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.

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.

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.

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.

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.

Laparoscopic pyeloplasty for ureteropelvic junction obstruction in infants.

PURPOSE: Laparoscopic pyeloplasty and open pyeloplasty have comparable efficacy for ureteropelvic junction obstruction in pediatric patients. The role of laparoscopic pyeloplasty in infants is less well defined. We present our updated experience with laparoscopic pyeloplasty in children younger than 1 year. MATERIALS AND METHODS: We retrospectively reviewed the records of all 29 infants treated with transperitoneal laparoscopic pyeloplasty for symptomatic and/or radiographic ureteropelvic junction obstruction from May 2005 to February 2012. Patients were followed with renal ultrasound at regular intervals. Treatment failure was defined as the inability to complete the intended procedure, persistent radiographic evidence of obstruction and/or the need for definitive adjunctive procedures. RESULTS: Transperitoneal laparoscopic pyeloplasty was performed in 29 infants 2 to 11 months old (mean age 6.0 months) weighing 4.1 to 10.9 kg (mean ± SD 7.9 ± 1.6). Followup was available in all except 5 patients (median 13.9 months, IQR 7.7-23.8). Mean operative time was 245 ± 44 minutes. All cases were completed laparoscopically. Three postoperative complications were reported, including ileus, superficial wound infection and pyelonephritis. Two patients had persistent symptomatic and/or radiographic evidence of obstruction, and required reoperative pyeloplasty. The overall success rate was 92%. CONCLUSIONS: Laparoscopic pyeloplasty in infants remains a technically challenging procedure limited to select centers. Our early experience revealed a success rate comparable to that of other treatment modalities with minimal morbidity.

Cost analysis of pediatric robot-assisted and laparoscopic pyeloplasty.

PURPOSE: An increasing percentage of pediatric pyeloplasties are being performed with assistance of the da Vinci® Surgical System. A review of the recent literature shows decreased operative times and length of hospital stays when robotic procedures are performed, although there are few published data comparing the cost of pediatric robotic and pure laparoscopic pyeloplasty. We reviewed a representative sample of pyeloplasties performed at our institution and performed a cost analysis. MATERIALS AND METHODS: We retrospectively identified 23 robot-assisted and 23 laparoscopic pyeloplasties performed at our institution between August 2008 and April 2012. Total cost was calculated from direct and indirect costs provided by our billing department. RESULTS: Robotic procedures were shorter than pure laparoscopic procedures (200 vs 265 minutes, p <0.001) but there was no significant difference in the total cost of the 2 procedures ($15,337 vs $16,067, p <0.46). When compared to laparoscopic cases, subgroup analysis demonstrated decreased operative times (140 vs 265 minutes, p <0.00001) and total cost ($11,949 vs $16,067, p <0.0001) in robotic cases where stents were placed in an antegrade fashion. CONCLUSIONS: With widespread use the cost of robotic instrumentation may decrease, and experience may further shorten operative times. However, it currently remains to be seen whether robotic technology will become a cost-effective replacement for pure laparoscopy in the management of pediatric ureteropelvic junction obstruction.

Advances in the surgical pediatric urologic armamentarium.

The surgical armamentarium of the pediatric urologist has changed greatly in the past 2 decades on account of new technology and careful adaptation of minimally invasive techniques in children. Conventional laparoscopy, robotic-assisted laparoscopy, laparoendoscopic single-site surgery, and endourologic surgery have, to varying degrees, provided new approaches to urologic surgery in the pediatric population. This article reviews the technology and adaptations behind these recent advances as well as their current applications in management of urologic disease in children.

Ureteral ectopia inserting into ipsilateral epididymis resulting in inflammatory paratesticular tumor.

An 8-year-old male with a history of VACTERL association was found to have a paratesticular mass. The patient was treated successfully with a radical orchiectomy and found to have ureteral ectopia inserting into the ipsilateral epididymis, resulting in this paratesticular mass caused by inflammation of the epididymis. This is the only reported case in the English literature of an ectopic ureter inserting into the epididymis and presenting as a paratesticular tumor. In unclear cases of paratesticular, inflammatory appearing masses, inguinal exploration is warranted.

Penoscrotal edema and purpura in a 12-year-old boy: a case report and review of causes.

We report the case of a 12-year-old patient with previously diagnosed Crohn disease who presented with penile edema and purpura, with extension into the scrotum. Subsequent work-up including biopsy led to the diagnosis of extraintestinal Crohn disease, a rare manifestation in the genital region. Prompt treatment with steroids led to complete resolution of both penoscrotal edema and purpura. We describe our case, followed by a discussion of etiologies of penoscrotal edema and purpura as a review for the practicing pediatric urologist.

Annual endoscopy and urine cytology for the surveillance of bladder tumors after enterocystoplasty for congenital bladder anomalies.

