Can Videourodynamics shape management in refractory pediatric lower urinary tract dysfunction?

INTRODUCTION: Bladder dysfunction, or more specifically lower urinary tract dysfunction (LUTD), remains a common reason for pediatric urology consultation, and the management of these patients is time consuming and frustrating for patients, families and providers alike. But what happens when the patient proves refractory to current treatment modalities? Is there a role for the use of videourodynamics (VUDS) to help guide therapy in the patient with refractory voiding dysfunction, and if so how might we select patients for this invasive study in order to increase the yield of useful information? OBJECTIVES: To determine the role, if any, for VUDS in the evaluation of pediatric patients with refractory LUTD and to identify parameters that might be used to select patients for this invasive study in order to increase the yield of useful information. STUDY DESIGN: Through our IRB-approved prospectively maintained urodynamics database, we retrospectively identified 110 patients with non-neurogenic LUTD over a period from 2015 to 2022 who underwent VUDS. We excluded patients with known neurologic or anatomic lesions and developmental delay. RESULTS: There were 76 females and 34 males (69%/31%) and their average age at the time of the study was 10.5 years ± 4 with a median age of 7.3 years. Patients had been followed for a mean of 5.9 ± 3.5 office visits prior to obtaining the VUDS and reported a mean Dysfunction Voiding and Incontinence Symptom Score (DVISS) of 15.6 ± 6.7 before the VUDS. VUDS resulted in a change in management in 86 of these 110 patients (78%). Management changes included a change in medication (53/110), consideration of CIC (11/110), PTENS (1/110) and surgery (14/110). As shown in the Figure, the DVISS score was significantly higher and the number of office visits prior to VUDS was significantly higher in the 86 patients whose management was changed versus the 24 patients in whom management did not change (P < 0.02). CONCLUSION: This retrospective analysis suggests that criteria for selecting these patients include: 1) long standing urinary incontinence that is refractory to biofeedback and medications, 2) ≥6 visits to LUTD clinic with no improvement, and 3) LUT symptom score of ≥16. Our findings suggest these criteria identify a cohort of patients in which a VUDS evaluation for the child with refractory LUTD can offer a more exact diagnosis that can shape management.

A Single Application of Cerebellar Transcranial Direct Current Stimulation Fails to Enhance Motor Skill Acquisition in Parkinson's Disease: A Pilot Study.

Parkinson's disease (PD) is a progressive neurodegenerative disorder that leads to numerous impairments in motor function that compromise the ability to perform activities of daily living. Practical and effective adjunct therapies are needed to complement current treatment approaches in PD. Transcranial direct current stimulation applied to the cerebellum (c-tDCS) can increase motor skill in young and older adults. Because the cerebellum is involved in PD pathology, c-tDCS application during motor practice could potentially enhance motor skill in PD. The primary purpose was to examine the influence of c-tDCS on motor skill acquisition in a complex, visuomotor isometric precision grip task (PGT) in PD in the OFF-medication state. The secondary purpose was to determine the influence of c-tDCS on transfer of motor skill in PD. The study utilized a double-blind, SHAM-controlled, within-subjects design. A total of 16 participants completed a c-tDCS condition and a SHAM condition in two experimental sessions separated by a 7-day washout period. Each session involved practice of the PGT concurrent with either c-tDCS or SHAM. Additionally, motor transfer tasks were quantified before and after the practice and stimulation period. The force error in the PGT was not significantly different between the c-tDCS and SHAM conditions. Similarly, transfer task performance was not significantly different between the c-tDCS and SHAM conditions. These findings indicate that a single session of c-tDCS does not elicit acute improvements in motor skill acquisition or transfer in hand and arm tasks in PD while participants are off medications.

Who needs an ultrasound? Using patient symptom questionnaire & UTI history to determine when to obtain an RBUS in children with non-neurogenic lower urinary tract dysfunction.

