Size matters: Total testicular volume predicts sperm count in Tanner V varicocele patients.

INTRODUCTION: It is known the prevalence of varicoceles in adolescent men is 14-29% but there is debate surrounding implications on fertility. As obtaining a semen analysis (SA) may be challenging, there is need for objective tests as measures of fecundity. Our aim was to investigate the relationship between testicular volume differential (TVD), varicocele grade, and total testicular volume (TTV) on seminal parameters including total motile sperm count (TMSC). MATERIALS AND METHODS: We conducted a retrospective single-center chart review over 14 years of 486 Tanner V adolescent males. Three hundred and four met inclusion of palpable, non-operated left-sided varicocele who underwent at least one SA and ultrasound. Abnormal TMSC was defined by World Health Organization 2010 criteria for minimal reference ranges. Multivariate logistic regression, receiver operating characteristic analysis with Youden J-statistic and descriptive statistics were performed. RESULTS: Three hundred and four Tanner V adolescents with median age of 18.0 years (18.0-19.0), median TTV of 34.5 cc (28.9, 40.2) and median TMSC of 62.5 million/ejaculate (25.4, 123.4) were evaluated. TTV cutoff of 29.5 cc was found to predict TMSC of <9 million/ejaculate with negative predictive value of 96.2% and odds ratio of 6.08 ([2.13-17.42], p < 0.001). TVD greater than 20% did not reach statistical significance with an odds ratio of 1.66 ([0.41-6.62], p = 0.50). DISCUSSION: In clinical practice, each patient will need to have an individualized plan. Based on our data, for older adolescents (17 or 18 years) with varicocele and an abnormal TTV, clinicians may have a lower threshold for advising SA, and if unable to obtain, surgical intervention and/or closer surveillance should be stressed. Patients should be informed of their six-fold increase in abnormal SA. Patients with normal TTV should be advised they are at lower risk of having abnormal SA. Younger patients with varicocele and an initial TVD>20%, should be followed closely but intervention delayed until 17 or 18 to better assess TTV. The importance of trending patient data should be emphasized as a single measurement has low predictive value for developing adolescents. Limitations of our study include a retrospective design and the lack of uniform correlation between adolescent SA and paternity. CONCLUSIONS: Total testicular volume less than 29.5 cc increased odds of abnormal semen analysis by over six times and had a negative predictive value of 96.2%. Ultrasound results may be useful for risk stratification and counselling on appropriateness of surgical intervention.

Management of the refractory nocturnal enuresis patient to desmopressin in a pediatric population: Desmopressin + oxybutynin vs. desmopressin + imipramine.

INTRODUCTION AND OBJECTIVE: Desmopressin is well accepted as first-line medical therapy for enuresis. If ineffective, combination therapy of desmopressin + oxybutynin or desmopressin + imipramine has been used. This study assessed the efficacy of adjunct therapy with either imipramine or oxybutynin in the management of enuresis patients who failed desmopressin treatment. STUDY DESIGN: A retrospective chart review of our database for patients with enuresis was performed. Patients who were prescribed desmopressin, oxybutynin, and imipramine over 14 years for enuresis were included. Two cohorts of patients were examined; group OXY was treated with desmopressin and oxybutynin, and group IMP received desmopressin and imipramine. Pretreatment measurement of Vancouver Symptom Scores (VSS) were used to compare groups using the VSS question "I wet my bed at night" where 4: every night, 3: 4-5 nights per week, 2: 1-2 nights per week, 1: 3-4 nights per month, and 0: never. International Children's Continence Society (ICCS) criteria for continence success was utilized to determine outcomes. RESULTS: 2521 patients prescribed one of the 3 medications were identified. Among them, 81 patients (mean age: 10.5 ± 2.8 years) received combination therapy. Of which, 55 were male and 26 female. Specifically, 58 were prescribed both desmopressin and imipramine (group IMP), 23 desmopressin and oxybutynin (group OXY), and 4 transitioned from OXY to IMP. Mean pretreatment VSS showed no difference between groups. Both groups experienced minimal drops in wet nights with desmopressin alone. A comparison revealed that group IMP reduced wet nights significantly more than group OXY (VSS wet night score 0.7 ± 1.2 vs. 2.3 ± 1.1 respectively, p < 0.0001). Non-intent-to-treat complete response rate was 68% vs 5% (OR = 42.5, p < 0.001) (IMP vs. OXY respectively). Intent-to-treat response rates were 58%. DISCUSSION: Although first-line desmopressin treatment for enuresis is effective, it does not work for all patients, and many parents and children desire nighttime dryness. Clinicians have combined desmopressin with oxybutynin or imipramine for improved results, but research comparing these modalities is scarce. Our study suggests that the desmopressin and imipramine combination is superior at reducing nights wet compared to desmopressin and oxybutynin, attributed to imipramine's probable central mechanism rather than its secondary anticholinergic properties. Limitations include a modest sample size, retrospective design, and subjective responses to the Vancouver questionnaire. CONCLUSION: A combination of desmopressin and imipramine was more effective in reducing wet nights and had a complete response rate that was 42.5 times greater than desmopressin and oxybutynin.

