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.

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.

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.

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.

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.

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.

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.

Urethral Stone Disease Leading to Retention After Hair-bearing Neophalloplasty.

A 35-year-old male patient with a past history of traumatic penile amputation and subsequent penile reconstruction with a radial artery free flap phalloplasty presented to the urology clinic for urinary retention and complaint of a firm penile mass. The patient had been lost to follow-up for 2 years before this presentation. Patient had a suprapubic tube in place from initial surgery, with imaging showing 2 large uroliths encrusted around the end. Urethral stricture was suspected in the patient. On cystoscopy, an additional obstructing urolith was found in penile urethra, appearing to have formed in situ.

Spontaneous Extraperitoneal Bladder Rupture Because of Chronic Appendicitis.

A 62-year-old man presented to the emergency department with an episode of syncope after 2-3 weeks of diffuse abdominal pain, now complaining of a severe increase in pain concurrent with >24 hours of no urine output. His workup showed an idiopathic extraperitoneal rupture of the bladder on computed tomography, which was handled conservatively with Foley insertion. Repeated follow-up and imaging showed no resolution or etiology over 2 months. The patient underwent exploratory laparotomy that showed an elongated appendix with a chronic tip appendicitis that had induced bladder rupture by chronic inflammatory changes. After repair, the patient had no further complaints.