INTRODUCTION: Non-neurogenic lower urinary tract dysfunction (LUTD) is one of the most common reasons for presentation to a pediatric urologist, affecting up to 20% of children. Predicting who will benefit from RBUS as part of their work-up is challenging as the majority will have normal imaging. OBJECTIVE: Our objective was to assess the utility of using the Dysfunctional Voiding and Incontinence Scoring System (DVISS) and urinary tract infection (UTI) history to predict which LUTD patients were most likely to have an abnormal RBUS as well as determine a DVISS cutoff to aid in making this prediction. We hypothesized that higher DVISS scores and a positive urinary tract infection (UTI) history would be associated with increased likelihood of RBUS abnormality. STUDY DESIGN: We retrospectively reviewed outpatients seen for LUTD from 5/2014-1/2016 who received an RBUS. Association between prior UTI, DVISS score, gender, and race and RBUS abnormality were evaluated using logistic regression analysis. Receiver operating characteristic (ROC) curves were created to evaluate the predictive model and a Youden index calculated to determine the optimal cutoff for DVISS score to predict abnormal RBUS. RESULTS: 15 of 333 patients (4.5%) had a clinically significant RBUS abnormality. Significantly more patients with abnormal RBUS had a positive UTI history and median DVISS was higher. UTI history and DVISS score were associated with RBUS abnormality whereas neither gender nor race were. A DVISS score cutoff of 12 was determined to be ideal for predicting abnormal imaging. Using DVISS≥12 and positive UTI history, patients with both risk factors were significantly more likely to have an abnormal RBUS than those with zero or one risk factor (Figure). DISCUSSION: To the best of our knowledge this is the first study to try to identify risk factors associated with RBUS abnormality in pediatric LUTD patients and create an evidence-based approach to imaging these patients. We found both DVISS cutoff ≥12 and positive UTI history to be useful to risk stratify LUTD patients' likelihood of abnormal RBUS. Limitations include the study's retrospective nature as well as the fact the population was drawn from a tertiary care pediatric hospital with a large referral population and the fact that the decision to order an RBUS was based on individual clinician preference and decision making. CONCLUSIONS: We found that DVISS score≥12 and UTI history are useful in guiding the decision to obtain RBUS in pediatric LUTD patients.

Does Preoperative Testosterone Administration Decrease Complications in Distal Hypospadias Repair With Urethroplasty?

PURPOSE: Testosterone administration prior to hypospadias repair is common practice among pediatric urologists; however, its impact on surgical outcomes remains controversial. We hypothesize that testosterone administration prior to distal hypospadias repair with urethroplasty significantly decreases postoperative complications. MATERIALS AND METHODS: We queried our hypospadias database for primary distal hypospadias repairs with urethroplasty from 2015 to 2021. Patients undergoing repair without urethroplasty were excluded. We collected information on patient age, procedure type, testosterone administration status, initial visit and intraoperative glans width, urethroplasty length, and postoperative complications. To determine the role of testosterone administration on incidence of complications, a logistic regression adjusting for initial visit glans width, urethroplasty length, and age was performed. RESULTS: A total of 368 patients underwent distal hypospadias repair with urethroplasty. One hundred thirty-three patients received testosterone and 235 did not. Initial visit glans width was significantly larger in the no-testosterone vs testosterone group (14.5 mm vs 13.1 mm, P = .001). Testosterone patients had significantly larger glans width at the time of surgery (17.1 mm vs 14.6 mm [no-testosterone group], P = .001). On multivariable logistic regression analysis after controlling for age at surgery, preoperative glans width, testosterone status, and urethroplasty length, testosterone administration did show significant association with reduced odds of postoperative complications (OR 0.4, P = .039). CONCLUSIONS: This retrospective review of patients shows that on multivariable analysis there is significant association between testosterone administration and decreased incidence of complications in patients undergoing distal hypospadias repair with urethroplasty. Future studies on testosterone administration should focus on specific cohorts of patients with hypospadias as benefits of testosterone may be more evident in some subgroups than others.

Caudal anesthesia is not associated with post-operative complications following distal hypospadias repair.

