Infarction of Paratesticular Inflammatory Myofibroblastic Tumor Mimicking Testicular Torsion.

This report describes a 14-year-old male with a rare paratesticular inflammatory myofibroblastic tumor that presented atypically with acute unilateral scrotal pain and swelling. This presentation, which raised suspicion for testicular torsion, contrasts with the typical presentation of a slow-growing scrotal mass. Scrotal exploration revealed an infarcted right testis, demonstrating this locally aggressive tumor can undergo vascular invasion and occlude testicular blood supply. Thus, inflammatory myofibroblastic tumor should be considered in the differential diagnosis when evaluating patients with acute scrotal pain suspicious for testicular infarction.

Salvage Surgery Rates for Pediatric Testicular Torsion: Comparison of the Pre- and Post-Quality Metric Eras.

PURPOSE: Pediatric testicular torsion is a urological emergency that requires timely intervention. In 2015, quality metrics for testicular torsion were implemented in the U.S. News & World Report "Best Children's Hospitals" rankings. Our study examines and compares testicular salvage surgery rates before and after the institution of national quality metrics from a multi-institutional database. MATERIALS AND METHODS: The Pediatric Health Information System® was surveyed for all testicular torsion encounters using ICD (International Classification of Diseases), Ninth and Tenth Revisions coding from 52 hospitals between January 2010 and December 2019. Patients <1 year and ≥18 years of age were excluded. Only hospitals that reported outcomes before and after quality scoring were included. Testicular salvage surgery was defined as patients having undergone orchiopexy without concomitant orchiectomy. Age, race, distance from hospital, household income and insurance status were compared. RESULTS: A total of 890 patients (551 pre-metric and 339 post-metric) from 38 hospitals were included. The testicular salvage surgery rate was 12.5% higher in the post-metric cohort (70.9% versus 58.4%). Hospital compliance to testicular torsion quality metrics increased from 62% in 2015 to 98% in 2019. Mean age, race, distance to hospital, household income, insurance status and use of ultrasound were not statistically different between pre- and post-metric cohorts. CONCLUSIONS: Since the implementation of quality metrics, salvage surgery rates for testicular torsion increased to 70.9%, an improvement of 12.5% when compared to pre-metric outcomes. Patient factors were similar between the 2 groups. Multi-institutional prospective data are needed to validate this database study and evaluate overall testicular salvage rate.

Infantile Primary Hyperoxaluria Type 1 Treated With Lumasiran in Twin Males.

Primary hyperoxaluria type 1 (PH1) is a rare genetic disease that results in oxalate overproduction leading to nephrolithiasis (NL), nephrocalcinosis (NC), kidney failure, and systemic oxalosis. Infantile PH1 is its most severe form, and it may require intensive hemodialysis followed by a liver-kidney transplant. Lumasiran is an RNA interference (RNAi) therapeutic agent that reduces hepatic oxalate production, which has been recently approved for the treatment of PH1. In this report, we present a case of twin males with infantile PH1 and bilateral NL and NC who were treated with lumasiran at 12 months of age. Their symptoms abated after therapy was started without disease progression. To the best of our knowledge, this is the first report of PH1 occurring in twins and the first report on using lumasiran to treat infantile PH1 outside of a clinical trial. Lumasiran appears to be a successful therapeutic option for infantile PH1.

Ovotesticular Disorder of Sex Development: Approach and Management of an Index Case in the Dominican Republic.

Disorders of sex development (DSD) are a group of congenital conditions associated with anomalous development of internal and external genital organs. Ovotesticular disorder of sex development (OT-DSD) is a condition in which a child is born with both testicular tissue (that possesses variable fertility potential within seminiferous tubules) and ovarian tissue (with primordial follicles). These tissues may be co-existent in the same gonad (ovotestis) or independently in separate gonads. Here, we report the clinical case of a 21-month-old boy that we met during a humanitarian surgical mission performed at Hospital Dr. Francisco Moscoso Puello, Santo Domingo, Dominican Republic. The child was referred for management of hypospadias, cryptorchidism, and symptomatic right inguinal and umbilical hernias. With further chromosomal evaluation, the diagnosis of SRY-negative OT-DSD was made, and shared decision-making was used to determine the timing of gender assignment, reconstruction, and the child's long-term care team. OT-DSD is an uncommon condition with unclear causes. Once a DSD condition is suspected at birth, a complete investigation should be performed, encompassing a descriptive examination, a basic electrolyte and hormonal profile, genetic assessment, and pelvic ultrasound. Consultation with a multidisciplinary team is warranted, including pediatric urology or pediatric surgery with urologic training, endocrinology, genetics, psychology, pathology, and the patient's pediatrician at minimum before surgical reconstruction. It is crucial to involve the patient and their family with shared decision-making before surgery or gender assignment.

