Recommendations for 46,XX Congenital Adrenal Hyperplasia Across Two Decades: Insights from the North American Differences of Sex Development Clinician Survey.

Several aspects of clinical management of 46,XX congenital adrenal hyperplasia (CAH) remain unsettled and controversial. The North American Disorders/Differences of Sex Development (DSD) Clinician Survey investigated changes, over the last two decades, in clinical recommendations by specialists involved in the management of newborns with DSD. Members of the (Lawson Wilkins) Pediatric Endocrine Society and the Societies for Pediatric Urology participated in a web-based survey at three timepoints: 2003-2004 (T1, n = 432), 2010-2011 (T2, n = 441), and 2020 (T3, n = 272). Participants were presented with two clinical case scenarios-newborns with 46,XX CAH and either mild-to-moderate or severe genital masculinization-and asked for clinical recommendations. Across timepoints, most participants recommended rearing the newborn as a girl, that parents (in consultation with physicians) should make surgical decisions, performing early genitoplasty, and disclosing surgical history at younger ages. Several trends were identified: a small, but significant shift toward recommending a gender other than girl; recommending that adolescent patients serve as the genital surgery decision maker; performing genital surgery at later ages; and disclosing surgical details at younger ages. This is the first study assessing physician recommendations across two decades. Despite variability in the recommendations, most experts followed CAH clinical practice guidelines. The observation that some of the emerging trends do not align with expert opinion or empirical evidence should serve as both a cautionary note and a call for prospective studies examining patient outcomes associated with these changes.

Bowel bladder dysfunction in control children in a pediatric urology office.

INTRODUCTION: Bowel bladder dysfunction (BBD) is common in children. Risk factors for BBD include age, gender, obesity, and behavioral issues such as ADHD. We investigated the modified Swedish Bowel-Bladder questionnaire as a sensitive indicator of BBD in healthy children. OBJECTIVES: We tested the usefulness of the Swedish Bowel-Bladder Questionnaire (BBQ) as an indicator of BBD in children not complaining of bowel/bladder dysfunction at the time of their visit to a pediatric urologist. Our secondary aim was to identify correlations between BBQ scores and risk factors such as gender, BMI, and ADHD. STUDY DESIGN: All families in our Pediatric Urology practice with patients >30 months old who were reportedly toilet trained were provided the Swedish BBQ. Total score as well as sub-scores for storage, emptying, and constipation were prospectively collected. Presenting diagnosis, gender, BMI, and ADHD history were collected. BBQ scores for patients with voiding dysfunction were compared to controls: a) those with genital problems (e.g. hydrocele/undescended testes), b) those with CAKUT (congenital anomalies of the kidneys and urinary tract). BBQ scores were analyzed as a continuous variable vs the potential risk factors (ADHD, obesity, age, and gender) using univariable/multivariable regression analysis. RESULTS: The median BBQ score for the 328 control patients (95 CAKUT and 233 genital) was 2.25 with an IQR: 1 to 6. In contrast, the median BBQ was higher for those with possible voiding dysfunction; n = 282; 9 with an IQR: 5 to 15). Total BBQ score exceeded 6 in 16% (52/328) of control patients. On multivariable analysis, age-adjusted total BBQ scores increased with ADHD in our controls (p = 0.03) but were unaffected by gender or BMI. On multivariable analysis of the voiding dysfunction group, total BBQ scores similarly decreased with age (p < 0.001) and increased with ADHD (p < 0.001) and were affected by gender (p = 0.024). BMI percentile had no significant effect in either cohort. DISCUSSION AND CONCLUSION: The Swedish BBQ was used in a U.S population and demonstrated trends towards increased voiding dysfunction associated with younger age, female gender, and ADHD. We learned that voiding dysfunction in children with ADHD is not associated with constipation and improves over time. Additionally, 16% of children with genital or urinary abnormalities and no voiding complaints have noticeable BBD uncovered by the BBQ. Hence, we conclude that the Swedish BBQ is a sensitive indicator of BBD even in children not complaining of those problems.

