Intratesticular arteriovenous malformation: A rare benign testicular lesion in an adolescent male.

We present the clinical course and imaging findings of an intratesticular arteriovenous malformation found in an adolescent patient. The patient presented for evaluation of a possible testicular mass. Evaluation included grayscale and Doppler ultrasound that demonstrated a vascular mass. Serum tumor markers were unremarkable. Magnetic resonance imaging was used to make the diagnosis of intratesticular arteriovenous malformation. Intratesticular arteriovenous malformations are exceedingly rare, with only four other cases noted on literature review. This case presents unique findings including testicular microlithiasis and a history of cryptorchidism. The case was managed conservatively with ultrasound surveillance at six months.

First Case of a Term Male Born with Aphallia and Complete Urethral Atresia.

Aphallia is an exceedingly rare condition often associated with an array of genitourinary anomalies. Classically, aphallia was thought to have to co-exist with a urethral fistula for adequate urine outflow to avoid the sequelae of oligohydramnios, while the absence of a fistula has historically been incompatible with life. We report the first case of a prenatally unrecognized aphallia with complete urethral atresia in a full-term baby, without an associated fistula or ectopic urethra. We postulate a urachal cyst noted on prenatal ultrasound resolved into a patent urachus providing sufficient outflow to avoid sequelae of oligohydramnios and allowed for term birth.

Rare case: Ureteropelvic junction complication presenting with bilateral labial abscesses and urosepsis requiring nephrectomy.

Ureteropelvic junction obstruction (UPJO) is the most common cause of urinary tract obstruction in pediatric patients. Debates in management include ureteral stent versus nephrostomy tube placement prior to surgical correction if intervention is warranted. We present a female patient with left UPJO, diagnosed at 15-years-old, treated with ureteral stent placement. Stent removal two years later resulted in extensive complications, including retroperitoneal infection, labial abscesses, and nephrectomy. Management of UPJO in the pediatric population prior to surgical correction is not well-standardized. The severity of complications following the removal of the two-year-old stent suggests caution for placing ureteral stents without proper follow-up.

Attitudes towards child life specialists and their utilization within pediatric urology.

INTRODUCTION: The American Academy of Pediatrics views Certified Child Life Specialists (CCLS) as "an important component of pediatric hospital based care to address the psychosocial concerns that accompany hospitalization." CCLSs help patients and parents navigate the complex medical system in order to minimize psychosocial and emotional stress by implementing age appropriate coping skills. This survey explores the perceptions towards CCLS and their utilization with pediatric urology. MATERIALS AND METHODS: A Survey Monkey questionnaire was developed and distributed to Society for Pediatric Urology members (SPU) (n = 314). Providers were queried about CCLS implementation and their perception regarding CCLS's role in improving health related quality of life (HRQOL). RESULTS: There was a 34.1% response rate (n = 107). Ninety-four providers (87.9%) reported CCLS interaction with their patients and greater than 95% of providers felt CCLS imparted some degree of benefit to their patients' HRQOL. Only 4.7% felt CCLS offered no benefit to the patients. CCLS were consistently used in a minority of inpatient and outpatient settings and never in the radiological setting. They were used at least 50% of the time by the most responders in inpatient and radiological setting and nearly the same in the ambulatory surgery setting. CONCLUSIONS: This survey illuminates that the majority of providers interact with CCLS in clinical settings and believe their involvement is beneficial. However, CCLSs are under-utilized during invasive urological procedures where patient anxiety is high. By understanding perceptions of providers and their practice patterns we can overcome barriers to CCLS use and improve their quality of life.

A Case of Evan's Syndrome Induced by Vedolizumab.

Vedolizumab is a monoclonal antibody against the ɑ4ß7 integrin receptor used for the treatment of ulcerative colitis and Crohn's disease. Clinical trials have shown vedolizumab to be a safe and highly effective therapy in treating inflammatory bowel disease. Its unique gut-specific mechanism of action has made it an attractive agent in recent years. However, vedolizumab's side effect profile and long-term effects are not fully understood. We report a patient with ulcerative colitis who presented with epistaxis 1 week after receiving the first induction dose of vedolizumab found to have a severe immune-mediated thrombocytopenia and hemolytic anemia, otherwise known as Evan's syndrome.

