Evaluation of the scrotal thermal environment and the testicular sizes in prepubertal hemiscrotal agenesis without cryptorchidism.

Introduction: Hemiscrotal agenesis is a rare scrotal developmental disorder. Orchiopexy on the opposite side of the scrotum with rugae or scrotoplasty combined with orchiopexy is usually performed as a surgical treatment for hemiscrotal agenesis with cryptorchidism. Till date, there are only eight published case studies of hemiscrotal agenesis. Case presentation: A 6-year-old boy, who had been previously treated for infantile hemangiomas, was referred by a pediatrician to our hospital for the follow-up of hemiscrotal agenesis without cryptorchidism. Thermography demonstrated that the temperature of the right scrotal skin with no rugae was higher than that of the left scrotal skin with rugae. The patient's parent declined scrotoplasty because the hemiscrotal agenesis was to be followed up without scrotoplasty and scrotal ultrasonography revealed no abnormal findings in both testes. Conclusion: High-temperature environment may not impair the testicular growth in prepubertal hemiscrotal agenesis without cryptorchidism.

The first detailed annual record on the National Clinical Database Urology Division in Japan: A report on five surgical procedures.

OBJECTIVES: The National Clinical Database (NCD) Urology Division commenced registration in April 2018 in Japan. This is the first report to focus on five surgeries for which detailed information is registered. METHODS: We herein describe annual trends in and the complication grades of the following five surgeries: partial nephrectomy, radical nephrectomy, radical cystectomy, radical prostatectomy, and pyeloplasty, using the NCD. A total of 149 417 patients treated with the five types of surgeries based on NCD data were enrolled in this report. RESULTS: The number of patients was 55 630 for partial/radical nephrectomy from April 2018 to December 2021, 83 653 for radical prostatectomy from April 2018 to December 2021, and 9342 for radical cystectomy from January 2020 to December 2021. In 2021, partial nephrectomy was performed on 7416 cases, radical nephrectomy on 7739 cases, radical prostatectomy on 22 692 cases, radical cystectomy on 4677 cases, and pyeloplasty on 792 cases. CONCLUSIONS: The results obtained showed that a robot-assisted or laparoscopic procedure has replaced open surgery as the common approach for all five surgeries. An analysis of NCD data may be useful for understanding trends in surgical procedures across the major field of urology.

Combination of robot-assisted laparoscopic pyeloplasty for lower moiety ureteropelvic junction obstruction in a partial duplex system and percutaneous endoscopic surgery for renal calculi reusing the port for robotic pyeloplasty.

Introduction: Ureteropelvic junction obstruction is often associated with renal calculus formation. However, there is no report of using robot-assisted laparoscopic pyeloplasty combined with percutaneous endoscopic surgery for ureteropelvic junction obstruction and renal calculi in a partial duplex system. Case presentation: A 19-year-old female patient with lower moiety ureteropelvic junction obstruction and renal calculi in a partial duplex system was referred to our hospital because of left lumbar pain, left acute pyelonephritis, and an increase in left renal calculi during follow-up at the referral hospital. To prevent the complication of percutaneous nephrolithotripsy following pyeloplasty, robot-assisted laparoscopic pyeloplasty combined with percutaneous endoscopic surgery was performed. Two years after surgery, the patient reported no left lumbar pain. Conclusion: The combination of robot-assisted laparoscopic pyeloplasty and percutaneous endoscopic surgery can be proposed as a safe and less-invasive treatment option for ureteropelvic junction obstruction and renal calculi in a partial duplex system.

Microdeletion at ESR1 Intron 6 (DEL_6_75504) Is a Susceptibility Factor for Cryptorchidism and Hypospadias.