A comparison of 467 uroflowmetry results in repaired hypospadias vs. normal male flows.

INTRODUCTION: There are currently no clinical criteria for obstructed urinary flow after hypospadias repair surgery. Previous studies have utilized adult and pediatric nomograms and flow shapes to define obstruction, however these methods are limited by a lack of standardization and lack of interrater reliability when determining flow shapes, respectively. The idealized voider derived flow indexes offer a way to track uroflowmetry results in a volume and age agnostic manner. OBJECTIVE: We sought to evaluate all our hypospadias patients over a 10-year period and identify patients without complications and those with complications and determine their respective flow parameters. Our secondary objective is to identify which uroflowmetry parameters are the most significant predictors of urethral stricture and meatal stenosis at the time of the uroflowmetry study. STUDY DESIGN: Retrospective chart review was used to compile demographic information, details of hypospadias repair surgeries, and uroflowmetry results from pediatric hypospadias repair patients. Subjects were divided into distal, midshaft, and proximal groups based on the initial location of their urethral meatus. Flows from the hypospadias repair groups were compared to flows from normal age matched controls from a previous study. We compared flows from hypospadias repair patients with no complications present with those who had urethral stricture or meatal stenosis present at the time of uroflowmetry. Binary logistic regression and ROC analysis was used to assess different uroflowmetry parameters' ability to detect the presence of obstructed urine flow. RESULTS: 467 uroflowmetry studies from 200 hypospadias repair patients were included in the database. Compared to controls, the hypospadias repair groups tended to have significantly lower Qmax, Qavg, Qmax FI, Qavg FI, and longer ttQmax. Significant differences in flow parameters were observed when comparing hypospadias repair patients with and without flow obstructing complications at the time of uroflowmetry. Binary logistic regression including various uroflowmetry parameters showed Qmax FI had a significant effect on the odds of observing the absence of a stricture in proximal and distal hypospadias cases. DISCUSSION: Of the uroflowmetry parameters analyzed, binary logistic regression and the likelihood ratio of a positive result all point to Qmax FI as the better parameter to use to detect the presence of complications in patients who have undergone distal or proximal hypospadias repair surgery. CONCLUSION: We have established normal parameters for post-operative hypospadias repair patients which can be used to follow patients over time and allow for the identification of complications by keeping track of flow indexes which are volume and age agnostic.

Pediatric Bladder Tumors: A Ten-Year Retrospective Analysis.

OBJECTIVE: To present our experience in a single pediatric urology practice over a 10-year period with bladder tumors in the pediatric population in an effort to add to the relatively small amount of existing data. We hope to expand the community's knowledge of presentations, management and natural history of pediatric bladder tumors. METHODS: We retrospectively queried our electronic medical records for International Classification of Diseases, Tenth Revision (ICD-10) and Current Procedural Terminology (CPT) codes relevant for bladder tumors. Patients with underlying bladder pathology, such as neurogenic bladder, history of bladder exstrophy, and history of bladder augmentation, were excluded. RESULTS: We identified 30 patients with bladder tumors from 2011 to 2021. There were 21 males and 9 females. Age at diagnosis ranged from 16 months to 19 years. Tumors identified were: 11 of various inflammatory subtypes; 4 papillomas; 4 rhabdomyosarcomas; 3 papillary urothelial neoplasms of low malignant potential and 8 of other types. Treatment included transurethral resection of bladder tumor, chemoradiation and laparoscopic partial cystectomy. Twenty nine patients had disease limited to the bladder and 1 had disease outside the bladder. Follow-up ranged from 2 weeks to 13 years (median 19 months). All patients had no evidence of disease at most recent follow-up. CONCLUSION: Pediatric bladder tumors range from aggressive rhabdomyosarcomas to more benign urothelial lesions. Fortunately, the latter type of tumor is the more prevalent lesion. Knowledge of the treatment options and natural history of these tumors will hopefully be of benefit to clinicians and parents alike.