Revisiting Postoperative Pediatric Urology Opioid Prescriptions.

OBJECTIVE: To measure our opioid prescription rate, determine if our rate has decreased since 2019, and identify areas for future interventions to further decrease our opioid prescription rate. METHODS: We retrospectively reviewed all pediatric urology patients (age ..±18 years) who underwent a procedure between October 1, 2020 and October 22, 2021. We collected data on opioid prescribing, age, sex, surgeon, procedure, ethnicity, and race. We grouped procedures into 6 categories: circumcision, cystoscopy with the removal of foreign body/stone/stent, scrotal surgery, hypospadias repair/penile surgery, pyeloplasty/ureteral reimplant, and others. RESULTS: We analyzed 821 operative cases. Only 2.2% (18/821) of discharges included an opioid prescription. The prescription rate of 1 pediatric urologist was 4.6% (17/369), which was higher than the other 2 practitioners... (0.40%, 1/250%, and 0%, 0/202) (P.ß<.ß.001). The median age of patients who received an opioid prescription was older than patients without an opioid prescription (16.5 vs.ß5.0 years, P.ß<.ß.001). Surgery performed in an inpatient setting was more likely to result in an opioid prescription (9.7%, 3/31) than in the outpatient setting (1.9%, 15/790) (P.ß=.ß.03). No adverse effects of reduced opioid usage were noted. CONCLUSION: From October 2020 to October 2021, our institution had an opioid prescription rate of 2.2%. This represented a decrease from our previously reported rate of 8% in 2019. At the same time, we found no significant pain issues in our post-operative patients. Seventeen out of 18 prescriptions were written under 1 provider. Though heightened awareness has made a difference, targeted feedback is needed if we wish to reduce opioid usage further.

24-Hour urine collection for first time pediatric stone formers: Is it worth it?

INTRODUCTION/OBJECTIVE: 24-h urine collections are recommended for motivated first-time stone formers. Given that children have a lifetime potential for recurrences, metabolic work-up has been recommended. 24-hour urine collections can be problematic, especially in children. We sought to study the benefits of 24-h urine collections in children with stones. STUDY DESIGN: We performed a single center, retrospective chart review of the most recent pediatric nephrolithiasis patients under age 18 at our center who supplied their first 24-h urine collection. We assessed whether 24-h urine results led to a change in management and if those patients were adherent to the recommendations. RESULTS: Seventy pediatric nephrolithiasis patients who had 24-h urine collection were reviewed. Recommendations other than standard dietary and fluid intake changes were made in 8/70 (11%). A low citrate/calcium ratio (327 vs. 525, p < 0.03) and whether the test was ordered by nephrology vs. urology (26% vs. 2%, p < 0.003) were predictive of an additional recommendation. Of the 8 patients who had changes recommended only 1/8 completed a repeat 24-h urine collection, 3/8 never returned for followed up and 2/8 stopped the medicines prior to follow up. There was no difference in early stone recurrence rates, 55% of the studies were incorrectly collected, and total costs are estimated at $9800. DISCUSSION: Our study aimed to evaluate the impact and value of 24-h urine collection in first time pediatric stone formers. We found that 24-h urine collections altered management from standard dietary recommendations in only 11% of cases. These collections were fraught with challenges - 55% of our samples appeared to be incorrectly collected, there was at least one abnormality noted in 100% of collections, these tests are expensive, and patients were poorly compliant with recommendations based on test results. Additionally, changes made based on the 24-h urine results seemed to vary depending on who evaluated the test results. Among cases in which changes were made, nephrologists made alterations at a far greater rate than urologists did. We do acknowledge there are several limitations to our study. First, this is a retrospective chart review. Second, for the urology patients, we were only able to review patient records that were available due to a transition from one electronic medical record to another, resulting in a loss of some earlier patient records. We highly doubt that those records we could not review were significantly different than those we did review. Third, this is a single center design and includes the practice patterns of the providers here. We acknowledge that our local practice patterns may or may not be reflective of national practice patterns, however, most clinicians are likely faced with similar interpretation issues and poor rates of compliance and could benefit from guidelines. CONCLUSION: 24-h urine collection for first time pediatric stone formers is expensive, difficult to accomplish and infrequently leads to treatment changes. Our data suggest it adds little for most children with stones and may be better reserved for those children with recurrent stone disease.