Propensity-matched analysis of patient-reported outcomes for neoadjuvant chemotherapy prior to radical cystectomy.

PURPOSE: To evaluate patient-reported outcomes (PROs) for bladder cancer patients undergoing neoadjuvant chemotherapy (NAC) prior to radical cystectomy (RC) using longitudinal data and propensity-matched scoring analyses. METHODS: 155 patients with muscle-invasive bladder cancer scheduled for RC completed the European Organization for Research and Treatment of Cancer questionnaires, EORTC QLQ-C30, EORTC QLQ-BLM30, Fear of Recurrence Scale, Mental Health Inventory and Satisfaction with Life Scale within 4 weeks of surgery. A propensity-matched analysis was performed comparing pre-surgery PROs among 101 patients who completed NAC versus 54 patients who did not receive NAC. We also compared PROs pre- and post-chemotherapy for 16 patients who had data available for both time points. RESULTS: In propensity-matched analysis, NAC-treated patients reported better emotional and sexual function, mental health, urinary function and fewer financial concerns compared to those that did not receive NAC. Longitudinal analysis showed increases in fatigue, nausea and appetite loss following chemotherapy. CONCLUSION: Propensity-matched analysis did not demonstrate a negative effect of NAC on PRO. Several positive associations of NAC were found in the propensity-matched analysis, possibly due to other confounding differences between the two groups or actual clinical benefit. Longitudinal analysis of a small number of patients found small to modest detrimental effects from NAC similar to toxicities previously reported. Our preliminary findings, along with known survival and toxicity data, should be considered in decision-making for NAC.

Influential Factors in Pursuit of Pediatric Urology Fellowship Training.

INTRODUCTION: We investigated factors that helped recent fellows achieve a successful match and sought to understand the influences in pursuing a career in pediatric urology. Selecting to continue training after urology residency entails much consideration. However, there are few tangible resources available to help residents understand the process. METHODS: A 20-item web based survey was e-mailed to second year fellows and recent graduates of pediatric urology fellowships (103) from the graduating classes of 2013 to 2015. The survey explored factors that affected the decision to pursue this field along with the influence of mentors, fellows and other residents who applied to pediatric urology. We assessed the key factors that helped applicants achieve a successful match. Descriptive statistics and univariate logistic regression analysis were used to determine relationships between program characteristics and respondents' decisions to apply and interview at various programs. RESULTS: In total, 59 of 103 individuals surveyed (57.3%) responded to the survey. Faculty, program reputation and clinical volume had key roles in ranking programs, while satisfaction with the match result was contingent upon accurately assessing programs based on key factors such as family considerations, location, program reputation, faculty, clinical year volume and gut feeling. In addition, our survey demonstrated that having a pediatric urology fellow at one's institution had little impact on a resident's decision to pursue pediatric urology. CONCLUSIONS: As the subspecialty of pediatric urology continues to grow, we identified key elements that help shape and influence choices to participate in the future of this ever evolving field.

The role of inheritance in the development of adolescent varicoceles.

BACKGROUND: The prevalence of varicoceles is estimated to be 15% in the general population but is nearly 35% among men with primary infertility and increases by 10% with each decade of life. Studies among adults infer a higher rate of varicoceles among first-degree relatives of patients with varicoceles. However, these studies do not consider the presence of varicoceles, or other venous abnormalities, at the time of desired paternity in first-degree relatives of adolescent patients. Our aim was to document the prevalence of varicoceles and varicose veins among first-degree relatives of pediatric patients diagnosed with varicoceles. METHODS: A series of 884 patients between the ages of 17 and 22 diagnosed with a varicocele were contacted and of the responders, permission was asked to discuss family history. Each patient underwent a telephone survey regarding the presence of a varicocele in the father prior to achieving paternity, the presence of a varicocele in brothers in childhood, or varicose veins in the father and mother prior to achieving pregnancy. If a varicocele or varicose vein was present, a history of intervention was surveyed. Descriptive statistics were performed to assess overall prevalence of varicocele and varicose veins in first-degree relatives. Chi-squared and logistic regression analyses were performed. RESULTS: In total, 152 patients (17.2%) responded and 139 (91.4%) consented to the telephone survey (mean age 19.5±1.84 years old). Overall, 12.9% of fathers had varicoceles and 8.6% of fathers underwent surgical intervention. Among siblings, 18 individuals had a varicocele and 5.6% of siblings with varicocele required intervention. Varicose veins were diagnosed in 7.9% of fathers and 25.2% of mothers; 8.6% of mothers underwent intervention. Surveillance was employed in 76.3% of patients, and surgery undertaken 21.6%. Patients with grade 2 or 3 varicoceles were more likely to have a father with a varicocele than those with grade 1 varicocele (P=0.037). Patients with grade 2 varicocele were more likely to have a father who required varicocele surgery than grade 1 (P=0.019). There was no statistical association of adolescent varicocele and varicose veins in first degree relatives (P=0.306). CONCLUSIONS: This series shows that while a father's prevalence is similar to that of the general population, higher grade varicoceles were associated with a higher prevalence of varicoceles and surgical ligation. This suggests the existence of a hereditary predisposition to high grade varicoceles. Furthermore, varicocele is not associated with varicose veins in first degree relatives.