Diphallia, Double Bladder, and Two Hemiscrotums: A Case Report.

Complete diphallia, a rare urogenital congenital anomaly in which a male is born with two fully formed phalluses, occurs in one out of every five to six million live births. The condition is characterized by two separate phalluses, each of which comprise a pair of corpora cavernosa and one corpus spongiosum with an orthotopic urethra. Approximately 100 cases have been reported worldwide, and it is thought that each case is unique. This article discusses diphallia, urethral duplication, and bladder duplication and concludes with a case study involving a three-year-old male born to consanguineous parents from a small, remote community in Ecuador who underwent surgery for correction of a complete coronal penile and bladder duplication. After consultation, the patient was scheduled for a right penectomy and cystoplasty.

Robot-assisted Laparoscopic Urachal Excision in Children.

OBJECTIVE: To report the first exclusively pediatric series of robot-assisted urachal remnant excisions in children. METHODS: We reviewed the medical records of all children who underwent robot-assisted excision of urachal remnants from 2010 to 2016. For the procedure, a 3-port approach was performed in all cases. Excision of the urachus was performed, along with partial cystectomy if there was clear or suspected bladder involvement. Outcomes and complications were reviewed. RESULTS: Sixteen cases of robotic urachal excision were performed during the study period in patients aged 0.8-16.5 years. Complete excision was accomplished in all cases with no conversions. Partial cystectomy was performed in 11 cases, in which a urinary catheter was left for 1 day in all cases (no catheter was left in the absence of partial cystectomy). The only complication was a bladder leak requiring open surgical repair. There were no bowel injuries or hernias. The median operative time was 107 minutes. The length of stay was 2 days with partial cystectomy and 1 day without partial cystectomy. All patients were well at follow-up. CONCLUSION: We report the largest known series of robot-assisted urachal remnant excisions in children, demonstrating this minimally invasive approach to be safe and effective.

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.

Barriers to use of semen analysis in the adolescent with a varicocele: Survey of patient, parental, and practitioner attitudes.

BACKGROUND: The American Society for Reproductive Medicine Practice Committee recommends obtaining a semen analysis (SA) in pediatric patients presenting with a varicocele in the absence of significant testicular atrophy. Among infertile adults with a varicocele, surgery is indicated in the presence of abnormal semen analysis regardless of testicular atrophy. Despite these two statements, semen analysis is not widely utilized by pediatric urologists in the USA managing a patient with a varicocele. OBJECTIVE: We explored the attitudes of patients, parents, and practitioners toward SA to identify potential barriers to the use of SA in the evaluation of the adolescent varicocele. STUDY DESIGN: We conducted a survey of Society for Pediatric Urology members regarding their management of adolescent varicoceles, with focus on the utilization of SA. The survey consisted of 14 multiple choice questions and two open-ended questions regarding use of SA in practice, barriers to its use, indications for varicocelectomy, and demographics. We also surveyed patients presenting for initial evaluation of a varicocele, as well as their parents, regarding their knowledge about SA and their attitude towards obtaining it. Statistical analysis was performed (p < 0.05 significant). RESULTS: The practitioner survey response rate was 53% (168). Only 13.1% routinely incorporated SA in their practice, with 48% of all responders having some degree of discomfort asking for a SA. Of practitioners who cited discomfort, 90% never order a SA for patients with varicoceles. From the 46% of physicians who ordered a SA, we noted significant practice variability (see Figure). The patient/parent survey demonstrated that this population was uncomfortable with the notion of obtaining a SA, with most patients/parents citing lack of knowledge about SA as the main barrier. Patient and parent knowledge was found to correlate. DISCUSSION: This study uniquely addresses an issue that has not been discussed in the adolescent varicocele literature to date. It can increase awareness of the option of incorporating SA data in management of the adolescent who presents with a varicocele. CONCLUSION: Recognizing and then breaking through the barriers to obtaining a SA, would improve patient care, providing a direct assessment of the impact of a varicocele on fertility potential and thus best determining which patients require surveillance versus surgical intervention. This study suggested that the barriers to SA are surmountable.

Tunneled buccal mucosa tube grafts for repair of proximal hypospadias.

