Identifying infrequent genetic changes in monozygotic twins afflicted with hypospadias via targeted panel sequencing.

PURPOSE: We aimed to identify the genetic causes of hypospadias in children using targeted gene panel sequencing for disorders of sex development (DSD). MATERIALS AND METHODS: This study included 18 twin boys with hypospadias: seven and two pairs were monozygotic and dizygotic twins, respectively, and six were discordant and three were concordant twins. Targeted gene panel sequencing for 67 known DSD genes was performed. Sequence variants were classified into five different categories, pathogenic, likely pathogenic, variants of uncertain significance, likely benign, and benign, following the American College of Medical Genetics and Genomics Standards and Guidelines. RESULTS: The mean gestational age and birth weight were 35.3±2.0 weeks and 1.96±0.61 kg, respectively, with seven patients being small for gestational age. Hypospadias was present in 12 patients, with posterior type in 33.3% and anterior type in 66.7%. In three families with twins, both siblings had hypospadias. In addition, cryptorchidism was observed in one subject. Surgical correction of hypospadias was performed at a mean age of 22.1 months. Molecular analysis identified 12 different genetic variants, including two pathogenic mutations in the AMH (p.E389*) and SRD5A2 (p.R246Q) genes, found in subjects with hypospadias, respectively. However, only heterozygous mutations were detected. CONCLUSIONS: This study did not identify a definitive genetic component contributing to the development of hypospadias; however, the findings suggest that intrauterine growth retardation may play a significant role.

A Comparative Analysis of Robot-Assisted Laparoscopic Pyeloplasty in Pediatric and Adult Patients: Does Age Matter?

We investigated factors that affect the surgical outcomes of robotic pyeloplasty by comparing the surgical results of pediatric and adult patients with ureteropelvic junction stricture (UPJO). We retrospectively reviewed patients who underwent robotic pyeloplasty for UPJO between January 2013 and February 2022. The patients were categorized into two groups: the pediatric (≤18 years) and adult (>18 years) groups. The perioperative and postoperative outcomes and surgical complications were comparatively analyzed. Prognostic factors for predicting surgical failure were analyzed with multivariable logistic regression analysis. The pediatric group showed longer total operation and console times. The mean pain score was lower in the pediatric group than in the adult group on days 1 and 2 after surgery. The average amount of morphine used in the pediatric group was lower during postoperative days 0−2. No differences in the length of hospital stay, incidence of surgical failure, and incidence of urolithiasis requiring treatment after robotic pyeloplasty were observed between the groups. The only factor that predicted surgical failure was a history of urolithiasis before surgery. The results showed that age did not affect the surgical outcome.

Deep-learning segmentation of ultrasound images for automated calculation of the hydronephrosis area to renal parenchyma ratio.

PURPOSE: We investigated the feasibility of measuring the hydronephrosis area to renal parenchyma (HARP) ratio from ultrasound images using a deep-learning network. MATERIALS AND METHODS: The coronal renal ultrasound images of 195 pediatric and adolescent patients who underwent pyeloplasty to repair ureteropelvic junction obstruction were retrospectively reviewed. After excluding cases without a representative longitudinal renal image, we used a dataset of 168 images for deep-learning segmentation. Ten novel networks, such as combinations of DeepLabV3+ and UNet++, were assessed for their ability to calculate hydronephrosis and kidney areas, and the ensemble method was applied for further improvement. By dividing the image set into four, cross-validation was conducted, and the segmentation performance of the deep-learning network was evaluated using sensitivity, specificity, and dice similarity coefficients by comparison with the manually traced area. RESULTS: All 10 networks and ensemble methods showed good visual correlation with the manually traced kidney and hydronephrosis areas. The dice similarity coefficient of the 10-model ensemble was 0.9108 on average, and the best 5-model ensemble had a dice similarity coefficient of 0.9113 on average. We included patients with severe hydronephrosis who underwent renal ultrasonography at a single institution; thus, external validation of our algorithm in a heterogeneous ultrasonography examination setup with a diverse set of instruments is recommended. CONCLUSIONS: Deep-learning-based calculation of the HARP ratio is feasible and showed high accuracy for imaging of the severity of hydronephrosis using ultrasonography. This algorithm can help physicians make more accurate and reproducible diagnoses of hydronephrosis using ultrasonography.

Development and Validation of a Scoring System for Assessment of Clinical Failure after Pediatric Robot-Assisted Laparoscopic Extravesical Ureteral Reimplantation: A Multi-Center Study.

We aimed to develop and validate a scoring system as an objective assessment tool for predicting clinical failure after pediatric robotic extravesical ureteral reimplantation. Data for this multi-institutional retrospective cohort was obtained from two tertiary referral hospitals. We defined clinical failure as incomplete radiographic resolution or post-operative febrile UTI. Patients were stratified into low, intermediate, and high-risk groups according to the score. External validation was performed using the model projected to the external validation cohort. An amount of 115 renal units in the development cohort and 46 renal units in the validation cohort were analyzed. The prediction score was calculated with weighted points to each variable according to their regression coefficient as age (year) + BMI + BBD times 10 + VUR grade times 7 + console time (h) + hospital stay times 6. The C-index of our scoring system was 0.850 and 0.770 in the development and validation cohorts, respectively. Clinical failure was significantly different among risk groups: 0% (low-risk), 3.3% (intermediate-risk), and 22.2% (high-risk) (p = 0.004) in the development cohort. A novel scoring system using multiple pre- and intra-operative variables provides a prediction of children at risk of failure after robotic extravesical ureteral reimplantation.