INTRODUCTION: The use of caudal anesthesia at the time of hypospadias repair remains controversial as some prior studies have reported increased postoperative complication rates associated with caudal nerve block. However, these results have been called into question due to confounding factors and heterogeneous study groups. OBJECTIVE: Given the importance of identifying true risk factors associated with increased hypospadias complication rate, we examined our experience with caudal anesthesia limiting our analysis to distal repairs. We hypothesized that caudal anesthesia would not be associated with increased postoperative complications. STUDY DESIGN: We retrospectively reviewed our institutional hypospadias database from June 2007 to January 2021. All boys who underwent single-stage distal hypospadias repair with either caudal or penile block with minimum 1 month follow up were included. Records were reviewed to determine the type of local anesthesia, type of hypospadias repair, all complications, and time to complication. Association between any complication and local anesthesia type was evaluated by univariate and multivariate logistic regression analysis controlling for age at surgery and type of repair. A sub-analysis was performed for complications occurring ≤30 days. RESULTS: Overall, 1008 boys, 832 (82.5%) who received caudal and 176 (17.5%) penile block, were included. Median age at surgery was 8.1 months and median follow up was 13 months. Overall complication rate was 16.4% with 13.8% of patients requiring repeat operation. Median time to complication was 10.59 months and was significantly shorter in the caudal group (8.45 vs. 25.2 months). Caudal anesthesia was associated with higher likelihood of complication on univariate analysis; however, this was not true on multivariate analysis when controlling for age and type of repair. Caudal anesthesia was not associated with increased likelihood of complication within 30 days. DISCUSSION: Since the association between caudal anesthesia and hypospadias complications was first suggested, several studies have tried to answer this question with variable results. Our findings add to the evidence that there is no association between caudal anesthesia and increased hypospadias complications in either the short or long term. The major strengths of our study are a large, homogenous study population, robust follow up and inclusion of data from 14 surgeons over 14 years. Limitations include the study's retrospective nature as well as lack of standardized follow up protocol throughout the study period. CONCLUSIONS: After controlling for possible confounders, caudal nerve block was not associated with increased risk of postoperative complications following distal hypospadias repair.

Deferring gonadectomy in patients with turner syndrome with a genetic Y component is not a safe practice.

INTRODUCTION: Patients with Turner syndrome who harbor Y chromosome material are known to be at increased risk of developing germ cell neoplasms. The optimal timing to perform gonadectomy to reduce the risk of cancer development in these patients is not well defined. We present outcomes of Turner with a Y component (TSY) patients who underwent gonadectomy at our institution. HYPOTHESIS/OBJECTIVE: We hypothesized that tumors could occur in a significant portion of TSY patients at any age and gonadectomy can be safely performed at diagnosis rather than deferred. STUDY DESIGN: We performed an IRB-approved retrospective single center study in which we queried our institutions electronic health record to identify all patients with TSY who underwent gonadectomy at our institution from 2012 to 2021. RESULTS: In our series of 18 consecutive TSY patients, a tumor was identified in 6 patients (33.3%): 4 (22.2%) with dysgerminoma (DG) [Fig. 1] and 2 (11.1%) with gonadoblastoma (GB). DISCUSSION: Our cohort of 18 consecutive TSY who underwent gonadectomy over a 9-year period is the largest published single site cohort to date. Additionally, our patient who was found to have GB at 40 days is to our knowledge the youngest TSY patient to be diagnosed with GB in the literature. This patient's remarkably early incidence of tumor occurrence illustrates the urgency of protective gonadectomy. Given the high incidence of tumor formation in this population and the minimal morbidity associated with gonadectomy, we do not recommend delaying gonadectomy in this population for any reason. Our study is vulnerable to selection bias and confounding innate to any retrospective study. There was variation with respect to the frequency and timing of pre-operative imaging as a strict preoperative imaging protocol with sequential studies was not in place at our institution. Additionally, we do not have a comparison cohort of patients who are being followed without operative intervention as all TSY patients at our institution have undergone gonadectomy. CONCLUSION: TSY patients cannot be safely observed for tumor formation based on clinical factors such as imaging or age. Gonadectomy is safe with a low complication rate and without tumor recurrence during three-year follow-up. We continue to recommend bilateral gonadectomy in this patient population at the time of diagnosis.

Evolving epidemiology of transthyretin amyloid cardiomyopathy due to increased recognition in women.

