Undescended testis: A roundtable discussion based on clinical scenarios - Part 2.

Undescended testis (UDT, cryptorchidism) is the most frequent genital anomaly in boys. However, its treatment varies widely throughout the world. This second part of our roundtable discussion aims to continue to ask global experts to express their attitudes towards several case scenarios of UDT in order to explore the rationale for their clinical decisions. As the European Association of Urology - Young Academic Urologists Pediatric Urology Working Group, we believe that this roundtable series will facilitate colleagues all over the world to reflect and improve their practices regarding the treatment of UDT.

[A Case of Testicular Torsion of Retractile Testis Requiring Orchidectomy].

We report a case of testicular torsion in an 8-year-old who was referred to our hospital for right groin pain. He was diagnosed with right retractile testis during a 12-month check-up. However, instead of performing orchiopexy, he was placed under observation until the age of 5, after which he did not seek medical attention. Physical examination revealed swelling and tenderness in the right inguinal region and no palpable testis in the right scrotum. Ultrasound and computed tomography revealed right testicular torsion, and emergency surgery was performed. Intraoperative findings revealed a dark and ischemic testis that was twisted at 180°in the right inguinal region. There was no improvement in blood flow even after the testicular torsion was released; therefore, right orchidectomy with left orchiopexy was performed. Although the incidence of testicular torsion is higher in patients with an undescended testis than in those with a normally positioned scrotal position testis, reports of testicular torsion associated with a retractile testis are rare.

Undescended testis: A roundtable discussion based on clinical scenarios - Part 1.

Undescended testis (UDT, cryptorchidism) is the most common congenital anomaly of the genital tract. Despite its high incidence, the management of UDT varies between specialties (urology, pediatric surgery, pediatric urology, pediatric endocrinology). Therefore, as the European Association of Urology - Young Academic Urologists Pediatric Urology Working Group, we requested experts around the world to express their own personal approaches against various case scenarios of UDT in order to explore their individual reasoning. We intended to broaden the perspectives of our colleagues who deal with the treatment of this frequent genital malformation.

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.

Comparison of the short-term efficacy and safety of bipolar transurethral electro vaporization and holmium laser enucleation of the prostate for moderate and large benign prostatic enlargement.

BACKGROUND: To compare the efficacy and safety of bipolar transurethral vaporization of the prostate (B-TUVP) with holmium laser enucleation of the prostate (HoLEP) for moderate [prostate volume (PV) 30-80 ml] and large (≥ 80 ml) benign prostatic enlargement (BPE). MATERIALS AND METHODS: Male patients with lower urinary tract symptom (LUTS) or urinary retention who underwent B-TUVP or HoLEP treatment in two regional centers were respectively enrolled. Patient characteristics and treatment outcomes were retrospectively compared between B-TUVP and HoLEP. RESULTS: In patients with moderate and large prostate volume,B-TUVP showed shorter operative time (P < 0.001) and less hemoglobin decrease (P < 0.001) than in HoLEP. In uncatheterised patients, voiding symptoms and patients' quality of life improved after B-TUVP and HoLEP, but these improvement rates were consistently bigger in HoLEP than in B-TUVP. In catheterised patients, the rate of achieving catheter-free status after surgery was higher in HoLEP than in B-TUVP for patients with PV > 80 ml.(P < 0.001) The incidence of postoperative fever was higher in B-TUVP than in HoLEP for patients with PV 30-80 ml (P < 0.001) but not for those with PV > 80 ml.(P=0.08) The Incidence of postoperative stress incontinence(SUI) was higher in HoLEP than in B-TUVP for patients with moderate and large prostate volume. CONCLUSIONS: There are few studies that investigated the short-term efficacy and safety of second-generation B-TUVP in comparison with HoLEP for moderate and large BPE. Improvement in LUTS and achievement of catheter-free status were predominant in HoLEP, and these outcomes were more prominent in patients with large BPE of PV > 80 ml. However, B-TUVP resulted in less blood loss, shorter operative duration, and less SUI suggesting that B-TUVP is also well-tolerated surgical modality.

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.

BMP10 expression in the adult rat central nervous system.

Bone morphogenetic protein 10 (BMP10), is a member of the transforming growth factor ß (TGFß) superfamily. Although BMP10 plays pivotal roles during development, including vascular development and cardiogenesis, little information is available for BMP10 expression in the central nervous system (CNS). We, thus, investigated BMP10 expression in the adult rat CNS using immunohistochemistry. BMP10 was intensely expressed in most neurons and their axons. Furthermore, we found that astrocytes and ependymal cells also express BMP10 protein. These data indicate that BMP10 is widely expressed throughout the adult CNS, and this abundant expression strongly supports the idea that BMP10 also plays important roles in the adult CNS.

[A Case of Dedifferentiated Liposarcoma of the Spermatic Cord].