Individualized care for patients with intersex (differences of sex development): part 4/5.Considering the Ifs, Whens, and Whats regarding sexual-reproductive system surgery.

Issues and concerns regarding surgery of the sexual-reproductive anatomy during infancy and early childhood are discussed using four actual examples. A case of a 46, XX infant with 21 hydroxylase deficiency congenital adrenal hyperplasia (CAH) with atypical (ambiguous) genitalia is discussed regarding timing and potential harms and benefits of surgery. We present the perspective of balancing the child's rights to bodily autonomy and right to an open future versus parents' decision making authority regarding what they perceive as their child's future best interests. The second case is a newborn with complete androgen insensitivity syndrome and we discuss the harms, benefits and timing of gonadectomy. The third case examines the physical and psychological impact of penile shaft hypospadias, raising the question of whether surgery is justified to prevent what may or may not be considered a permanent disability. The fourth case involves an adult woman with classic CAH, born with a urogenital sinus and clitoromegaly, who never had genital surgery and is now requesting vaginoplasty, but not clitoral reduction. The primary message of this article, as the previous articles in this series, is to encourage patient-family centered care that individualizes treatment guided by shared decision making.

A Case of Ewing Sarcoma of the Bladder Presenting in Early Infancy.

A 6-week-old female presented with gross hematuria and was diagnosed with Ewing sarcoma of the bladder through ultrasound and cystoscopic biopsies, along with a negative metastatic workup. She was treated with transurethral resection, chemotherapy consisting of with vincristine, cycolphosphamide, doxorubicin, ifosfamide and etoposide, and partial cystectomy. After completing chemotherapy, the patient has been doing well with no evidence of disease. There have been 14 other cases, 4 pediatric, of Ewing sarcoma of the bladder reported. To our knowledge, our case is the youngest patient reported with this disease.

Variation in Urinary Stone Parameters Throughout the Day and the Effect of Increased Fluid and Citrate Supplementation.

Background: Because 24-hour urine collections are cumbersome, many studies have evaluated the use of spot urine samples as a substitute, mostly finding poor concordance between the two. Daily variation in stone parameters probably contributes to the lack of concordance, but specific variation in various stone parameters is not well delineated. The variations likely lead to peaks and troughs, which can increase the risk of stone formation. Methods: We prospectively recruited 20 nonstone-forming patients, recording their total fluid intake over 24 hours and collecting voids at first morning, 9 to 10 A.M., 1 to 2 P.M., and 4 to 5 P.M. for evaluation of pH, specific gravity, calcium, citrate, and creatinine. Participants were then asked to double their fluid intake and take a daily True Lemon supplement over the course of the next 3 days. Urine was recollected postintervention. Results: Baseline [citrate]/[creatinine] increased throughout the day such that the 5 P.M. level was significantly higher compared with first void (0.58 vs 0.42, p = 0.027); [calcium]/[creatinine] daily variation was not statistically significant, but showed a distinct pattern that was present in both sets of collections. Daily [calcium]/[citrate] variation was significantly (p = 0.004) and consistently highest in the early morning on both day 1 (0.43) and day 4 (0.45). There was no significant variation in specific gravity and pH. Increasing fluid intake and citrate supplementation increase the daily variation in pH and [citrate]/[creatinine], but did not increase the values compared with their respective preintervention void times. There was also no detectable postintervention effect on [Ca]/[creatinine] or specific gravity. Conclusions: Urinary citrate concentration follows a circadian pattern, while urinary calcium has a diurnal excretion pattern. [Calcium]:[citrate] is highest in the early morning, indicating a high-risk time of day for stone formation. Spot urine samples identify a key time of day, which 24-hour urine collections may miss, for clinical monitoring.

