of opioid use and days until return to baseline behavior. Differences in outcomes by race/ethnicity, primary language, median neighborhood household income, and health insurance type were analyzed using negative binomial regression models. Overall, patients reported a median of 1.0 day (IQR: 2.0) of postoperative opioid use and 3.0 days (IQR: 6.0) of recovery time. After controlling for covariates, patients with non-English speaking parents took opioids for 26.5% (95% CI: 11.4-41.7%) longer and had 27.8% (95% CI: 8.1-51.0%) longer recovery time than patients with English-speaking parents. Hispanic patients took opioids for 27.5% (95% CI: 0.1-54.9%) longer than White patients. Patients with public insurance used opioids for 47.6% (95% CI: 5.0-107.4%) longer than privately insured patients. Non-English speaking, Hispanic, and publicly insured patients had a longer duration of postoperative opioid use than primarily English-speaking, White, and privately insured patients, respectively. Identifying these disparities is important for designing equitable postoperative care pathways.

Three cases utilizing the Hidden Incision Endoscopic Surgery approach to port placement during penile inversion vaginoplasty utilizing a peritoneal flap.

INTRODUCTION: Younger transgender patients undergoing penile inversion vaginoplasty (PIV) after pubertal suppression often require modified techniques to augment neovaginal tissue. Peritoneal flap vaginoplasty (PFV) is a well-established technique for improving neovaginal depth. Utilizing a Hidden Incision Endoscopic Surgery (HIdES) approach to port placement improves cosmetic outcomes of this identity-affirming procedure. OBJECTIVE: This video shows the efficacy of the HIdES port placement configuration on the daVinci robot for peritoneal flap creation and closure during PIV using the modified laparoscopic Davydov procedure. MATERIALS AND METHODS: Three young adult transgender females who had not undergone laser hair removal/epilation underwent PIV with robotic PFV utilizing HIdES port placement. The anterior superior iliac spine (ASIS) was demarcated bilaterally, and ports were placed at or below these lines. Two surgical teams, external genitoplasty and internal peritoneal flap closure via daVinci Xi robot, worked simultaneously. RESULTS: All patients underwent successful robotic assisted PIV with HIdES port placement. All patients had similar postoperative courses of return of bowel function with flatus on postoperative day (POD) 2, vaginal dressing, urethral catheter removal, and initiation of vaginal dilation on POD 5-6, and hospital discharge between POD 5-8. CONCLUSION: We present our initial experience with robotic/laparoscopic PFV using a hidden incision approach. This technique allows for excellent neovaginal depth with improved abdominal wound aesthetics.

Epithelioid Hemangioma of the Glans Penis in an Adolescent Male: A Case Report.

Epithelioid hemangioma is a rare vascular lesion consisting of capillaries and inflammatory infiltrate containing lymphocytes, and mast cells. The presentation of penile epithelioid hemangioma has been previously described in the adult literature; however, few cases have been reported in the pediatric population. Herein we present a case of penile epithelioid hemangioma in a 15-year-old patient with regrowth following surgical resection, requiring more extensive surgical excision with urethral reconstruction. This rare case highlights the importance of a proper diagnosis and complete microscopic removal.

Growing up with clitoromegaly: experiences of North American women with congenital adrenal hyperplasia.