CONTEXT: We have previously reported that a specific "AGATC" haplotype in a >34 kb tight linkage disequilibrium (LD) block within ESR1 is strongly associated with cryptorchidism and hypospadias in Japanese boys. OBJECTIVE: We aimed to determine the true susceptibility factor for cryptorchidism and hypospadias linked to the "AGATC" haplotype. METHODS: We performed various molecular studies in hitherto unreported 230 Italian boys (80 with cryptorchidism and 150 with normal genitalia) and previously reported and newly recruited 415 Japanese boys (149 with cryptorchidism, 141 with hypospadias, and 125 with normal genitalia). We also performed ESR1 expression analyses using breast cancer-derived MCF-7 cells. RESULTS: Haplotype analysis revealed the LD block and positive association of the "AGATC" haplotype with cryptorchidism in Italian boys. Whole genome sequencing identified an identical 2249-bp microdeletion (ΔESR1) generated by a microhomology-mediated replication error in both Japanese and Italian boys with the specific haplotype. ΔESR1 was found to be strongly associated with cryptorchidism and hypospadias by Cochran-Armitage trend test and was revealed to show nearly absolute LD with the "AGATC" haplotype. ESR1 expression was upregulated in MCF-7 cells with a homozygous deletion encompassing ΔESR1 and those with a homozygous deletion involving a CTCF-binding site within ΔESR1. CONCLUSION: The results reveal that ΔESR1, which has been registered as "DEL_6_75504" in gnomAD SVs v2.1, is the true susceptibility factor for cryptorchidism and hypospadias. It appears that ΔESR1 was produced in a single ancestral founder of modern humans and has been maintained within the genome of multiple ethnic groups by selection.

Surgical management of prepubertal testicular tumors: A 30-year study in our institution.

OBJECTIVES: The 2021 European Association of Urology-European Society for Paediatric Urology guidelines on Pediatric Urology recommended testis-sparing surgery (TSS) as the primary approach to treat prepubertal testicular tumors exhibiting favorable preoperative ultrasound diagnoses. However, prepubertal testicular tumors are rare and clinical data regarding them is limited. Here, we analyzed the surgical management of prepubertal testicular tumors based on cases observed over approximately 30 years. METHODS: Data were retrospectively reviewed from medical records of consecutive patients aged <14 years with testicular tumors who received treatment at our institution between 1987 and 2020. We compared patients by their clinical characteristics, namely, those who underwent TSS versus radical orchiectomy (RO) and those who received surgery in 2005 onward versus prior to 2005. RESULTS: We identified 17 patients, with a median age at surgery of 3.2 years (range 0.6-14.0) and a median tumor size of 15 mm (range 6-67). The tumor size was significantly smaller in patients who underwent TSS than in those who underwent RO (p = 0.007). Patients treated in 2005 onward were more likely to undergo TSS than those treated prior to 2005 (71% vs. 10%, respectively), without significant differences in tumor size or the rate of preoperative ultrasound. No TSS cases required conversion to RO. CONCLUSIONS: Recent improvements in ultrasound imaging technology allow for more accurate clinical diagnosis. Therefore, the indications of TSS for prepubertal testicular tumors can be judged based not only on the tumor size but also on the diagnosis of benign tumors by preoperative ultrasound.

Conservative management of pubertal segmental testicular infarction associated with epididymitis.

Urgent scrotal exploration is performed for segmental testicular infarction (STI) because malignant testicular tumors cannot be ruled out even when STI is suspected on color Doppler ultrasound (US). This report describes the case of a 14-year-old boy who was successfully diagnosed with STI associated with epididymitis using color Doppler US to avoid radical orchiectomy. To our knowledge, this is the first report of STI being diagnosed during puberty and managed using color Doppler US and contrast-enhanced magnetic resonance imaging-guided conservative treatment.

Ureterocele prolapse eliciting unusual patient behavior to control intermittent voiding.

Ureterocele prolapse, an unusual but distinctive finding, may cause voiding difficulty. A 6-year-old male patient was referred to our hospital after his mother discovered that he tapped his lower abdomen when his urinary stream was interrupted during voiding. Voiding cystourethrography indicated a ureterocele prolapse causing the intermittency of voiding; therefore, transvesicoscopic ureteral reimplantation with ureterocelectomy was performed and the voiding consequently improved. However, this condition would not have been diagnosed had the unusual voiding behavior gone unnoticed. Therefore, diagnosing congenital bladder obstructions could be challenging if a patient adapts to a voiding difficulty.

Transvesicoscopic ureteral reimplantation and ureteroscopy for management of primary obstructed non-refluxing megaureter with ureteral calculus.