BACKGROUND: Transthyretin amyloid cardiomyopathy (ATTR-CM), particularly wild type (wtATTR-CM), is thought to mainly affect men. Non-invasive diagnosis and approved therapeutics have been associated with increased disease recognition. We investigated the trajectory of ATTR-CM diagnosis in women. METHODS: This observational study utilized data collected on 140 consecutive ATTR-CM patients diagnosed between 2005 and 2022 who are followed at the Oregon Health and Science University Amyloidosis Clinic. Subgroup analysis was performed on patients with wtATTR-CM which included 113 subjects (80.1%). The proportion of women among patients diagnosed with ATTR-CM prior to 2019 was compared with that of those diagnosed 2019-2022 (2019 was the year of tafamidis approval by the FDA). The clinical characteristics of male and female ATTR-CM patients were compared as well. RESULTS: Of the 140 ATTR-CM patients, 16 (11.4%) were women (age 77 ± 9 years) and 124 (88.6%) were men (age 76 ± 9 years). There was an increase in the rate of women diagnosed with ATTR-CM from pre 2019 to 2019-2022 in the overall cohort (4/68 [5.9%] vs 12/72 [16.7%]) and wild type subgroup (0/51 [0%] vs 7/62 [11.3%]). There were several differences in baseline clinical characteristics between women and men in this cohort, yet all women had a clear clinical phenotype of ATTR-CM. CONCLUSIONS: There has been a significant increase in the rate of wtATTR-CM diagnoses in women, who presented with clear phenotypes of ATTR-CM. Further studies are needed to understand the effect of increased recognition of ATTR-CM in women on disease epidemiology, natural history, and outcomes.

Deep learning imaging features derived from kidney ultrasounds predict chronic kidney disease progression in children with posterior urethral valves.

BACKGROUND: We sought to use deep learning to extract anatomic features from postnatal kidney ultrasounds and evaluate their performance in predicting the risk and timing of chronic kidney disease (CKD) progression for boys with posterior urethral valves (PUV). We hypothesized that these features would predict CKD progression better than clinical characteristics such as nadir creatinine alone. METHODS: We performed a retrospective cohort study of boys with PUV treated at two pediatric health systems from 1990 to 2021. Features of kidneys were extracted from initial postnatal kidney ultrasound images using a deep learning model. Three time-to-event prediction models were built using random survival forests. The Imaging Model included deep learning imaging features, the Clinical Model included clinical data, and the Ensemble Model combined imaging features and clinical data. Separate models were built to include time-dependent clinical data that were available at 6 months, 1 year, 3 years, and 5 years. RESULTS: Two-hundred and twenty-five patients were included in the analysis. All models performed well with C-indices of 0.7 or greater. The Clinical Model outperformed the Imaging Model at all time points with nadir creatinine driving the performance of the Clinical Model. Combining the 6-month Imaging Model (C-index 0.7; 95% confidence interval [CI] 0.6, 0.79) with the 6-month Clinical Model (C-index 0.79; 95% CI 0.71, 0.86) resulted in a 6-month Ensemble Model that performed better (C-index 0.82; 95% CI 0.77, 0.88) than either model alone. CONCLUSIONS: Deep learning imaging features extracted from initial postnatal kidney ultrasounds may improve early prediction of CKD progression among children with PUV. A higher resolution version of the Graphical abstract is available as Supplementary information.

Impact of Race, Gender, and Socioeconomic Status on Symptom Severity at Time of Urologic Referral.

BACKGROUND AND OBJECTIVES: The Dysfunctional Voiding and Incontinence Scoring System (DVISS) is a validated tool to evaluate lower urinary tract dysfunction (LUTD) severity in children. DVISS provides a quantitative score (0-35) including a quality-of-life measure, with higher values indicating more/worse symptoms. Clinically, variability exists in symptom severity when patients present to pediatric urology with LUTD. We hypothesized that symptom severity at consultation varied based on race, gender, and/or socioeconomic status. METHODS: All urology encounters at a single institution with completed modified DVISS scores 6/2015-3/2018 were reviewed. Initial visits for patients 5-21 years old with non-neurogenic LUTD were included. Patients with neurologic disorders or genitourinary tract anomalies were excluded. Wilcoxon rank sum tests compared scores between White and Black patients and between male and female patients. Multiple regression models examined relationships among race, gender, estimated median household income, and insurance payor type. All statistics were performed using Stata 15. RESULTS: In total, 4086 initial patient visits for non-neurogenic LUTD were identified. Median DVISS scores were higher in Black (10) versus White (8) patients (p < 0.001). Symptom severity was higher in females (9) versus males (8) (p < 0.001). When estimated median income and insurance payer types were introduced into a multiple regression model, race, gender, and insurance payer type were significantly associated with symptom severity at presentation. CONCLUSIONS: Race, gender, and socioeconomic status significantly impact LUTS severity at the time of urologic consultation. Future studies are needed to clarify the etiologies of these disparities and to determine their clinical significance.