INTRODUCTION: To describe experiences of clitoromegaly in women with congenital adrenal hyperplasia (CAH). METHODS: CAH females (46XX, ≥16 years old) from the United States and Canada were eligible for a cross-sectional online survey (2019-2020) if reporting clitoromegaly (life-long: "growing up with a larger than average clitoris," secondary: "clitoris grew over weeks or months"). A multidisciplinary team and women with CAH drafted questions assessing net effects of clitoromegaly on 10 activities and 10 life domains. Fisher's exact test was used to compare net effect (positive-negative) vs. no effect (Bonferroni p = 0.05/10 = 0.005). RESULTS: Of 97 women with CAH enrolled, 53 women (55%, median age: 36 years, advocacy group recruitment: 81%) reported recognizing clitoromegaly at median 11-13 years old, with 21% identifying it in adulthood. There was no difference in self-reported timing or clitoral shape between life-long or secondary clitoromegaly (p ≥ 0.06). There were no net positive effects of clitoromegaly. Rather, clitoromegaly had net negative effects on 7/10 activities (p ≤ 0.003) and no net effect (neutral) on 3 (Table). Women were less likely to wear tight clothing, change clothes in public locker rooms and play group sports. Women reported net negative effects for most romantic activities (dating, any sexual activity, pain-free sexual activity, having a partner see their genitalia, p=<0.003), but did not report a net effect on pleasurable sexual activity (p = 0.12). Clitoromegaly had net negative effects in 9/10 life domains (p < 0.001) and neutral on job self-perception (p = 0.25). Few women reported any positive impact (2-6%). However, 49-59% of women experienced poor self-esteem, anxiety, gender self-perception and body image, while 36% felt "down or depressed." Also, 21-23% experienced negative self-perception as friends and parents, 42-47% reported negative effects on plans for romantic and sexual relationships. Responses did not differ with advocacy group membership (p ≥ 0.02). DISCUSSION: Our findings support qualitative and case series evidence that clitoromegaly has a negative psychological outcome on women with CAH. Clitoromegaly may add to the burden of living with a chronic endocrine disease. Women with positive and negative experiences had the same opportunity to participate. Since we could not assess objective clitoral size, baseline virilization and exact nature of any childhood clitoral procedures, these data cannot be used to estimate the impact of specific clitoral size or effectiveness of early clitoral treatments. CONCLUSIONS: Clitoromegaly appears to be common among women with CAH. While experiences of clitoromegaly vary between women, the overall experience is negative in multiple social, romantic, and emotional activities and domains.

Sample Size, Power, and Risk of Misclassification in Pediatric Urology Hospital Rankings.

PURPOSE: We investigated 2019 and 2020 U.S. News & World Report methodologies of assessing pediatric urology surgical revision rates for distal hypospadias, pyeloplasty, and ureteral reimplantation to evaluate statistical power and misclassification risks. MATERIALS AND METHODS: Median annual volumes of distal hypospadias, pyeloplasty, and ureteral reimplantation procedures by hospital from 2016 to 2018 were calculated using the Pediatric Health Information System® database. U.S. News & World Report 2019 and 2020 methodologies were assessed to calculate power required to detect differences between hospitals and risk of hospital misclassifications. RESULTS: Median (IQR) annual hospital procedure volume was 72 (43-97) for distal hypospadias procedures, 19 (9-34) for pyeloplasties, and 35 (19-50) for ureteral reimplantations. Based on 2019 methodology, in order to achieve 80% power 764 cases/hospital are required to distinguish between a 1% vs 3% surgical revision rate, 1,500 cases/hospital are required to distinguish between a 3% vs 5% revision rate, and 282 cases/hospital are required to distinguish between a 1% vs 5% revision rate. Based on 2020 methodology, 98.0% of hospitals do not have adequate ureteral reimplantation volume to achieve full points even when reporting no revisions; similarly, 66.0% do not have adequate pyeloplasty volume, and 10.9% do not have adequate distal hypospadias volume. Risks of misclassification exceed 50% in several instances among hospitals reporting distal hypospadias and pyeloplasty revisions using both 2019 and 2020 methodology. CONCLUSIONS: Based on median-volume hospitals, current U.S. News & World Report methods for classifying revision rates for distal hypospadias, pyeloplasty, and ureteral reimplantation have insufficient power and are at high risk for misclassification.

The impact of patient age and procedure type on postoperative opioid use following ambulatory pediatric urologic procedures.