Introduction: Primary obstructed non-refluxing megaureter, a type of congenitally dilated ureter, often resolves spontaneously. Surgery may be indicated in symptomatic cases; however, there are no reports of transvesicoscopic ureteral implantation and ureteroscopy for ureteral stones. Therefore, we describe the treatment of primary obstructed non-refluxing megaureter and ureteral calculi using this technique. Case presentation: A 6-year-old Japanese girl was referred for abdominal pain and gross hematuria due to right megaureter with multiple stones in the renal lower-pole calyces and ureter. She was diagnosed with primary obstructed non-refluxing megaureter and ureterovesical junction obstruction. The stones were removed using mini-percutaneous nephrolithotomy and transvesicoureteroscopic surgery, respectively. A narrow segment of the right ureter was cut, and transvesicoscopic ureteral plication and reimplantation were performed. The procedures were successful without postoperative complications. Conclusion: Transvesicoscopic ureteral reimplantation with ureteroscopy may be a safe, effective and minimally invasive surgical option for ureterovesical junction obstruction with ureteral stones.

Pelviureteric junction obstruction of the ipsilateral kidney caused by hydronephrosis secondary to crossed fused renal ectopia.

Introduction: Crossed fused renal ectopia is rare and usually asymptomatic. However, it is associated with urological anomalies. Case presentation: A 15-year-old Japanese boy was transported to our hospital with right abdominal pain and hematuria after a soccer ball hit his right abdomen. Computed tomography revealed right hydronephrosis beyond the center of the body and no left kidney. Percutaneous nephrostomy was performed immediately, and a pyeloplasty was scheduled for 5 months later. Right hydronephrosis was noted to have been caused by left pelvic expansion due to a crossed fused ectopic kidney (secondary to a left pelviureteric junction obstruction). Subsequently, a left dismembered pyeloplasty was performed. Twenty-four months later, pain and hematuria were absent, and the creatinine level was 1.1 mg/dL. Ultrasonography revealed a shrunken right kidney. Conclusion: We encountered a unique urological anomaly with crossed fused renal ectopia. Comprehensive anatomical evaluation before surgery is important for maintaining long-term renal function.

A novel method of transvesicoscopic ureteral reimplantation of an ectopic ureter with a mate ureter in a duplex kidney.

INTRODUCTION: The reimplantation of an ectopic ureter is still performed as an open surgery, although laparoscopic or robot-assisted laparoscopic surgery has gained popularity as a minimally invasive treatment for pediatric urological disorders. CASE PRESENTATION: A 15-day-old Japanese boy was referred to our hospital with right hydronephrosis. A detailed examination revealed complete ureteral duplication on the right side and a dilated ectopic upper pole ureter, opening into the prostatic urethra. Since the patient had recurrent febrile urinary tract infections, we performed plication and ureteral reimplantation of the dilated ectopic ureter using a transvesicoscopic surgical method at the age of 2 years and 5 months. CONCLUSION: We safely performed transvesicoscopic ureteral reimplantation for an ectopic upper pole ureter with a mate ureter in a duplex kidney, following the detection of an ectopic ureter within the bladder, due to the prior understanding of the wrapping of both ureters in a common sheath.

A case of posterior urethral valve identified in an older child by straining to void.

Posterior urethral valves (PUVs) are the most common cause of congenital urethral obstruction. However, the diagnosis of a PUV is sometimes difficult. A 13-year-old Japanese boy and his mother visited our hospital, and his mother complained that he frequently strained to void although he had no complaint. Uroflowmetry revealed a plateau-shaped curve and a voiding cystourethrography (VCUG) revealed a PUV. Thus, we performed a transurethral incision of the PUV, and his voiding status improved. Because some patients with mild PUV may not notice their dysuria, we believe that VCUG should be performed without hesitation when a urethral lesion is suspected.

Antireflux endoscopic injection therapy in post-pubertal patients via techniques adopted for the dilated ureteral orifice: a retrospective single-center study.