Renal sonographic changes in heterogeneity index and echogenicity in children with hypertension: a novel assessment.

The kidneys are thought to contribute to the pathogenesis of primary hypertension, but hypertension is also known to cause target organ damage in the kidney. Noninvasive methods to capture possible changes in the kidney related to hypertension are limited. A new program that has been used to quantify the heterogeneity and percent echogenicity in renal ultrasound images was implemented to assess patients with hypertension. Children and adolescents <21 years with primary hypertension diagnosed by ambulatory blood pressure monitoring were compared with normotensive age- and sex-matched controls. Renal ultrasound images were evaluated by a technique that measured pixels of gray-scale images and transformed them into a binary map, which was converted to a heterogeneity index (HI) and percent echogenicity score. This study included 99 children with hypertension and 99 control subjects. Body mass index (BMI) was greater in the hypertension group. Average HI for hypertension was significantly higher than in controls (1.37 ± 0.19 vs. 1.2 ± 0.23, P = .001), while echogenicity scores were not different (26.6 ± 8.9 vs. 25.9 ± 10, P = .8). In regression analysis adjusting for BMI z-score and race, hypertension was associated with greater HI compared with controls (ß = 0.11, 95% confidence interval 0.03-0.18, P = .005). In a model adjusted for age, sex, and BMI z-score in the hypertension group only, no ambulatory blood pressure monitoring measures were associated with HI or echogenicity scores (P > .05).HI was significantly greater in the hypertension group compared with normotensive controls. HI may be a novel method to detect changes in the kidney related to hypertension.

Synchronous, but separate, bladder and vaginal rhabdomyosarcoma: a novel genetic case report.

Embryonal rhabdomyosarcoma is a rare cancer that often requires multimodality therapy to treat; however, these therapies can cause changes in the biology of the tumor. Several reports have documented pathologic changes but only recently have genetic changes been mapped. We present case of two separate synchronous primary rhabdomyosarcomas in a 17-month-old patient and discuss the pathophysiology and genetic changes that occur with treatment. We hypothesize that a genetic field defect arising in development of the urogenital sinus caused the tumors, but that treatment modalities may have caused genetic alterations changing clinical behavior of the tumors and responses to treatment.

Persistent cough as a paraneoplastic presenting symptom in six patients with renal cell carcinoma.

OBJECTIVE: With today's modern imaging modalities, patients diagnosed with renal cell carcinoma (RCC) rarely present symptomatically. In some cases, however, they can develop paraneoplastic syndromes with associated symptoms. To date, only three cases of RCC presenting with chronic dry cough have been reported. We describe six patients who presented with cough that improved following radical nephrectomy. METHODS: A retrospective review of patients undergoing partial or radical nephrectomy for renal masses between January 2015 and March 2016 was performed, and patients presenting with a cough were examined. RESULTS: Six patients presented with chronic cough and were discovered to have a large renal mass. Postoperative spontaneous resolution of cough was noted in all but one patient, in whom coughing was reduced and limited to the mornings. Cough duration ranged from 3 months to just over a year. All patients were treated with radical nephrectomy, which was cytoreductive in four patients. Average tumor size was 10.9 cm (SD = 2.2 cm). Five of the tumors had clear cell pathology, and every tumor was Fuhrman grade IV, unifocal, and demonstrated necrosis. Sarcomatoid features were reported in four of the tumors. CONCLUSION: Our study presents the largest series of patients with RCC who presented with a chronic cough that was significantly improved following radical nephrectomy. We believe the cause of cough is multifactorial and further investigation is needed to clearly elucidate the etiology.