PURPOSE: The aim of this study is to determine whether patient age and procedure type are associated with duration of opioid use in pediatric patients undergoing ambulatory urologic procedures. METHODS: We retrospectively reviewed pediatric patients who underwent outpatient urologic procedures from 2013 to 2017. At postoperative visits, parents reported the number of days their child took opioid pain medication. Factors associated with duration of opioid use were evaluated using negative binomial regression models. RESULTS: 805 patients were included: 320 infants (39.8%), 430 children (53.4%), and 55 adolescents (6.8%). Overall mean length of opioid use was 1.7 (± 2.6) days. On average, infants used opioids for the shortest duration: 1.5 (± 2.3) days, followed by children: 1.7 (± 2.5) days, and adolescents: 3.1 (± 4.6) days. In adjusted models, adolescents used opioids for 85.2% longer (95% CI 13.1-161.8%; p < 0.001) than children and infants used opioids for 19.4% shorter duration (95% CI 0.4-34.7%; p = 0.05) than children. Each 1-year increase in age was associated with 6.1% increased duration of opioid use (95% CI 3.9-8.5%; p < 0.0001). Patients who underwent circumcision, hypospadias repair, and penile reconstruction took opioids for 75.9% (95% CI 42.6-117.1%; p < 0.001), 144.2% (95% CI 76.4-238.0%; p < 0.001), and 126.7% (95% CI 48.8-245.3%; p < 0.001) longer respectively than patients who underwent inguinal procedures. CONCLUSIONS: Increasing age, circumcision, hypospadias repair, and penile reconstruction are associated with increased duration of opioid use.

Social media engagement, perpetuating selected information, and accuracy regarding CA SB-201: Treatment or intervention on the sex characteristics of a minor.

INTRODUCTION: Disorders/differences of sex development (DSD) is a medical term used to encompass patients born with congenital conditions that lead to atypical development of the genitalia and reproductive structures. OBJECTIVE: To evaluate the factual accuracy of shared articles from popular social media platforms regarding the California State Bill, SB-201, Treatment or Intervention: Sex Characteristics of a Minor. DESIGN: We used the BuzzSumo© search engine to analyze the terms "SB 201", "intersex," "DSD," and "surgery ban" for worldwide social media engagement (Facebook, Twitter, Pinterest, and Reedit) one month before and after bill introduction on January 31, 2019, and final hearing on January 13, 2020. Articles were categorized based on source, opinion of the author, accuracy of scientific information, use of term intersex versus disorder/difference of sex development (DSD), definition of intersex, advocacy group quoted, reference to surgical "gender assignment," mention of negative consequences of the bill/other banned surgeries, the definition of medical necessity, parental rights, psychosocial concerns, and photographic content. RESULTS: Twenty unique articles with peak activity were analyzed. Eighteen were from news and two from editorial web sources. All mentioned SB-201.50% were classified as one-sided, meaning both arguments for and against were not presented. 60% of articles were perpetuating selected information correlating with the author's opinion. 65% of articles were misleading in terms of factual accuracy. All articles used the term intersex. 20% of articles used scientific terms such as atypical genitalia (2), DSD (2), and born with variations of sex characteristics (1). A urologist was quoted in 45% of articles, while 75% quoted intersex advocacy groups. 55% of articles referred to the surgeries as "gender normalizing," and 75% referred to "assigning gender". Three articles mentioned other non-DSD surgeries that SB-201 would ban in addition to any that "normalize appearance." 45% (9) included any definition of medical necessity, the most common being inability to urinate (7), which is incompatible with post-natal viability. DISCUSSION: Our study demonstrates that some of the most disseminated information on social media surrounding the introduction and hearing of SB-201 that did not include input from the medical community, perpetuated selected information, and lacked appropriate factual content. CONCLUSION: Misinformation in the media can be harmful to patients and the general public. This study highlights the need for balanced and accurate reporting on medical topics that can have emotional and political consequences when speaking to broader audiences.

What Do Patients and Parents Say About Parents' Ability to Choose Early Genital Surgery for Girls with Congenital Adrenal Hyperplasia?