PURPOSE: Buccal mucosa is the favored graft material for patients with long urethral defects and a paucity of skin. Since 2007, we have used the novel tunneled buccal mucosa tube graft urethroplasty technique in these patients. We describe this operative technique and report our surgical and functional outcomes. MATERIALS AND METHODS: Between 2007 and 2013, 37 males with proximal hypospadias underwent tunneled buccal mucosa tube graft urethroplasty. After the penile shaft was optimized at a prior stage a free buccal graft was tubularized and tunneled under the intact ventral shaft skin and into the glans. We retrospectively reviewed all charts to report our results. We assessed uroflowmetry and bladder ultrasound for post-void residual urine. RESULTS: The overall complications rate in 34 patients with more than 1-year followup was 32% (11), including fistula in 5, proximal stricture in 4 and meatal stenosis in 2. In the first 10 patients a total of 7 complications (70%) developed but there were only 4 complications in the next 24 (16%). Surgeon experience was the only significant predictor of complications (p = 0.003). We obtained uroflow and post-void residual urine data on 13 of 37 patients, of whom 9 achieved a normal flow pattern and post-void residual urine, and 4 had a blunted flow pattern. CONCLUSIONS: The novel technique of the tunneled buccal mucosa tube graft in patients with proximal hypospadias represents a good alternative for a long urethroplasty in patients with a paucity of skin. After the learning curve plateaus the rate and degree of complications decrease. Furthermore, voiding function is adequate, as assessed by uroflow studies and post-void residual urine measurement.

Bladder ganglioneuroma in a 5-year-old girl presenting with a urinary tract infection and hematuria: case report and review of the literature.

Ganglioneuromas are rare benign tumors arising from neural crest cells of the autonomic nervous system. These tumors may rarely localize to the bladder, and few cases have been reported in the adult literature. To date, however, bladder ganglioneuromas have not been reported in the pediatric literature. We report the case of a 5-year-old girl who presented with hematuria and a urinary tract infection and on workup was found to have a large bladder mass. Transurethral resection and pathologic examination revealed the mass to be a ganglioneuroma. The case is presented followed by a brief review of the literature.

Laparoscopic orchiopexy and varicocelectomy: is there really an advantage?

The role of laparoscopy in the case of nonpalpable cryptorchidism is both diagnostic and therapeutic. Laparoscopic orchiopexy for nonpalpable testes in the pediatric population has become the preferred surgical approach among pediatric urologists over the last 20 years. In contrast, laparoscopic varicocelectomy is considered one of several possible approaches to the treatment of a varicocele in an adolescent; however, it has many challengers and it has not gained universal acceptance as the gold standard. This article reviews the published evidence regarding these surgical techniques.

Laparoendoscopic single-site radical nephrectomy for large renal masses.

PURPOSE: To report our operative experience and short-term outcomes for the laparoendoscopic single-site (LESS) management of large renal tumors and tumors of advanced stage. PATIENTS AND METHODS: Ten consecutive patients underwent LESS-radical nephrectomy (RN) for large (≥ 7 cm) and/or locally advanced tumors (>T(2)). Intraoperative, postoperative, and short-term follow-up data were analyzed. RESULTS: Median surgical time was 146 minutes (range 73-164 min), and median estimated blood loss was 100 mL (range 25-400 mL). No procedure needed conversion to open RN or hand-assisted laparoscopic RN. The median hospital stay was 47 hours (range 42 hours-12 days). One (10%) patient had a minor complication (postoperative fever treated with antibiotics) and one (10%) patient had a major complication (small bowel obstruction necessitating reoperation). Of the 10 tumors, 2 were pathologic stage T(1b), 4 were pathologic stage T(2), and 4 were stage T(3a). At a median follow-up of 12.3 months (range 1-16 mos), six (60%) patients were alive without evidence of recurrence, and 4 (40%) patients were alive with disease. Of those four patients, all four had known metastatic disease before surgery. CONCLUSION: LESS-RN for large or advanced stage renal masses is a technically challenging operation. In experienced hands, however, it is a safe and feasible therapeutic option for the management of these tumors.

Varicocele: standard and alternative indications for repair.