A government mandated consent safely reduces opioid utilization for major pediatric genitourinary surgeries.

INTRODUCTION: We describe the effect of a state mandated opioid consent on opioid utilization and multimodal pain use for major pediatric genitourinary surgeries. METHODS: All patients who underwent an inpatient pediatric genitourinary surgery at the Children's Hospital of Pittsburgh between August 2015 and February 2020 were identified. Inpatient and outpatient multimodal pain control utilization were assessed. Delayed prescriptions or emergency department visits within 30 days were identified and when applicable referenced against National Surgical Quality Improvement Program data. RESULTS: After accounting for a 6-month transition period following policy implementation, 462 major pediatric genitourinary procedures were identified. The frequency of discharge opiate prescriptions decreased from 68.4% prior to the transition period to 10.7% afterward(p<0.001). Morphine milligram equivalents per prescription decreased from 75(IQR 45-150) to 45(IQR 22.5-75)(p<0.001). The rate of delayed non-opioid analgesic prescriptions (6.6% vs 7.4%), delayed opioid prescriptions (1.5% vs 0.3%), or emergency department visits (11.8% vs 12.6%)(p = 0.809) remained unchanged. Data agreed with National Surgical Quality Improvement Program data in 98.5% of cases. After excluding one surgeon who departed after the transition period, regional block utilization did not change from 61/115(53%) to 147/320(45.9%)(p = 0.425). CONCLUSIONS: A state mandated opioid consent safely reduced opioid utilization for most major pediatric genitourinary surgeries. LEVEL OF EVIDENCE: Level III.

Government Mandated Consent Dramatically Reduces Pediatric Urologist Opioid Utilization for Outpatient and Minor Emergency Surgeries.

PURPOSE: Postoperative opioids are overprescribed in the United States. In November 2016 the State of Pennsylvania required an opioid consent for minors. Our hypothesis is that this mandate decreased postoperative opioid prescriptions in our division. MATERIALS AND METHODS: All patients who received a urological outpatient or minor emergency procedure from August 2015 to August 2019 were identified. Surgeries performed within 6 months after mandate implementation were excluded to account for the transition period. Perioperative data including case type were extracted by a clinical data warehouse from preexisting fields within the health record. The frequencies of postoperative prescriptions, delayed prescriptions and emergency department encounters were assessed. A multivariable logistic regression to identify predictors of opioid prescription at discharge was performed. RESULTS: A total of 4,349 patients were analyzed. The frequency of postsurgical opioid prescriptions decreased from 45.3% to 2.6% (p <0.001). The median morphine milligram equivalent decreased by 22.5 among children prescribed an opioid (p <0.001). Rates of an emergency department visits (3% vs 2.7%) or delayed nonopioid prescriptions (0.8% vs 1.2%) within 30 days of discharge were unchanged (p >0.05). Fewer patients received a delayed opioid prescription after mandate implementation (0.03% vs 0.5%, p <0.001). Female patients were less likely (OR 0.309, 95% CI 0.195-0.491; p <0.001) to receive opioids prior to but not after the mandate (OR 0.309, 95% CI 0.544-2.035; p=0.122). Increasing age was predictive of receiving an opioid before (OR 1.187, 95% CI 1.157-1.218; p <0.001) and after (OR 1.241, 95% CI 1.186-1.299; p <0.001) the mandate. CONCLUSIONS: A state mandated opioid consent for minors greatly reduced post-urological surgery opioid prescription rates without increasing rates of readmission or delayed prescriptions.

Point-of-care ultrasound is an accurate, time-saving, and cost-effective modality for post-operative imaging after pyeloplasty.