PURPOSE: We assessed opinions of females with congenital adrenal hyperplasia and their parents about the parent's ability to choose early genital surgery for these patients. MATERIALS AND METHODS: We conducted an online survey of females with congenital adrenal hyperplasia (46XX,16+ years old) and independently recruited parents (2019-2020) diagnosed in first year of life in the United States. A multidisciplinary medical team, women with congenital adrenal hyperplasia and parents drafted the survey. Fisher exact test was used. RESULTS: Of 57 females with congenital adrenal hyperplasia (median age 39 years), 93.0% underwent genital surgery (median 1-2 years old). Most females (79.0%) believed legislation prohibiting surgery in childhood would cause harm. Most (64.9%) believed a ban "would have been harmful to me" (24.6% not harmful, 10.5% neutral). Most females (70.2%) believed a ban undermined parental rights to make medical decisions in their child's best interest. While 75.4% did not believe a ban was in the best interest of females with congenital adrenal hyperplasia, 14.0% did (10.5% neutral). For 132 parents of females with congenital adrenal hyperplasia (parent/child median age 40/11 years), 78.8% of children underwent surgery (median <1 year old). Most parents (93.9%) believed legislation prohibiting surgery in childhood would cause harm. Most (77.3%) believed a ban "would have harmed my daughter" (12.1% no harm, 5.3% neutral, 5.3% no answer). Parents were more likely than females with congenital adrenal hyperplasia to oppose a ban (p ≤0.02). Most parents (90.9%) believed a ban undermined parental rights. While 93.9% did not believe a ban was in the best interest of females with congenital adrenal hyperplasia, 3.8% did (2.3% neutral). CONCLUSIONS: The majority of females with congenital adrenal hyperplasia and their parents support the parents' ability to decide about potential genital surgery, opposing moratoria on surgery in childhood.

Robotic adrenalectomy in the pediatric population: initial experience case series from a tertiary center.

BACKGROUND: Laparoscopic resection is the most well described minimally-invasive approach for adrenalectomy. While it allows for improved cosmesis, faster recovery and decreased length of hospital stay compared with the open approach, instrument articulation limitations can hamper surgical dexterity in pediatric patients. Use of robotic assistance can greatly enhance operative field visualization and instrument control, and is in the early stages of adoption in academic centers for pediatric populations. CASE PRESENTATION: We present a single-institution series of pediatric adrenalectomy cases. The da Vinci Xi surgical system was used to perform adrenalectomies on three consecutive patients (ages, 2-13 years) at our center. Final pathology revealed ganglioneuroblastoma (n = 2) and pheochromocytoma (n = 1). Median operating time was 244 min (range, 244-265 min); median blood loss was estimated at 100 ml (range, 15-175 ml). Specimens were delivered intact and all margins were negative. Median post-operative hospital stay was 2 days (range, 1-6 days). All patients remain disease-free at median follow-up of 19 months (range, 12-30 months). CONCLUSION: Our experience continues to evolve, and suggests that robotic surgery is safe, feasible and oncologically effective for resection of adrenal masses in well-selected pediatric patients.

A survey and panel discussion of the effects of the COVID-19 pandemic on paediatric urological productivity, guideline adherence and provider stress.