A Unique Case of Pentaorchidism.

Polyorchidism is a rare congenital anomaly with less than 200 case reports in literature. Triorchidism, the condition of having 3 testicles, is the most common presentation. We present an unusual case of a patient who was diagnosed with 5 testicles by magnetic resonance imaging. To the best of our knowledge, this rare presentation has not previously been reported in the medical literature.

Wilms Tumor With Metastasis to the Vagina: A Case Report.

A 12-year-old female presented with abdominal pain, night sweats, weight loss, constipation, dysmenorrhea, menorrhagia, and vaginal discharge. Examination revealed a palpable flank mass and a large tumor adherent to the anterior vaginal wall. Computed tomography scan demonstrated a 23 cm mass in the left kidney, a separate 10.8 cm pelvic mass, and metastatic disease. Biopsies were consistent with Wilms tumor. Neoadjuvant chemotherapy and a left radical nephrectomy were performed for her stage IV disease as the kidney was amiable to complete resection. The patient received radiation and resumed chemotherapy. She was doing well with improved symptoms at follow-up.

Idiographic quality of life assessment before radical cystectomy.

BACKGROUND: We sought to determine if idiographic, or self-defined, measures added to our understanding of patients with bladder cancer's quality of life (QOL) prior to radical cystectomy (RC). We tested whether idiographic measures increased prediction of global QOL beyond standard (nomothetic) measures of QOL components. METHODS: We administered the European Organization for Research and Treatment of Cancer Quality of Life Questionnaires (QLQ)-C30 and QLQ-BLM30, and our own idiographic Quality of Life Appraisal Profile prior to RC. Idiographic measures included number of goal statements, distance from goal attainment, and ability to complete goal attainment activities. Multivariate linear regression was used to predict measures of global QOL and related constructs of life satisfaction and mental health. RESULTS: Two hundred fiftheen patients reported a median of 8 (interquartile range [IQR] 6, 11) goals and half had an average goal attainment rating above 6.9 out of 10 (IQR 5.5, 8.2). On multivariable analysis, QLQ-C30 role functioning and QLQ-BLM30 future perspective explained 15.7% of the variability in preoperative global QOL. Including goal attainment and activity difficulty explained an additional 12% of global QOL variance. Smaller gains were seen on measures of global health, life satisfaction, mental health, and activity, suggesting that idiographic measures capture aspects of QOL distinct from health and functional status defined by nomothetic scales. CONCLUSIONS: Idiographic assessment of QOL added to prediction of global QOL above and beyond health-related components measured using nomothetic instruments. This self-defined information may be valuable in communicating with cancer patients about their QOL. Copyright © 2015 John Wiley & Sons, Ltd.

Pediatric Urologists' Personal Point-of-view of Health Related Quality of Life.

OBJECTIVE: To assess pediatric urologists' understanding and use of health-related quality of life (HRQOL) resources in clinical practice. HRQOL is becoming an increasingly vital part of patient care. Although HRQOL has been evaluated and instruments have been developed for its assessment for several pediatric conditions, it is underrepresented in pediatric urology. METHODS: A SurveyMonkey 7-item questionnaire was sent to the members of the Society for Pediatric Urology. The questions inquired as to knowledge of surveys available, views on appropriate age for beginning discussion of HRQOL, patients' and providers' ability to assess patient HRQOL, and the need for disease-specific surveys. RESULTS: One-hundred and eighteen providers (37.6%) completed the survey. Remarkably, 85.6% of the responders believe disease-focused HRQOL instruments would be useful for specific urologic diseases; however, only 14.4% of the providers state use of any HRQOL instrument. Only 5 unique measures were reported. The majority felt the age at which patients can start reporting his or her HRQOL is 6-8 (41.3%) or 9-11 (31.2%) years. A preponderance of providers believed both themselves and parents could "somewhat" evaluate the patient HRQOL, but the majority believe it is important to inquire about parents' perceptions of patient HRQOL. CONCLUSION: The overwhelming majority of pediatric urologists believe HRQOL is important but only a minority utilize the available instruments. Further studies are needed to develop instruments to fill this essential gap.