BACKGROUND: Dismembered pyeloplasty is considered the gold standard treatment for ureteropelvic junction obstruction (UPJO). Although the frequency and timing of follow up imaging after pyeloplasty is variable, renal ultrasound (RUS) is commonly utilized. With minimal training, point-of-care ultrasound (POCUS) can be easily performed by a urologist during a post-operative visit. OBJECTIVE: Our hypothesis is that POCUS is an accurate, time-saving, and cost-effective alternative to a complete retroperitoneal ultrasound (CRUS) performed by the Radiology Department after pyeloplasty. STUDY DESIGN: The clinical records of all children who underwent pyeloplasty (by any method) over a 12 month period at our institution were retrospectively reviewed. The exact timing and method (POCUS vs. CRUS) of follow up imaging was surgeon-dependent. Statistical analysis was performed to compare the time and cost of POCUS vs. CRUS. The clinical course of each patient who had each type of imaging was assessed. RESULTS: A total 45 patients were included in this analysis. Over a mean follow up period of 29 months, a total of 73 CRUS and 67 POCUS were performed. Each CRUS on average added 2 h to each patient's healthcare encounter. Had the 73 CRUS been performed as POCUS instead, this would have corresponded to $83,751 less charges to payers. There was no difference in the rate of the detection of worsening, stable, or improved hydronephrosis (HN) between either modality (p > 0.05). The recommended follow up time for observed HN was no different between CRUS and POCUS (p > 0.05). Children with worsening HN on POCUS underwent functional studies without confirmatory CRUS. Interestingly, two patients had metachronous, contralateral UPJO discovered during post-operative imaging. These were both discovered by POCUS. Nineteen (42%) patients who had attended at least one post-operative visit were eventually loss to follow-up. This occurred exclusively in those who did not have worsening ultrasound (p < 0.01). There was no difference in the loss to follow-up after POCUS (8) or CRUS (12) (p > 0.05). CONCLUSIONS: POCUS performed by a urologist is an accurate assessment of HN after pyeloplasty with time and cost savings to compared to a CRUS performed by a radiologist. POCUS is not associated with any difference in rate of detection of worsening HN or rate of loss to follow up.

Optimizing Opioid Pain Medication Use After Vasectomy-A Prospective Study.

OBJECTIVE: To act as good stewards, urologists need to balance patient's pain requirements against the risk of narcotic abuse. MATERIALS AND METHODS: We prospectively consented subjects who underwent vasectomies. Procedural technique was not standardized. All subjects received hydrooxycodone/acetaminophen 5-325 mg tablets and Ibuprofen 800 mg tablets. The subjects were then contacted by phone 1-3 weeks after their procedure with a follow-up questionnaire. Data collected included age, weight, number of pills used and pills remaining, number of days pain medication used, need for additional medication, pain treatment satisfaction, disposal knowledge, and complications. RESULTS: A total of 76 subjects completed the study. Overall, 88.3% rated excellent pain treatment satisfaction with score ⩾4 (scale 1-5). No opioid medication was used by 18.2% of subjects, 33.8% used 1-5 tablets, and 24.7% used all 15 tablets. At the end, 9 subjects (11.7%) reporting needing more pain medication. Using Pearson correlation, younger age was significantly related to number of pills used. (P <.001) In total, 648 additional narcotic tablets were prescribed. In terms of disposal, 20 (25.9%) subjects disposed of extra medication, 14 (24.7%) used all medication, and 50.6% did not dispose of medication. Proper disposal technique was known by 50 (64.9%) subjects. CONCLUSION: Opioid medication use after vasectomy is variable though correlated with age. Clinicians should weigh the need versus potential abuse to determine the amount of tablets they are comfortable prescribing. Counseling and documentation on proper use and disposal of opioid medication is strongly encouraged.

Fibroepithelial Vaginal Polyps in a Newborn Female.

Discussion, diagnosis, and management of fibroepithelial polyps in a newborn female.

Stones in special situations.

There are several special situations in which urinary lithiasis presents management challenges to the urologist. An in-depth knowledge of the pathophysiology, unique anatomy, and treatment options is crucial in order to maintain good health in these patients. In this review, we summarize the current literature on the management of the following scenarios: bladder stones, stones in bowel disease, during pregnancy, in association with renal anomalies, with skeletal deformities, in urinary diversions, and in children.

Cystatin C-calculated Glomerular Filtration Rate-A Marker of Early Renal Dysfunction in Patients With Neuropathic Bladder.