Failure to Attend Urology Appointments: A Retrospective Analysis of a Single Urban Academic Practice.

INTRODUCTION: We sought to examine why patients miss appointments in a large academic urology practice. METHODS: We conducted a retrospective analysis of 83,983 patient appointments in our faculty urology group between May 1, 2017 and December 1, 2020. Appointment data from 17 providers were included. Data were collected on diagnosis, age, gender, insurance type, nonattendance history, lead time between scheduling and appointment, clinic location and outpatient procedures vs general office visit or telehealth visit. RESULTS: A total of 7,592 (9.0%) appointments were missed. Patients seen for oncologic diagnosis had the lowest missed appointment rate (4.5%), as compared with benign urology (9.6%) and pediatrics (13.0%). Previous nonattendance history within the last year was associated with nonattendance again (OR 2.47, 95% CI 2.29-2.66). Patients with Medicaid had the highest rate of missed appointments (17.2%; OR 2.16, 95% CI 2.02-2.32). Increased lead time between appointments increased the odds of nonattendance (OR 1.018/week, CI 1.016-1.020). Patients undergoing procedures had the lowest nonattendance rate (3.4%), compared with both new (11.4%) and followup (10.5%) visits, while both telephone (2.9%; OR 0.41, 95% CI 0.32-0.53) and video (2.8%; OR 0.37, 95% CI 0.20-0.71) visits had lower rates of nonattendance when compared to in-person visits. CONCLUSIONS: We found a nonattendance rate of 9% in our practice. Those patients with oncologic diagnoses and those having procedures or telemedicine visits had the lowest rates. Those who have missed an appointment in the past are at the highest risk nonattendance and should be targeted to improve patient health as well as practice efficiency.

Readability Metrics of Provider Postoperative Handouts in Urology.

OBJECTIVE: To examine the readability of postoperative urology handouts and assess for areas of improvement. We hypothesize that the majority of provider handouts exceed the National Institutes of Health recommendation of writing at a sixth-grade reading level. METHODS: We reviewed 238 postoperative patient handouts in the public domain representing United States academic and private practices. All handouts were categorized and re-formatted into text-only using Microsoft Word. A median reading grade was calculated using the Readability.io web application using Flesch-Kincaid Grade Level, Gunning Fog index, Coleman-Liau index, Simple Measure of Gobbledygook, and Automated-Reading Index. Word count was also assessed. RESULTS: Provider handouts were written at a median 9.3 grade reading level (range 5.8-14, IQR 8.45-10). A total of 15 (6.8%) handouts were written at a sixth-grade reading level, with only 1 (0.4%) handout written below the target. Six (2.7%) handouts were written at college-level. There were no significant differences between different subspecialties. Median word count was 509 (range 90-3796, IQR 361-738). Although a high word count may make it more difficult for patients to follow suggestions, the readability of each handout did not correlate with word count. CONCLUSIONS: Our data show that over 93% of analyzed handouts failed to meet National Institutes of Health recommendations for grade level. Longer word counts did not correlate with higher reading levels. It will be important to assess patient satisfaction with handouts and to correlate the complexity of postoperative handouts with outcome, such as unplanned phone calls and unscheduled visits.

Gender identity: A psychosocial primer for providing care to patients with a disorder/difference of sex development and their families [individualized care for patients with intersex (Disorders/differences of sex development): Part 2].