Multi-institutional Validation of Improved Vesicoureteral Reflux Assessment With Simple and Machine Learning Approaches.

PURPOSE: Vesicoureteral reflux grading from voiding cystourethrograms is highly subjective with low reliability. We aimed to demonstrate improved reliability for vesicoureteral reflux grading with simple and machine learning approaches using ureteral tortuosity and dilatation on voiding cystourethrograms. MATERIALS AND METHODS: Voiding cystourethrograms were collected from our institution for training and 5 external data sets for validation. Each voiding cystourethrogram was graded by 5-7 raters to determine a consensus vesicoureteral reflux grade label and inter- and intra-rater reliability was assessed. Each voiding cystourethrogram was assessed for 4 features: ureteral tortuosity, proximal, distal, and maximum ureteral dilatation. The labels were then assigned to the combination of the 4 features. A machine learning-based model, qVUR, was trained to predict vesicoureteral reflux grade from these features and model performance was assessed by AUROC (area under the receiver-operator-characteristic). RESULTS: A total of 1,492 kidneys and ureters were collected from voiding cystourethrograms resulting in a total of 8,230 independent gradings. The internal inter-rater reliability for vesicoureteral reflux grading was 0.44 with a median percent agreement of 0.71 and low intra-rater reliability. Higher values for each feature were associated with higher vesicoureteral reflux grade. qVUR performed with an accuracy of 0.62 (AUROC=0.84) with stable performance across all external data sets. The model improved vesicoureteral reflux grade reliability by 3.6-fold compared to traditional grading (P < .001). CONCLUSIONS: In a large pediatric population from multiple institutions, we show that machine learning-based assessment for vesicoureteral reflux improves reliability compared to current grading methods. qVUR is generalizable and robust with similar accuracy to clinicians but the added prognostic value of quantitative measures warrants further study.

Trends in opioid and nonsteroidal anti-inflammatory (NSAID) usage in children undergoing common urinary tract reconstruction: A large, single-institutional analysis.

INTRODUCTION AND OBJECTIVE: Opioid stewardship is recognized as a critical clinical priority. We previously reported marked reductions in narcotic administration after implementation of an opioid reduction protocol for pediatric ambulatory urologic surgery. We hypothesize that a decrease in post-operative and discharge opioid administration will not increase short-term adverse events. STUDY DESIGN: All pediatric patients undergoing open or robot-assisted laparoscopic pyeloplasty or ureteral reimplantation between 2015 and 2019 were included. Patients' demographics, opioid and NSAID administration, urology or pain-related emergency department (ED) visits, readmissions, and reoperations within 30 days of surgery, were aggregated. RESULTS: 438 patients, with a median age of 3.5 years (IQR 1.5-8.3) at the time of surgery, met the inclusion criteria. Annual rates of inpatient opioid administration and prescriptions decreased significantly over the study period, while rates of intra-operative, inpatient, and prescribed NSAIDs significantly increased. There was no significant difference in the occurrence of ED visits, readmissions, or reoperations within 30 days of surgery between patients who received an opioid prescription and those who did not. Multivariate regression showed that patients who did not receive an opioid prescription at discharge were found to be at a lower risk for unplanned encounters including ED visits, readmissions, or reoperations (OR:0.5, 95%CI: 0.2-0.9, p = 0.04). DISCUSSION: The present study shows the decreasing trend in inpatient opioid administration and opioid prescription after discharge, when accompanied by an increase NSAID administration, does not result in a significant change in rates of unplanned encounters and complications, similar to results from previous studies on non-urological and ambulatory urological surgeries. CONCLUSIONS: Non-opioid pain control after major pediatric urologic reconstruction is safe and effective. We found that a reduction in opioid administration can be associated with a reduced risk of unplanned ED visits, readmissions, or reoperations. Further investigations are required to corroborate this finding.

Thoracic spinal arachnoid cyst in a pediatric patient presenting with isolated bladder and bowel incontinence.