OBJECTIVE: To asses if cystatin c-calculated glomerular filtration rate (GFR) can reveal chronic kidney disease (CKD) not detected by creatinine-based calculations in a larger prospective cohort of children with myelomeningocele (MMC). Wheelchair-bound MMC patients frequently have low muscle mass, and assessing renal deterioration based on creatinine-based GFR is imprecise. MMC patients are also at risk for end-stage renal disease. METHODS: Prospectively enrolled patients with MMC underwent annual serum creatinine and cystatin c testing. Anthropometric measurements were obtained from clinic visit. The modified (bedside) Schwartz formula for creatinine-based GFR and the Zappitelli cystatin C formula were utilized for calculation. The exclusion criteria were patients with reduced GFR (CKD stage 2) or chronic CKD (CKD stage 3 and greater); these patients were excluded from analysis on the premise that they had already been identified for closer renal monitoring. RESULTS: A total of 131 patients were included in the analysis. The median creatinine-based estimated GFR was 126.5 mL/min/1.73 m2 (range: 22-310). The median cystatin C-based estimated rate was 98.5 mL/min/1.73 m2 (range: 16-171), yielding an absolute median rate reduction of 30.2%. Using cystatin c-calculated GFR, CKD stage was upgraded from stage 1 to ≥2 in 34 patients (26%). CONCLUSION: In MMC patients with poor muscle mass, cystatin C-based GFR is more sensitive than creatinine-based GFR in detecting early CKD. In this high-risk population, serial cystatin C estimation is a valuable tool in identifying children who may benefit from early nephrology referral and intervention.

Beyond Ohdo syndrome: A familial missense mutation broadens the MED12 spectrum.

Intellectual disability (ID) is estimated to affect 1-3% of the general population and is a common reason for referrals to pediatric and adult geneticists, as well as neurologists. There are many genetic and non-genetic causes of ID; X-linked forms are identifiable through their characteristic inheritance pattern. Current testing methods have been able to identify over 100 genes on the X chromosome responsible for X-linked intellectual disability (XLID) syndromes. MED12 [MIM *300188] (mediator complex subunit 12) mutations have been linked to numerous XLID syndromes, including Lujan, FG, and Ohdo, and MED12 is included in many XLID panels. MED12 is located at Xq13.1 and its product has roles in transcriptional activation and repression. We describe two affected male siblings and their unaffected mother with a novel missense mutation in MED12, c.4147G>A (p.Ala1383Thr). The siblings share some features of Ohdo syndrome, including feeding difficulties, microcephaly, and speech delay. However, additional attributes such as hypertonia, eosinophilic esophagitis, penile chordee, and particular facial dysmorphisms depart sufficiently from individuals previously described such that they appear to represent a new and expanded phenotype. This case lends credence to the evolving theory that the subtypes of Ohdo, and perhaps other MED12 disorders, reflect a spectrum of characteristics, rather than distinct syndromes. As XLID panel testing and whole exome sequencing (WES) becomes a standard of care for affected males, further MED12 mutations will broaden the phenotype of these intriguing disorders and challenge clinicians to rethink the current diagnostic boundaries.

Complete penile amputation during ritual neonatal circumcision and successful replantation using postoperative leech therapy.

Circumcision is the most common surgical procedure in males in the United States, and minor complications are not uncommon. Major complications like partial penile amputations have been reported with successful replantation. Complete penile amputations in adult males have been described, and successful replantation has been reported with increasing success. We report a case of complete penile amputation at the penopubic junction using a Mogen clamp in a 7-day-old neonate with replantation using postoperative leech therapy. To our knowledge this is the first time leech therapy has been used postoperatively for neonatal penile amputation.

Cystatin C as a marker of early renal insufficiency in children with congenital neuropathic bladder.

PURPOSE: Due to decreased muscle mass in children with congenital neuropathic bladder there may be significant inaccuracy when using the creatinine based estimated glomerular filtration rate. Cystatin C is highly sensitive and specific for measuring changes in the glomerular filtration rate in children and in patients with muscle wasting conditions. We hypothesized that a cystatin C calculated glomerular filtration rate would be more sensitive than the standard creatinine based modified Schwartz equation to detect renal insufficiency in children with congenital neuropathic bladder. MATERIALS AND METHODS: We prospectively identified children with congenital neuropathic bladder at a multidisciplinary spina bifida clinic who underwent serum creatinine and serum cystatin C testing. Clinical history and anthropomorphic variables at the time of laboratory testing were catalogued. The creatinine based glomerular filtration rate was estimated using the modified (bedside) Schwartz formula and the cystatin C based rate was calculated using the Zappitelli cystatin C formula. RESULTS: Dual estimated glomerular filtration rate calculation was done in 69 children at a total of 74 patient encounters. Absolute creatinine was within age range normal limits in each patient, including 1 with chronic kidney disease stage 3A. The median creatinine based estimated glomerular filtration rate was 123 ml per minute/1.73 m(2) (range 58 to 229). The median cystatin C based estimated rate was 103 ml per minute/1.73 m(2) (range 47 to 144) for an absolute median rate reduction of 15.4%. Using cystatin C estimates chronic kidney disease stage was upgraded from stage 1 to 2 in 13 patients (18.8%). CONCLUSIONS: In children with neuropathic bladder the cystatin C estimated glomerular filtration rate is a better screening test for early renal insufficiency that is not detected by creatinine based rate calculations. To our knowledge it remains to be determined whether the cystatin C estimated glomerular filtration rate can ultimately improve the clinical outcome in this population.