PURPOSE: It is currently recommended that patients with congenital bladder anomalies managed by enterocystoplasty undergo annual surveillance with urine cytology and endoscopy. We reviewed our experience with this protocol and suggest modifications based on this experience. MATERIALS AND METHODS: A total of 65 patients 10 years or more after enterocystoplasty were placed on an annual surveillance protocol consisting of interval medical history, renal-bladder ultrasound, serum B12, electrolytes, creatinine, urinalysis, urine cytology and endoscopy. RESULTS: Of the 65 patients 50 (77%) with enterocystoplasty (ileal in 40 and colonic in 10) remain on the protocol. Median age at the initiation of surveillance was 28 years (range 24 to 40) with a median time from augmentation of 15 years (range 12 to 29). During the first 5 years of surveillance 26 of 250 cytology results (10.5%) were suspicious for cancer. Further evaluation revealed no evidence of malignancy. Specificity for cytology was 90% with unknown sensitivity. Of 250 surveillance endoscopic evaluations 4 lesions (1.6%) were identified and biopsied/removed. Pathological evaluation revealed 1 adenomatous polyp, 1 squamous metaplasia and 2 nephrogenic adenomas. Due to the low event rate and high cost routine cytology and endoscopy were discontinued after each patient completed 5 years of followup and annual evaluations were maintained. No tumors developed during the median surveillance interval of 15 years (range 12 to 20). Currently median patient age is 42 years (range 36 to 59) and median time since augmentation is 27 years (range 23 to 40). CONCLUSIONS: Due to the low incidence of malignancy, lack of proven benefit and enhanced cost containment we recommend that annual surveillance endoscopy and cytology be discontinued.

Vesicoureteral reflux in children with urachal anomalies.

OBJECTIVE: We report the largest known series of vesicoureteral reflux (VUR) in children with urachal anomalies (UA). METHODS: Two institutions' records were reviewed for children with UA (1951‒2007). RESULTS: Of 30 girls and 36 boys with UA (34 urachal cysts, 14 patent urachus, 10 urachal diverticula, 7 urachal sinuses, and 1 unknown), 57 (86%) underwent surgical resection or drainage. A voiding cystourethrogram was obtained in 22 (33%). VUR was demonstrated in 14 of the 22 children (64%), and rates were similar among the various types of UA. The median age with versus without VUR was not different (1.3 vs 1.7 years, P=0.97). Of 24 refluxing renal units, classification was grade≤3 in 71%, 4‒5 in 12%, and unspecified in 17%. Four children (26%) underwent ureteroneocystostomy and 10 observed patients resolved spontaneously. CONCLUSION: To our knowledge, this is the first series of VUR associated with UA. The increased incidence of VUR (64%) in this small subset of patients warrants prospective studies to determine if there is a positive correlation with UA. We believe thorough genitourinary and family histories are important when evaluating children with UA to help detect clinically significant VUR.

Ureteroarterial fistula treatment with open surgery versus endovascular management: long-term outcomes.

PURPOSE: Ureteroarterial fistulas can be treated with open vascular or percutaneous arterial stent placement. We compared the long-term outcomes of each treatment. MATERIALS AND METHODS: A single center, retrospective review of ureteroarterial fistulas (1996 to 2008) was performed. RESULTS: We identified 20 ureteroarterial fistulas in 19 patients. All patients had undergone extirpative surgery with pelvic radiation in 74% and long-term ureteral stents in 84%. At a mean followup of 15.5 months (range 1 to 99) survival was 53%. Of the 70% (14 of 20) treated with percutaneous endovascular iliac artery stenting or embolization, 2 patients later required open vascular graft and 12 were treated with long-term ureteral stenting. Of the 30% (6 of 20) of patients treated with open surgical repair or bypass 2 required bypass revision and/or thrombectomy, and 4 had concomitant ureteral ligation or nephrectomy. Despite undergoing anticoagulation 10 patients (53%) experienced lower extremity morbidity including ulceration, ischemia and amputation. In each treatment group 2 patients had recurrent hemorrhage requiring a secondary procedure, leading to death in 2 for an overall 10% acute mortality rate. Overall noncause specific mortality of ureteroarterial fistulas was 47% and 10% to 20% was related to the fistula or treatment complications. CONCLUSIONS: Endovascular stenting is increasingly used in lieu of open techniques due to the high operative risk and comorbidities in patients with ureteroarterial fistulas. This retrospective review fails to identify a clear advantage for endovascular or open vascular surgical management. Thus, endovascular stenting is preferred in most cases. Regardless of therapy, patients are at risk for recurrent bleeding, lower extremity complications and stent/graft complications. The use of antibiotics and long-term anticoagulant therapy appear prudent but not proved.