BACKGROUND: To investigate the efficacy and safety of endoscopic injection therapy for vesicoureteral reflux in post-pubertal patients with dilated ureteral orifice via modified hydrodistension implantation techniques. METHODS: We retrospectively reviewed medical records including operational procedure and clinical course of all consecutive patients over 12 years old with a history of injection therapy. Endoscopic injection of dextranomer/hyaluronic acid copolymer was performed under hydrodistension implantation technique with some modifications in order to inject through dilated ureteral orifice align with the intramural ureter. Technical selections were done according to hydrodistension grade of the ureteral orifice. Voiding cystourethrography was evaluated at 3 months postoperatively. Hydronephrosis was evaluated using ultrasonography preoperatively until 6 months postoperatively. RESULTS: From 2016 to 2019, 12 patients (all female, 16 ureteral units; median age 32 [range 15-61] years) underwent endoscopic injection therapy at one of our institutions. We have identified grade II vesicoureteral reflux in 5 ureters, grade III in 8, and grade IV in 3 ureters. Grade 3 ureteral-orifice dilation were presented in 12 ureters (75%), grade 2 in 3 and grade 1 in 1 ureter in the present cases. Postoperatively, vesicoureteral reflux was diminished to grade 0 in 12 ureteral units (75%), decreased to grade I in 3 (9%), and remained grade III in 1 (6%). Three patients reported dull flank pain for several days postoperatively and there was 1 case of acute pyelonephritis. Temporary hydronephrosis was confirmed in 3 ureteral units (19%) at 1 month postoperatively. Median follow-up duration was 23 (range 13-63) months long. Although, 3 patients were experienced f-UTI 1-2 times, repeated VCUG showed no VUR recurrence. CONCLUSIONS: According to hydrodistension grade of the ureteral orifice, endoscopic injection therapy via modified hydrodistension implantation technique is an effective and safe treatment for vesicoureteral reflux in post-pubertal female patients with dilated ureteral orifice. While ureteral deformities or a history of anti-reflux surgery may increase the risks, these can be managed with appropriate methods that ensure sufficient mound appearance and height.

Urodynamic effectiveness of a beta-3 adrenoreceptor agonist (vibegron) for a pediatric patient with anticholinergic-resistant neurogenic detrusor overactivity: a case report.

BACKGROUND: Myelomeningocele, which causes a neurogenic bladder, is usually treated with anticholinergics in children with neurogenic detrusor overactivity (NDO); however, anticholinergics cause side effects such as dry mouth, constipation, attention deficit, and inadequate reduction in detrusor leak point pressure. Vibegron, a novel selective beta-3 adrenoreceptor agonist, is a well-established alternative to anticholinergics in adults with an overactive bladder. It remains unknown whether this agent can be used for pediatric patients. We report the case of a girl with anticholinergic-resistant NDO due to tethered cord syndrome after myelomeningocele repair, who was treated with vibegron. CASE PRESENTATION: A 4-year-old Filipino girl had increased frequency of daytime urinary incontinence and foul-smelling urine since the age of 3. Clinical examination revealed constipation, and urinalysis revealed bacteriuria. Voiding cystourethrography revealed an enlarged and trabeculated bladder without vesicoureteral reflux. On the urodynamic study (UDS), she was found to have detrusor overactivity (DO) and low bladder compliance. She could not void and was diagnosed with overflow incontinence. Clean intermittent catheterization (CIC) and orally administered propiverine (0.8 mg/kg/day) were initiated, and urinary incontinence was resolved. She underwent a UDS annually; the UDS at 6 years of age still revealed DO and low bladder compliance in spite of receiving propiverine. The treatment was switched from propiverine to vibegron (1.4 mg/kg/day). On the UDS after a 5-week treatment schedule of vibegron, the DO disappeared and the bladder compliance improved. CIC and orally administered vibegron have been continued for 7 months so far, and she has had no urinary tract infection with no drug-related adverse events. CONCLUSIONS: Vibegron was effective and well tolerated in the treatment of a pediatric patient with NDO. Vibegron improved the urodynamic parameters for anticholinergic-resistant neurogenic bladder. This agent can be a beneficial and preferable alternative therapeutic agent to anticholinergics in patients with anticholinergic-resistant NDO.

Genetic and histopathological analysis of transverse testicular ectopia without persistent Müllerian duct syndrome: two case reports.