Spinal arachnoid cysts (SAC) are uncommon benign spinal cord lesions, particularly in children, that can result in a variety of neurologic symptoms, including neurogenic bladder. Here we present the case of a 7-year-old female with new onset, isolated urinary and stool incontinence who was found to have a T4-T7 SAC. Though this was initially believed to be an incidental imaging finding, after thorough work-up and persistence of her symptoms despite conservative measures she underwent neurosurgical intervention with complete resolution of incontinence. SAC represents a very rare but potentially reversible cause of neurogenic bladder that the urologist should be aware of.

Incidence and resolution of de novo hydronephrosis after pediatric robot-assisted laparoscopic extravesical ureteral reimplantation for primary vesicoureteral reflux.

INTRODUCTION: With the advent of robot-assisted laparoscopic ureteral reimplantation (RALUR) for primary vesicoureteral reflux (VUR), understanding and minimizing its complications continues to be critical. Incidence of de novo hydronephrosis after RALUR could be indicative of an outcome that needs further study or could be a benign transient finding. OBJECTIVE: In the present study, we hypothesized that the incidence of de novo hydronephrosis after RALUR is low and resolves spontaneously. METHODS: An IRB-approved prospective single-institutional registry was utilized to identify all patients undergoing RALUR via an extravesical approach from 2012 to 2020. Patients with primary VUR and minimal (Grade I SFU) or no hydronephrosis prior to surgery were included. Patients who had other associated pathology or underwent concomitant procedures were excluded. Preoperative characteristics including VUR and hydronephrosis grades as well as post-operative clinical details and hydronephrosis grades were aggregated and analyzed. RESULTS: 86/172 (50%) patients (133 ureters), with median reflux grade of 3 (IQR: G2, G3) met the inclusion criteria. Patients underwent RALUR at a median age of 5.7 years (IQR: 3.4, 8.7) with median 36.2 months (IQR: 19.6, 63.6) follow-up. Renal ultrasound at 4-6 weeks post-op showed de novo hydronephrosis in 18 (13.5%) ureters; the complete resolution was seen in 13 ureters (72%) at a median of 4.5 months from surgery. Among the 5 with non-resolved hydronephrosis (SFU G2:4, G3:1), 2 patients (3 ureters) underwent subsequent interventions. DISCUSSION: The present study evaluating the natural history of de novo hydronephrosis after RALUR-EV performed for primary VUR, is to our knowledge the largest cohort of patients undergoing RALUR that this has been studied in. In our cohort, the incidence of de novo hydronephrosis after RALUR was 13.5%, similar to rates reported in two OUR cohorts, and significantly lower than reported incidence rates of 22-26% in several OUR cohorts, and 30% in a RALUR cohort. In the present cohort, hydronephrosis resolved spontaneously in more than 72% of cases. The median time from surgery until resolution of hydronephrosis was 4.5 (1.6, 10.5) months, which is shorter in comparison to the average time to resolution of 7.6 months, reported by Kim et al. in an earlier study. CONCLUSIONS: De novo hydronephrosis after RALUR can be followed with serial renal ultrasounds. The majority of de novo hydronephrosis post-RALUR is transient and resolves spontaneously within a year of surgery with a very low re-intervention rate.

Intrascrotal Lipoblastoma: A Rare Cause of Paratesticular Mass in a Pediatric Patient.

There is a broad differential diagnosis for a pre-pubertal child presenting with a scrotal mass including both benign and malignant etiologies. Lipoblastomas are rare benign neoplasms originating from fat cells that occur most commonly on the trunk or extremities of young children. There have been less than 20 cases of scrotal lipoblastomas reported in the literature, with the majority occurring in children less than 3 years of age. Here we present a unique case of an 18-month male presenting with a paratesticular mass found to be a lipoblastoma on final pathology.

Office Based Pediatric Urologic Procedures: A Safe and Effective Alternative to Interventions Under Anesthesia.