Percutaneous nephrolithotomy in octogenarians and beyond: How old is too old?

OBJECTIVE: To specifically report perioperative characteristics and outcomes in patients 80 years and older undergoing percutaneous nephrolithotomy (PCNL). PCNL has been established as feasible in the elderly; however, to our knowledge no one has specifically reported feasibility in patients 80 years and older. METHODS: We retrospectively reviewed perioperative data of octogenarians who underwent PCNL at a high stone volume single institution, and matched them to patients <65 years of age by stone burden and sex. Patient demographics, perioperative outcomes and postoperative complications were compared. RESULTS: Thirty-three octogenarians (mean age 83.6 years) with 36 renal units were matched to 67 controls (mean age 48.6 years) with 72 renal units. Octogenarians had a higher mean American Society of Anesthesiologists (ASA) score, more comorbidities, and worse renal function. There were no differences in operative characteristics, length of hospital stay or stone free rates. Of the patients with preoperative urinary decompression (ureteral stent or nephrostomy tube) prior to PCNL, the elderly were more likely to have a history of urosepsis. Octogenarians did not experience more minor Clavien (I - II) or major Clavien (IIIa - IVb) complications. CONCLUSION: Octogenarians who undergo PCNL were more likely to have cardiovascular comorbidities and a prior history of sepsis. Despite these risk factors, in appropriately selected patients PCNL can be safely and successfully performed in octogenarians without increased perioperative complications relative to a younger cohort.

Tubulovillous Adenoma in a Urethral Neobladder Anastomosis.

We present a case of a tubulovillous adenoma arising in a neobladder that was managed by cystoscopic resection. A 64 year-old male underwent a cystectomy with creation of an ileocolic neobladder urinary diversion for T2 urothelial carcinoma of the bladder. Nine years following his surgery, the patient noted several episodes of gross hematuria. Cystoscopic evaluation revealed the rare occurrence of a 3 cm tubulovillous adenoma with high-grade dysplasia at the neck of the neobladder.

Percutaneous nephrolithotomy during uninterrupted aspirin therapy in high-cardiovascular risk patients: preliminary report.

OBJECTIVE: To determine the feasibility and safety of performing percutaneous nephrolithotomy (PCNL) in high-cardiovascular risk patients remaining on aspirin therapy. METHODS: We retrospectively reviewed all PCNLs performed by 3 fellowship-trained endourologists at a single institution between July 2012 and January 2014. All patients remaining on aspirin for imperative indications through the day of surgery were evaluated for surgical outcomes and thromboembolic events. RESULTS: Of 281 PCNL procedures performed during the study period, 16 (5.7%) were performed in 14 patients taking aspirin, uninterrupted, through surgery. Mean surgery time was 66 minutes, mean estimated blood loss was 161 mL, and mean length of hospital stay was 2.8 days. All patients were stone free. There were no intraoperative complications. A total of 5 patients experienced a postoperative complication (n = 4, Clavien grade II; n = 1, Clavien grade IIIa). No patient experienced a perioperative thromboembolic or cardiac event. Three patients required a blood transfusion postoperatively, and none experienced delayed renal bleeding. CONCLUSION: PCNL can be performed safely and effectively in high-cardiovascular risk patients continuing aspirin perioperatively. Compared with the potential sequelae of a thromboembolic or cardiac event, PCNL is associated with an acceptably low transfusion rate, and should be considered a viable treatment option for large renal stones in this population.

Acute bladder and small bowel perforation as a complication of Foley catheterization.

The complications of chronic urethral catheterization are well documented in the medical published data. Chronic urethral catheterization has been demonstrated to cause inflammatory changes within the bladder wall, promoting perforation and small bowel fistualization. In this report, we document the clinical course of a patient who presented with acute perforation of the bladder and small bowel as a result of Foley catheterization. To our knowledge, this is the first documented acute perforation of the bladder and the small bowel during Foley catheterization.