INTRODUCTION: The COVID-19 pandemic has led to an unprecedented need to re-organise and re-align priorities for all surgical specialties. Despite the current declining numbers globally, the direct effects of the pandemic on institutional practices and on personal stress and coping mechanisms remains unknown. The aims of this study were to assess the effect of the pandemic on daily scheduling and work balances, its effects on stress, and to determine compliance with guidelines and to assess whether quarantining has led to other areas of increased productivity. METHODS: A trans-Atlantic convenience sample of paediatric urologists was created in which panellists (Zoom) discussed the direct effects of the COVID-19 pandemic on individual units, as well as creating a questionnaire using a mini-Delphi method to provide current semi-quantitative data regarding practice, and adherence levels to recently published risk stratification guidelines. They also filled out a Perceived Stress Scale (PSS) questionnaire to assess contemporary pandemic stress levels. RESULTS: There was an 86% response rate from paediatric urologists. The majority of respondents reported near complete disruption to planned operations (70%), and trainee education (70%). They were also worried about the effects of altered home-lives on productivity (≤90%), as well as a lack of personal protective equipment (57%). The baseline stress rate was measured at a very high level (PSS) during the pandemic. Adherence to recent operative guidelines for urgent cases was 100%. CONCLUSION: This study represents a panel discussion of a number of practical implications for paediatric urologists, and is one of the few papers to assess more pragmatic effects and combines opinions from both sides of the Atlantic. The impact of the pandemic has been very significant for paediatric urologists and includes a decrease in the number of patients seen and operated on, decreased salary, increased self-reported stress levels, substantially increased telemedicine usage, increased free time for various activities, and good compliance with guidelines and hospital management decisions.

Patient and Caregiver Attitudes toward Disorders of Sex Development Nomenclature.

PURPOSE: The medical terminology applied to differences/disorders of sex development has been viewed negatively by some affected individuals. A clinical population of patients with differences/disorders of sex development and their caregivers were surveyed regarding current nomenclature, hypothesizing that those unaffiliated with support groups would have more favorable attitudes. MATERIALS AND METHODS: We recruited English and Spanish speaking patients 13 years old or older with differences/disorders of sex development and their caregivers at 5 national tertiary care clinics from July 2016 to December 2018. No diagnoses were excluded. Participants completed a survey rating terminology commonly applied to differences/disorders of sex development. Responses were compared between subgroups, including members vs nonmembers of a support group. RESULTS: Of 185 potential participants approached 133 completed the survey (72% response rate). Congenital adrenal hyperplasia (33%) was the most common diagnosis. "Variation of sex development" was the most liked term (37%) but was not liked more significantly than "disorders of sex development" (27%, p=0.16). No term was liked by a majority of respondents. "Disorders of sex development" (37%) and "intersex" (53%) were the only terms most frequently viewed unfavorably. Support group members were significantly more likely to dislike the term "intersex" (p=0.02) and to like "variation of sex development" (p=0.02). CONCLUSIONS: A clinical population of patients and their caregivers had generally neutral attitudes toward nomenclature applied to differences/disorders of sex development. Members of a support group had clearer terminology preferences. "Variation of sex development" was the most liked term, and "disorders of sex development" and "intersex" were the most disliked. No term was liked by most respondents, and no clear alternative to the present nomenclature was identified.

Absence of Testicular Adrenal Rest Tumors in Newborns, Infants, and Toddlers with Classical Congenital Adrenal Hyperplasia.

INTRODUCTION: Testicular adrenal rest tumors (TART) are a known consequence for males with classical congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. TART are associated with potential infertility in adults. However, little is known about TART in very young males with CAH. OBJECTIVE: We assessed the presence of TART in newborn, infant, and toddler males with classical CAH via scrotal ultrasound. METHODS: Males with CAH had scrotal ultrasounds during the first 4 years of life, evaluating testes for morphology, blood flow, and presence of TART. Newborn screen 17-hydroxyprogesterone (17-OHP) and serum 17-OHP at the time of ultrasound were recorded. Bone ages were considered very advanced if ≥2 SD above chronological age. RESULTS: Thirty-one ultrasounds in 16 males were performed. An initial ultrasound was obtained in four newborns at diagnosis (6.8 ± 2.1 days), six infants (2.2 ± 0.9 months), and six toddlers (2.4 ± 0.9 years). Eleven males had at least one repeat ultrasound. A large proportion (11/16) were in poor hormonal control with an elevated 17-OHP (325 ± 298 nmol/L). One infant was in very poor hormonal control (17-OHP 447 nmol/L) at initial ultrasound, and two toddlers had advanced bone ages (+3.2 and +4.5 SD) representing exposure to postnatal androgens. However, no TART were detected in any subjects. CONCLUSIONS: TART were not found by scrotal ultrasound in males up to 4 years of age with classical CAH despite settings with expected high ACTH drive. Further research into the occurrence of TART in CAH may elucidate factors that contribute to the detection and individual predisposition to TART.