A 52-year-old man was referred to our hospital for evaluation of painless right scrotal swelling persisting for 3 months. Palpation detected swelling and induration centered on the head of the upper epididymis, and ultrasonography revealed a blood-filled nodular mass at the same site continuing to the spermatic cord. No abnormalities were observed in the bilateral testes. Blood tests were negative for tumor markers such as α fetoprotein and human chorionic gonadotropin-ß. Right radical inguinal orchiectomy was performed, and the pathological diagnosis was dedifferentiated liposarcoma of the spermatic cord. Although the spermatic cord stump was negative, the peri-spermatic cord stump, which had an exfoliated surface, was positive. No residual tumor was found on magnetic resonance imaging, but the tumor was suspected to remain. Thus, after approximately 1month, the tissue around the spermatic cord was resected. Eight months after the initial operation, no recurrence was observed. Here, we report a case of dedifferentiated liposarcoma of the spermatic cord, which is relatively rare, and review the related literature.

A Novel Transvesicoscopic Ureteral Reimplantation Technique to Treat an Infantile Ectopic Ureter in a Single System.

An ectopic ureter is a significant urinary tract malformation often treated during early childhood and is surgically managed based on its condition. Generally, only extravesical ureteral reimplantation is available as a treatment option for an ectopic ureter with well-preserved renal function. This report describes a case of a 28-months-old girl with an ectopic ureter in a single system who experienced repeated febrile urinary tract infections, successfully treated using a transvesicoscopic ureteral reimplantation as a minimally invasive treatment. To the best of our knowledge, this is the first report of transvesicoscopic ureteral reimplantation for an ectopic ureter in a single system.

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.

Low Serum Inhibin B/Follicle-Stimulating Hormones and Anti-Müllerian Hormone/Follicle-Stimulating Hormones Ratios as Markers of Decreased Germ Cells in Infants with Bilateral Cryptorchidism.

PURPOSE: In cryptorchidism, germ cell development failure presents from infancy and may be reflected by altered hormonal levels produced by Sertoli cells. Our object was to assess for associations between serum hormone levels and testicular histopathology in cryptorchidism with an infertility risk according to the pretreatment undescended testicular positions. MATERIALS AND METHODS: Prepubertal cryptorchid boys aged 7-91 (median 20) months who underwent orchidopexy between 2014 and 2019 were included (122 unilateral [median 19 months {range 7-91}], 23 bilateral [24 months {11-81}]). We evaluated the pretreatment testicular position and size; serum hormone levels; and the mean number of germ cells per tubule transverse section (G/T). We also performed a subgroup analysis of boys aged ≤24 months at orchidopexy. RESULTS: Serum inhibin B levels and G/T were significantly lower in bilateral than in unilateral cryptorchid boys (median 96 [range 46-197] pg/ml vs 125 [21-354] pg/ml, p=0.026; 0.20 [0-2.59] vs 0.65 [0-4.55], p <0.001, respectively). Inhibin B/follicle-stimulating hormones (FSH) and anti-Müllerian hormone (AMH)/FSH ratios were positively correlated with G/T in bilateral cryptorchid boys aged ≤24 months (12, p=0.008 and p=0.019, respectively). Low inhibin B/FSH and AMH/FSH ratios and high FSH were predictors of impaired G/T as per receiver operating characteristic curves (p=0.019, p=0.004 and p=0.004, respectively), whereas in unilateral cryptorchid boys aged ≤24 months, serum hormone levels and G/T did not differ with the pretreatment testicular positions. CONCLUSIONS: In bilateral cryptorchid boys aged ≤24 months at orchidopexy, low inhibin B/FSH and AMH/FSH ratios may reflect impaired G/T and future infertility risk.

[Clear Cell Variant of Invasive Urothelial Carcinoma of the Renal Pelvis: A Case Report].

A 74-year-old woman with gross hematuria for 3 months was referred to our hospital. Contrast-enhanced computed tomographic scan showed a mass on the upper-right renal calyx, and retrograde pyelography showed stenoses from the renal pelvis to the renal calyx infundibulum. We performed an endoscopic biopsy, which led to a diagnosis of urothelial cancer. Therefore, she underwent total right nephroureterectomy, and pathological examination revealed a clear cell variant of invasive urothelial carcinoma. Irradiation was performed mainly on the renal arteriovenous stump 2 months postoperatively; subsequently, three courses of combination chemotherapy comprising gemcitabine plus cisplatin (GC) were administered. Port-site recurrence and pelvic recurrence were observed 22 months after the operation, and GC therapy and pembrolizumab were administered. However she died 36 months after the operation. The clear cell variant of invasive urothelial carcinoma of the upper urethra is rarely reported. Moreover, since this was a very rare case, we have included a literature review in our report.