BACKGROUND: Transverse testicular ectopia (TTE) is a rare anomaly in which both testes descend through a single inguinal canal into the same hemiscrotum. Although almost 20-50% of patients with TTE exhibit persistent Müllerian duct syndrome (PMDS) and many genetic analyses have been performed, no reports have described the genes contributing to TTE without PMDS. Here, we report two cases of TTE without PMDS using immunohistochemical staining and genetic analysis. CASE PRESENTATION: Two Asian patients with TTE without PMDS were subjected to orchiopexy. We performed testicular biopsies during operation and obtained blood samples before the operation. Testicular tissues were stained for c-kit, placental alkaline phosphatase (PLAP), and undifferentiated embryonic cell transcription factor 1 (UTF1) to evaluate the presence of intratubular malignant germ cells. Additionally, we performed polymerase chain reaction-based direct sequencing to identify single nucleotide polymorphisms in genes associated with regression of the Müllerian duct and testicular descent (that is, anti-Müllerian hormone [AMH], AMH receptor 2 [AMHR2], insulin-like 3 [INSL3], and relaxin family peptide receptor 2 [RXFP2]). The three-dimensional structures of proteins were predicted using SWISS-MODEL. In immunohistochemical analysis, c-kit and UTF1 were positive, whereas PLAP was negative in three testicular tissue samples from the two patients. These features were also detected on the unaffected side. In variant analysis, common missense variants in the AMH gene (g.365G>T; c.165G>T; p.Ser49Ile [rs10407022]) were observed. All variants in INSL3 and RXFP2 genes were intronic or silent. CONCLUSIONS: Because UTF1, a specific marker of spermatogonial stem cell activity, was expressed in both the affected and unaffected sides in the testicular tissues of two patients, the risk of malignancy may be high in these patients. Although the etiology of TTE without PMDS remains unclear, our variant analysis results were consistent with previous reports, and variants in the AMH gene (rs10407022) may contribute to the specific phenotype of TTE without PMDS.

Clozapine-induced antineutrophil cytoplasmic antibody-associated vasculitis: a case report.

Clozapine is the most effective antipsychotic medication for refractory schizophrenia, but it has many possible serious side effects, including antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). However, the rare case reports available have not presented sufficient characteristic features of drug-induced AAV. Herein, we report a case of a 48-year-old Japanese woman with schizophrenia who presented with fever, arthralgia, myalgia and skin rash after 2 years of clozapine treatment. Her C-reactive protein (CRP) level increased, myeloperoxidase ANCA was positive and skin biopsy revealed leukocytoclastic vasculitis. Initially, steroid administration achieved remission, but her symptoms and high CRP levels relapsed every time the steroid dosage was tapered down. Upon discontinuation of clozapine, her symptoms and elevated CRP level immediately improved and the steroid was successfully tapered and discontinued. This outcome suggested that clozapine was the main cause of AAV.

Robot-Assisted Radical Cystectomy for Pediatric Bladder Rhabdomyosarcoma.

Background: The standard treatments for muscle-invasive bladder cancer with no metastasis are total cystectomy and urinary diversion. Although robot-assisted radical cystectomy (RARC) was covered from April 2018 by the Japanese National Health Insurance system, and the number of RARC is increasing, there has been no pediatric case report on RARC in Japan. Case Presentation: We report the case of a 6-year-old Japanese girl who was referred to our hospital with the chief complaint of a vulvar tumor protrusion during defecation. We resected the tumor from her external urethral meatus, and transurethral resection for the residual partial bladder neck tumor was performed for both a definitive diagnosis and as a possible curative therapy. The pathologic diagnosis was an embryonic type of rhabdomyosarcoma. Although she was treated by chemotherapy combined with proton therapy, a residual tumor at the neck and a new lesion at the top of bladder were observed 2 years after initial treatment. Thus, RARC was performed. The surgical specimen was placed in an end-pouch and was then removed through the incised vaginal wall, with cosmetic consideration. A bilateral cutaneous ureterostomy was performed at the sites of the working ports for urinary diversion. In the future, we plan to perform abdominal wall catheterization. Postoperatively, she was treated with adjuvant chemotherapy. There was no recurrence for 19 months. Conclusion: Because she was a child, particularly a girl, the wounds should be small and inconspicuous considering the cosmetic aspect. Although the posterior aspect of the bladder seemed difficult to detach because of the adhesions, it was possible to safely perform RARC.

Detailed presurgical evaluation of a case of congenital bladder diverticulum.