INTRODUCTION/BACKGROUND: Many parents of infants born with a DSD describe the process of initial sex assignment at birth as highly stressful. Parents of children with a DSD also note high distress when their children engage in behaviors that are not considered typical for their gender. OBJECTIVE: The goal of this article is to provide members of the health care team a brief overview of psychosocial facets of gender and gender identity particularly relevant to DSD for the purposes of enhancing shared decision-making and optimizing support for individuals with a DSD and their families. DISCUSSION: Gender identity is a multidimensional construct involving related but distinct concepts such as gender typicality, gender contentedness and felt pressure for gender differentiation, and can be assessed via standardized measures. Gender dysphoria is associated with poor psychological adjustment, and is mitigated by family and peer support. Family influences on gender identity include parental modeling of gender behavior and family composition (e.g., same-sex children vs both sons and daughters in a family). Cultural factors that may influence sex assignment include societal views on gender, and gender-related differential resource allocation within a society. In addition, religious beliefs and the presence of a "third-sex" category within a culture may also influence parental gender ideology. CLINICAL APPLICATION: Health care providers who work with patients with a DSD must have a strong grasp on the construct of gender identity, and must be able to clearly and consistently communicate with patients and families about gender beliefs in order to optimize family support and gender-related decisions.

Individualized care for patients with intersex (disorders/differences of sex development): Part 3.

The focus of this article is to review the complex determinants of gender assignment in a child with a disorder of sex development using four different clinical cases. While the care of patients with DSD may be shared across several specialties and opinions regarding their management may vary, this may be further complicated by psychosocial, cultural and economic factors. In this regard, access to behavioral health specialists with experience and specialization in the treatment of patients with DSD should be a foundational component of the standard of care and can greatly assist in the complex decision-making regarding gender assignment. We recommend an individualized approach by a multidisciplinary team utilizing a range of evolving strategies, including outcome data (or lack thereof) to support families during the decision-making process.

Individualized care for patients with intersex (disorders/differences of sex development): part I.

The care of individuals with disorders/differences of sex development aims to enable affected individuals and their families to have the best quality of life, particularly those born with severe genital ambiguity. Two of the biggest concerns for parents and health professionals are: (1) making a gender assignment and (2) the decisions of whether or not surgery is indicated, and if so, when is best for the patient and parents. These decisions, which can be overwhelming to families, are almost always made in the face of uncertainties. Such decisions must involve the parents, include multidisciplinary contributions, have an underlying principle of full disclosure, and respect familial, philosophical, and cultural values. Assignment as male or female is made with the realization that gender identity cannot be predicted with certainty. Because of the variability among those with the same diagnosis and complexity of phenotype-genotype correlation, the use of algorithms is inappropriate. The goal of this article is to emphasize the need for individualized care to make the best possible decisions for each patient's unique situation.

Robot set-up time in urologic surgery: an opportunity for quality improvement.

INTRODUCTION: Robotic-assisted techniques are widespread in urology. However, prolonged preparation time for robotic cases hinders operating room (OR) efficiency and frustrates robotic surgeons. Pre-operative times are an opportunity for quality improvement (QI) and enhancing OR throughput. We have previously shown that pre-operative times in robotic cases are highly variable and that increasing patient complexity was associated with longer times. Our objective was to characterize set-up times in robotic urology cases and to determine whether prolongation was due to robot set-up, in particular. MATERIALS AND METHODS: Patients undergoing robotic-assisted urology procedures at our academic institution had routine peri-operative collection of demographic data and OR time stamps. Following IRB approval, we retrospectively reviewed set-up times from an OR database. Multivariable analysis was used to assess the influence of independent patient variables-gender (M/F), smoking history, age, BMI, American Society of Anesthesiologists (ASA) Physical Status Classification, and Charlson Comorbidity Index (CCI)-on robot set-up times. Institutional factors including procedure, surgeon, and case year were also assessed. RESULTS: A total of 808 patients undergoing 816 robotic-assisted procedures from 2013 to 2018 met inclusion criteria. Robot set-up times varied only by gender (F > M) but not by general patient complexity. Age, BMI, smoking status, ASA, and CCI did not play a role in prolonging robot set-up times. There was marked variability of robot set-up times, even within procedure type. Robot set-up times generally improved over time for a given surgeon. CONCLUSIONS: Robot set-up time is not affected by patient complexity, in contrast to pre-operative time. It is affected by procedure type and does improve with experience. There is wide variability of robot set-up times and this is an important target for surgical QI.