Tamsulosin and spontaneous passage of ureteral stones in children: a multi-institutional cohort study.

PURPOSE: Tamsulosin is associated with increased passage of ureteral stones in adults but its effectiveness in children is uncertain. We determined the association between tamsulosin and the spontaneous passage of ureteral stones in children. MATERIALS AND METHODS: We performed a multi-institutional retrospective cohort study of patients 18 years or younger who presented between 2007 and 2012 with ureteral stones up to 10 mm and who were treated with tamsulosin or oral analgesics alone. The outcome was spontaneous stone passage, defined as radiographic clearance and/or patient report of passage. Subjects prescribed tamsulosin were matched with subjects prescribed analgesics alone, using nearest neighbor propensity score matching to adjust for treatment selection. Conditional logistic regression models were used to estimate the association between tamsulosin and spontaneous passage of ureteral stones, adjusting for stone size and location. RESULTS: Of 449 children with ureteral stones 334 were eligible for inclusion, and complete data were available for 274 patients from 4 institutions (99 receiving tamsulosin, 175 receiving analgesics alone). Following case matching, there were no differences in age, gender, weight, height, stone size or stone location between the 99 subjects prescribed tamsulosin and the 99 propensity score matched subjects prescribed analgesics alone. In the tamsulosin cohort 55% of ureteral stones passed, compared to 44% in the analgesics alone cohort (p=0.03). In multivariate analysis adjusting for stone size and location tamsulosin was associated with spontaneous passage of ureteral stones (OR 3.31, 95% CI 1.49-7.34). CONCLUSIONS: The odds of spontaneous passage of ureteral stones were greater in children prescribed tamsulosin vs analgesics alone.

Timing and predictors for urinary drainage in children with expectantly managed grade IV renal trauma.

PURPOSE: We determined which children sustaining blunt grade IV renal trauma are at greatest risk for failing nonoperative management and in what time frame they will likely present. MATERIALS AND METHODS: We retrospectively reviewed children presenting with nonvascular grade IV blunt renal trauma between 2003 and 2012. We compared characteristics on computerized tomography, reasons for intervention, type and timing of surgery, length of hospital stay and need for readmission between children undergoing early intervention (less than 72 hours after admission) and those managed conservatively (with any subsequent intervention undertaken more than 72 hours after admission). RESULTS: A total of 26 children were identified with nonvascular grade IV blunt renal trauma. Conservative management was attempted in 16 cases (62%). Seven of these patients (44%) required intervention (ureteral stent and/or percutaneous drain placement), with a mean time to intervention of 11 days. Collecting system clot and larger urinoma (1.45 cm in cases with successful and 4.29 cm in those with failed conservative management) significantly predicted failure of conservative management (p<0.05). Presence of dissociated renal fragments (57% vs 11%) and interpolar contrast extravasation (57% vs 0%) were increased in the early intervention group compared to the conservatively managed group (p>0.05), as was rehospitalization (43% vs 0%), mean length of stay (7.9 vs 5.4 days) and transfusion (14% vs 0%, p>0.05). CONCLUSIONS: Collecting system hematoma and urinoma size significantly predicted failure of conservative management, with a mean time to intervention of 11 days. Children with failed conservative management had a greater incidence of dissociated renal fragments and interpolar extravasation. Early identification of these patients may decrease hospital readmissions, length of stay and prolonged morbidity.

Metanephric adenofibroma.

A 10-year-old boy underwent a computed tomography (CT) scan for left flank pain following a fall. Imaging demonstrated a 5 cm left upper pole renal mass. Partial nephrectomy revealed metanephric adenofibroma, a benign stromal-epithelial tumor thought to represent a hyperdifferentiated, mature form of Wilms' tumor. We briefly discuss the histopathology and management of this rare tumor.