The effect of surgeon versus technologist control of fluoroscopy on radiation exposure during pediatric ureteroscopy: A randomized trial.

BACKGROUND: Fluoroscopy is commonly used during pediatric ureteroscopy (PURS) for urolithiasis, and the most important contributor to overall radiation exposure is fluoroscopy time (FT). One factor that may impact FT is who controls activation of the fluoroscope: the urologist (with a foot pedal) or the radiation technologist (as directed by the urologist). While there are plausible reasons to believe that either approach may lead to reduced FT, there are no systematic investigations of this question. We sought to compare FT with surgeon-control versus technologist control during PURS for urolithiasis. METHODS: We conducted a randomized controlled trial (Clinicaltrials.gov ID number: NCT02224287). Institutional Review Board approval was sought and obtained for this study. All subjects (or their legal guardians) provided informed consent. Each patient (age 5-26 years) was randomized to surgeon- or technologist-controlled fluoroscope activation. Block randomization was stratified by the surgeon. For technologist control, the surgeon verbally directed the technologist to activate the fluoroscope. For surgeon control, a foot pedal was used by the surgeon. The technologist controlled c-arm positioning, settings, and movement. The primary outcome was total FT for the procedure. Secondary outcomes included radiation exposure (entrance surface air kerma [ESAK] mGy). We also analyzed clinical and procedural predictors of FT and exposure. Mixed linear models accounting for clustering by surgeon were developed. RESULTS: Seventy-three procedures (5 surgeons) were included. The number of procedures per surgeon ranged from seven to 36. Forty-three percent were pre-stented. Thirty-one procedures were left side, 35 were right side, and seven were bilateral. Stones were treated in 71% of procedures (21% laser, 14% basket, and 65% laser/basket). Stone locations were distal ureter (11.5%), proximal/mid-ureter (8%), renal (69%), and ureteral/renal (11.5%). An access sheath was used in 77%. Median stone size was 8.0 mm (range 2.0-20.0). Median FT in the surgeon control group was 0.5 min (range 0.01-6.10) versus 0.55 min (range 0.10-5.50) in the technologist-control group (p = 0.284). Median ESAK in the surgeon control group was 46.02 mGy (range 5.44-3236.80) versus 46.99 mGy (range: 0.17-1039.31) in the technologist-control group (p = 0.362). Other factors associated with lower FT on univariate analysis included female sex (p = 0.015), no prior urologic surgeries (p = 0.041), shorter surgery (p = 0.011), and no access sheath (p = 0.006). On multivariable analysis only female sex (p = 0.017) and no access sheath (p = 0.049) remained significant. There was significant variation among surgeons (p < 0.0001); individual surgeon median FT ranged from 0.40 to 2.95 min. CONCLUSIONS: Fluoroscopy time and radiation exposure are similar whether the surgeon or technologist controls fluoroscope activation. Other strategies to reduce exposure might focus on surgeon-specific factors, given the significant variation between surgeons.

Melanotic Neuroectodermal Tumor of Infancy Presenting With Fast-Growing Scrotal Swelling: A Case Report and Literature Review.

Testicular melanotic neuroectodermal tumor of infancy (MNTI) is extremely rare, with 2 cases reported in the literature. Its rarity and rapid and infiltrative growth pattern pose a diagnostic challenge. A previously healthy 3-month-old male, presented with a history of worsening left hemiscrotal swelling for 1 week. An outside ultrasound was suggestive of testicular torsion. Left orchiectomy demonstrated a mass occupying almost entire testicle with a variegated cut surface, with areas of pigmentation, necrosis, and hemorrhage. Histological examination confirmed MNTI of the testis and epididymis. MNTI should be included in differential diagnosis in infants presenting with fast-growing scrotal swelling.