Enhanced Recovery after Partial and Radical Nephrectomy Reduces Length of Stay, Opioid Use and Cost.

INTRODUCTION: Enhanced recovery after surgery pathways are multidisciplinary, multimodal approaches to perioperative care that aim to improve patient outcomes. In this study we evaluate the outcomes of the implementation of enhanced recovery after surgery pathways in patients undergoing nephrectomy. METHODS: A retrospective analysis was performed comparing patients who underwent renal surgery before vs after implementation of enhanced recovery after surgery pathways. Data analyzed included length of stay, opioid use, cost and complications before and after the enhanced recovery after surgery protocol was implemented. RESULTS: There were 76 patients in the pre-enhanced recovery after surgery group and 42 in the enhanced recovery after surgery group. Median length of stay in the pre-enhanced vs enhanced recovery after surgery group was 3 days vs 2 days (p <0.005). For open procedures median length of stay was 5 days vs 2 days (p <0.001). For robotic procedures median length of stay decreased from 3 days to 2 days (p <0.001). Median length of stay was lower in the enhanced recovery after surgery group independent of age, sex, body mass index, American Society of Anesthesiologists® score and anesthesia time. Median total morphine equivalents decreased from 4 mg to 0 mg (p <0.005) while median total oxycodone went from 52.5 mg to 8.75 mg (p <0.005). Direct cost per patient decreased from $13,036 pre-enhanced recovery after surgery to $9,779 (p <0.001) in the enhanced recovery after surgery group, representing a 25% decrease. The 30-day readmission rates did not change after implementation of enhanced recovery after surgery protocol, and a National Surgical Quality Improvement Program sampling showed similar rates in complications, although this was not amenable to statistical analysis. CONCLUSIONS: Enhanced recovery after surgery improves the care of patients undergoing renal surgery. It significantly decreased length of stay, opioid use and hospital cost without having a significant effect on complications.

The Role of Patient and Procedure Specific Factors in Urology Operating Room Perioperative Times.

INTRODUCTION: Although not traditionally examined, the nonoperative time a patient spends in the operating room is potentially significant. We determined the role of patient and procedure specific characteristics in nonoperative times in urology cases. METHODS: All patients at our tertiary institution had routine preoperative collection of patient and procedure specific data. Following institutional review board approval, we retrospectively reviewed the time landmarks of preoperative operating room time (the time from when the patient enters the room until the procedure starts) and postoperative operating room time (the time from the procedure end until the patient exits the room). Study inclusion criteria consisted of ASA™ (American Society of Anesthesiologists™) class I-IV and those cases with complete available data. Emergency cases (ASA score greater than 4) were excluded from analysis. Multivariable regression was used to assess the influence of patient and procedure variables on preoperative and postoperative operating room time. RESULTS: A total of 1,488 patients undergoing 1,786 urology procedures during a 9-month period (January to September 2016) met inclusion criteria. Following multivariable analysis, ASA class and Charlson comorbidity index were significantly associated with an increase in preoperative time. The only variable that had a significant association with preoperative and postoperative times was location (hospital vs ambulatory). Procedure type also had a significant effect on perioperative operating room times. CONCLUSIONS: Our analysis is a novel approach to assessing operating room efficiency by characterizing the nonoperative time a patient spends in the operating room. Robotic cases have longer nonoperative times and increasing patient complexity prolongs preoperative time in the operating room. Better preparation of complex cases preoperatively will allow better use of constrained operating room resources.

Early experience with shared medical appointments for penile problems.

We examined the use of shared medical appointments (SMA) for educating and counseling children with penile problems. Forty-eight families were seen over 4 months with 21 participating in the SMA group and 27 in the traditional group. Using a questionnaire to assess adequacy of education, there was no difference in the overall scores between groups with a mean of 6.64/7 in the SMA and 6.56/7 in the traditional setting. With the increasing demands on providers, an SMA offers a solution to caring for more patients with penile problems in an efficient manner without impacting family education and satisfaction.