PURPOSE OF REVIEW: This review provides timely and relevant information to address the indications for repair of the adolescent varicocele. In this review, we summarize the most recent available evidence and provide clinical guidelines. RECENT FINDINGS: The indications for adolescent varicocelectomy commonly include testicular hypotrophy and pain but become confusing and contradictory when discussing potential infertility as an indication for repair. The preponderance of patients with varicocele in the infertile population leads to the search for causality and reversibility. However, in the era of assisted reproduction, the question of early prevention of infertility is brought to the forefront of discussion. Recent attempts have been made to determine the patient parameters that predict future infertility. Current publications have focused on predictors for clinically significant varicoceles that would benefit from intervention, such as hormonal profile and peak retrograde flow. Contradictory evidence exists regarding the significance of testicular asymmetry in adolescence. SUMMARY: Substantial effort has been made to tease out the clinically significant adolescent varicocele. However, definitive evidence regarding fertility outcomes and adolescent varicocele repair remains limited and controversial. Large-scale, long-term follow-up studies are necessary to determine the true benefit of adolescent varicocele repair on decreasing the risk of fertility problems in adulthood.

Pure primary prostatic osteosarcoma arising in a non-irradiated prostate.

A 68-year-old man who initially presented with hematuria was found on prostate biopsy to have sarcoma of the prostate with osteogenic features. Radiological examination revealed a locally advanced pelvic mass involving the prostate, seminal vesicles, and rectal wall without metastatic disease. The patient underwent total pelvic exenteration with intraoperative radiotherapy. The tumor was composed of two nodules measuring 7.5 and 4.5 cm involving the prostate, both seminal vesicles, the bladder, rectum, and perirectal fibroadipose tissue. The final diagnosis was osteogenic sarcoma of the prostate.

New concepts in Klinefelter syndrome.

PURPOSE OF REVIEW: Klinefelter syndrome, 47,XXY and its variants, is the most common chromosomal aberration among men, with estimated frequency of 1: 500 among newborns. Men with Klinefelter syndrome present with sequels of hormonal and spermatogenic testicular failure like infertility, low testosterone, erectile dysfunction, and low bone mineral density. This review is aimed to provide the practicing urologist with an important source of clinically relevant information about Klinefelter syndrome. RECENT FINDINGS: Sperm can be found in over 50% of men with Klinefelter syndrome, thus men with Klinefelter syndrome are not sterile. Recent evidence suggests that children with Klinefelter syndrome are born with spermatogonia and lose large numbers of germ cells during puberty. Early diagnosis and treatment can improve the quality of life and the overall health of men with Klinefelter syndrome. SUMMARY: Growing interest in Klinefelter syndrome among translational scientists and clinicians will result in better understanding of the pathophysiology of testicular failure. In some states, screening programs for Klinefelter syndrome are already in place, which will increase the number of patients with Klinefelter syndrome seen by practicing urologists in the near future. Diagnosis and management of patients with Klinefelter syndrome is within the scope and training of urologists. Development of randomized clinical trials comparing different forms of interventions in men and children with Klinefelter syndrome will allow us to standardize the care of these patients.

Renal cell carcinoma sub-typing by histopathology and fluorescence in situ hybridization on a needle-biopsy specimen.

OBJECTIVE: To determine the subtype of renal cell carcinoma (RCC) on needle-core biopsies of renal masses using histopathology and fluorescence in situ hybridization (FISH), and to evaluate the use of interphase FISH to augment the accuracy of needle-core biopsies. PATIENTS AND METHODS: Histology correlates with prognosis in RCC but, historically, biopsies are inaccurate for histological subtype. As histological subtypes of RCC have distinct cytogenetic abnormalities (loss of 3p in clear cell, trisomy 7 or 17 in papillary and widespread chromosomal losses in chromophobe), we hypothesized that FISH would improve the accuracy of biopsies. Forty patients with renal masses underwent nephrectomy, yielding 42 tumours. Needle-core biopsies were taken of the mass immediately after surgery. Interphase FISH was performed on one core for chromosomes 3, 7, 10, 13, 17, and 21 and the locus 3p25-26. Histopathology was performed on a second core. Results were compared in a 'blinded' fashion with final pathology. RESULTS: In all, 36 of 42 masses were RCC or oncocytoma. Histopathology of the biopsy correctly identified the tumour subtype in 27 (75%), while four (11%) were incorrectly classified and five (14%) were inadequate for diagnosis. With the addition of FISH, 31 (86%) were correctly subtyped, while two (6%) were incorrect and three (8%) were inadequate. In cases with adequate tissue, histology alone was 87% accurate, while the combined method was 94% accurate. CONCLUSION: Needle-core biopsy of renal tumours provides adequate material for evaluation of histological subtype. Adding FISH to histopathology might improve the accuracy of kidney tumour biopsies, providing important prognostic information that can guide management decisions.