OBJECTIVE: To report on our experience performing office-based pediatric urologic procedures. We hypothesize that office-based interventions are safe and effective for children, avoiding unnecessary risk and cost associated with general anesthesia. METHODS: We retrospectively identified patients undergoing office-based interventions from 2014 to 2019, including lysis of penile or labial adhesions, division of skin bridges, meatotomy and excision of benign lesion. Success was defined as a completed attempt in the office. Failure includes any unsuccessful office attempts. Complications include 30-day ED visits/readmissions and recurrent skin bridge post division of skin bridge. RESULTS: We identified 1326 interventions: 491 lyses of penile adhesions (37%), 320 division of skin bridges (24%), 128 lyses of labial adhesions (10%), 348 meatotomies (26%), and 39 excisions of benign lesions (3%) [Table 1]. There was a >95% success rate reported in every procedure with an overall complication rate of 0.6%. Excision of benign lesion had 100% success rate. ED visits within 30 days are rare (0.2%), and no patients required admission after their procedure [Table 2]. The rate of recurrence was highest following lysis of labial adhesions (13.3%). Of the 54 patients who underwent retreatment, very few required general anesthesia (n = 6). CONCLUSION: Office-based urologic interventions in children are well tolerated with excellent safety and efficacy. Complications and recurrence are universally low. Ultimately, 99.5% of this cohort was managed under local anesthetics, thereby avoiding the risks of anesthesia use in the pediatric population.

Routine ambulatory heart rhythm monitoring for detection of atrial arrhythmias in transthyretin cardiac amyloidosis.

BACKGROUND: Atrial fibrillation and flutter (AF/AFL) are common in transthyretin cardiac amyloidosis (ATTR-CM) which in turn is associated with higher risk of thromboembolism. Detecting AF/AFL may be especially important, but the role of routine ambulatory monitoring in ATTR-CM patients is unclear. OBJECTIVE: The objective is therefore to determine prevalence and outcomes of subclinical AF/AFL on routine ambulatory rhythm monitoring. METHODS: We report outcomes of an observational study of patients at our Amyloidosis Center with wild-type or variant ATTR-CM diagnosed between 2005 and 2019. Patients without known AF/AFL at baseline had ambulatory ECG monitoring (duration 2-30 days) every 6 months while those with cardiovascular implantable electronic devices (CIEDs) had device interrogations instead. RESULTS: Eighty-four patients with ATTR-CM (mean age 73.5 ± 9.7 years, 94% male) had mean follow-up 2.3 ± 1.9 years. Forty patients (48%) had AF/AFL before ATTR-CM diagnosis. In the remainder, 21 (48%) were subsequently diagnosed with AF/AFL: 10 (48%) based on symptoms, and 11 (52%) by monitoring. Anticoagulation (AC) was started in 9/11 (82%) patients with incidental AF/AFL. Among the entire cohort, stroke occurred in 9 patients (11%): 1 hemorrhagic and 8 ischemic (7 in patients with AF/AFL). No strokes occurred in patients on AC. CONCLUSION: Almost half of patients in our cohort had AF/AFL diagnosed prior to their ATTR-CM diagnosis. In the remainder, approximately half of AF/AFL diagnoses were established incidentally by routine monitoring, most of whom were promptly anticoagulated. Incidence of stroke was high overall, but no strokes occurred in anticoagulated patients. Optimal frequency and duration of monitoring needs further investigation.

Navigating the Complex Web of Prescribing Amyloidosis Therapeutics: A Primer.

Advancement in the diagnosis and treatment of transthyretin amyloid cardiomyopathy has made great strides in recent years. Novel therapeutics for transthyretin amyloidosis such as tafamidis, patisiran, and inotersen have shown significant benefits in a not-so-rare disease but come with high listing price tags ranging from a quarter to more than a half million dollars per year. These costs create significant financial barriers for the majority of patients, especially those with existing Medicare insurance plans. Of 72 patients reviewed, 67% were Medicare beneficiaries. Financial assistance was explored for the majority, and 37 (51%) patients with Medicare Part D received financial assistance that reduced their copayments to $0. Only one-third of our patients were able to afford these medications without any forms of financial assistance. Of these patients, 4 (6%) had the highest copayments ranging from $13 000 to $15 000 per year. To navigate the complexities of prescribing and affordability in amyloidosis, a multidisciplinary team including a dedicated clinical pharmacist is crucial in guaranteeing patients' success to secure these novel therapeutics. In this article, we discuss our experiences with prescribing, acquiring insurance authorizations, and financing these life-saving medications based on patient-specific insurance plans and socioeconomic status.