Congenital bladder diverticulum is rare and is usually observed in male children. An 8-year-old Japanese boy was referred to a hospital with fever and gross hematuria and was treated with a course of antibiotics. Because the dilatation of the ureter was suspected by abdominal ultrasonographic examination, he was referred to our hospital. We diagnosed congenital bladder diverticulum by computed tomographic scan and carried out laparoscopic bladder diverticulectomy and extravesical ureteral reimplantation. Four years later, there was no recurrence of gross hematuria or urinary tract infection.

Active Phagocytosis and Diachronic Processing of Calcium Oxalate Monohydrate Crystals in an in vitro Macrophage Model.

BACKGROUND: We previously discovered that renal macrophages (Mφs) phagocytose renal calcium oxalate monohydrate (COM) crystals. This study investigated the processing of engulfed crystals using in vitro models. METHODS: J774.1 mouse Mφs were exposed to COM crystals and observed for 24 h using polarized light microscopy with/without cytochalasin B (CB), an inhibitor of phagocytosis, to confirm active crystal phagocytosis. LysoTracker and immunohistochemical staining using transmission electron microscopy for lysosomal-associated membrane protein 1 were used to confirm engulfed COM crystal uptake into lysosomes. Diachronic tracking of specific Mφs was performed to capture the entire course of engulfed COM crystal processing using polarized light microscopy. Follow-up studies of fluorescent COM (f-COM) crystals using imaging cytometry were performed in the presence and absence of nigericin to dissipate the pH gradient in acidic organelles. RESULTS: Phagocytosis rates increased with COM density and were significantly lower in cells treated with CB (p < 0.01). We observed that engulfed crystals colocalized within lysosomes of the Mφs; moreover, diachronic observation indicated that the engulfed COM crystals were subdivided during Mφ division and eliminated by the 7th day of culture. Additionally, imaging cytometry showed that the fluorescence level of f-COM crystals in the nigericin (-) group after 48 h was significantly lower than that in the nigericin (+) group. CONCLUSIONS: This study confirmed active phagocytosis and lysosomal processing of engulfed COM crystals by Mφs. This discovery is expected to contribute to the development of future drugs that enhance the COM crystal phagocytic ability of Mφs.

Improvement in early urinary continence recovery after robotic-assisted radical prostatectomy based on postoperative pelvic anatomic features: a retrospective review.

BACKGROUND: We investigated the impact of postoperative membranous urethral length and other anatomic characteristics of the pelvic floor shape as measured by magnetic resonance imaging on the improvement in continence following robotic-assisted radical prostatectomy. METHODS: We retrospectively reviewed data from 73 patients who underwent postoperative prostate magnetic resonance imaging following robotic-assisted radical prostatectomy between 2013 and 2018. Patient demographics; pre-, peri-, and post-operative parameters; and pelvic anatomic features on magnetic resonance imaging were reviewed. Patients who used no urinary incontinence pads or pads for protection were considered to have achieved complete continence. RESULTS: Urinary continence was restored in 27.4, 53.4, 68.5, and 84.9% of patients at 1, 3, 6, and 12 months after robotic-assisted radical prostatectomy, respectively. When patients were divided into early and late continence groups based on urinary continence at 3 months after robotic-assisted radical prostatectomy, no significantly different clinical characteristics or surgical outcomes were found. However, the mean membranous urethral length (18.5 mm for the early continence group vs. 16.9 mm for the late continence group), levator muscle width (7.1 vs. 6.5 mm, respectively), and bladder neck width on the trigone side (7.2 mm vs. 5.4 mm, respectively) were significantly different between groups (all p < 0.05). Multivariate logistic regression analysis showed that membranous urethral length (odds ratio, 1.227; 95% confidence interval, 1.011-1.489; p = 0.038) and bladder neck width (odds ratio, 1.585; 95% confidence interval, 1.050-2.393; p = 0.028) were associated with the period of early urinary continence. CONCLUSIONS: Postoperative membranous urethral length and bladder neck width were significantly associated with early urinary continence recovery after robotic-assisted radical prostatectomy. It is highly recommended that surgeons focus on preserving the membranous urethral length and increasing the bladder neck width on the trigone side during surgery to achieve optimal continence outcomes after robotic-assisted radical prostatectomy.