Surgical Outcomes of Clitoroplasty in Children with Congenital Adrenal Hyperplasia and Clitoral Hypertrophy: A 19-Year Experience of a Single Surgeon.

This study aimed to describe the experience with clitoroplasty for clitoral hypertrophy in patients with congenital adrenal hyperplasia of a single surgeon. The medical records of female pediatric patients with congenital adrenal hyperplasia who underwent clitoroplasty at a tertiary referral hospital between 2002 and 2020 were retrospectively analyzed. Three different surgical techniques were applied for clitoroplasty: recession without reduction, reduction and recession, and girth reduction and recession. A total of 104 patients underwent clitoroplasty for clitoral hypertrophy. The median patient age at the time of surgery was 10 months (range, 4 months to 10 years). The operation time was longer in reduction clitoroplasty than in recession clitoroplasty without reduction (median, 153 vs. 111 min, p = 0.003). The mean postoperative pain score of the patients did not differ among the different clitoroplasty techniques. During the mean follow-up of 37.7 months, nine (8.6%) patients underwent reperformed clitoroplasty. The rate of reperformed operation was significantly higher in patients who underwent reduction clitoroplasty (17.3%) than in those who underwent recession without reduction (2%) or girth reduction and recession (0%) (p = 0.031). Early clitoroplasty in patients with congenital adrenal hyperplasia yielded good mid-term surgical outcomes in terms of cosmesis and recurrence rate, with minimal perioperative complications.

A New Case of Herlyn-Werner-Wunderlich Syndrome: Uterine Didelphys with Unilateral Cervical Dysgenesis, Vaginal Agenesis, Cervical Distal Ureteral Remnant Fistula, Ureterocele, and Renal Agenesis in a Patient with Contralateral Multicystic Dysplastic Kidney.

The aim of this study was to present a new case of congenital Herlyn-Werner-Wunderlich syndrome, a rare anomaly of the female reproductive tract, and review the related literature. A 12-year-old girl presented with severe dysmenorrhea since menarche and magnetic resonance imaging showing a bicornuate uterus, double cervix, right hematometra, and hematosalpinx with ipsilateral renal agenesis, accompanied by a remnant distal ureter with hydroureter. A diagnostic cystoscopy and a reduced-port robot-assisted laparoscopy with chromopertubation were performed in order to identify the anomaly. Uterine didelphys and right cervical dysgenesis with ipsilateral vaginal agenesis, cervical distal ureteral remnant fistula, ureterocele, and renal agenesis were diagnosed on the basis of histopathologic findings, and she subsequently underwent a robotic unilateral right total hysterectomy with salpingectomy. This case report reinforces the importance of the intraoperative biopsy for an accurate diagnosis, despite magnetic resonance imaging being considered the gold-standard diagnostic tool.

Preoperative Bladder Bowel Dysfunction Is the Most Important Predictive Factor for Postoperative Urinary Retention After Robot-Assisted Laparoscopic Ureteral Reimplantation via An Extravesical Approach: A Multi-Center Study.

Introduction: Postoperative acute urinary retention (pAUR) is a known occurrence after robot-assisted laparoscopic ureteral reimplantation via an extravesical approach (RALUR-EV). We hypothesized that the risk factor of pAUR after RALUR-EV might be similar to that of pAUR after open reimplantation. We aimed at performing a retrospective multi-institutional study to evaluate the risk factors for pAUR after RALUR-EV. Materials and Methods: Perioperative data collected from two tertiary referral hospitals included demographics and perioperative variables such as bladder bowel dysfunction (BBD) status, vesicoureteral reflux (VUR) grade, and laterality. pAUR was defined as the need for urethral catheter replacement after removal of the initial postoperative catheter. Univariate and multivariate analyses were performed to identify risk factors for pAUR. Results: A total of 117 patients with 174 renal units from the 2 hospitals were enrolled in this study. The median age at the time of surgery was 5 (0.3-19) years. Bilateral RALUR-EV was performed in 57 (48.7%) cases. pAUR rate was 3.4% in all patients and 7.0% in 57 patients with bilateral VUR. All four cases of pAUR occurred after bilateral surgery. Univariate analysis showed age (p = 0.037), weight (p = 0.039), height (p = 0.040), and bilaterality (p = 0.037) as risk factors of pAUR. In a multivariate analysis, BBD was the only significant risk factor of pAUR (p = 0.037). Conclusion: Urinary retention after RALUR-EV occurred less frequently when compared with the previously reported open surgery series. pAUR was seen only in bilateral cases in our series. Preoperative history of BBD, but not male gender or length of surgical time, was the only risk factor of pAUR after RALUR-EV.

Efficacy of additional tunica vaginalis flap coverage for protecting against urethrocutaneous fistulas in tubularized incised plate urethroplasty: A prospective, randomized controlled trial.

PURPOSE: We compared the protective effect of additional tunica vaginalis flap coverage combined with a dartos flap against urethrocutaneous fistulas in tubularized incised plate (TIP) urethroplasty in a randomized controlled trial. MATERIALS AND METHODS: This prospective, randomized controlled trial in a single tertiary center enrolled 50 patients in whom it was feasible to perform single TIP urethroplasty between 2016 and 2017. Consecutive children were randomly allocated to study group A (additional tunica vaginalis flap coverage, n=25) or control group B (dartos-only coverage, n=25). All patients were examined in the outpatient clinic at 1, 3, 12, and 24 months. Postoperative cosmetic outcomes were evaluated by surgeons and parents using the Pediatric Penile Perception Scale questionnaire. RESULTS: In group B, 1 of 25 patients (4.0%) developed an urethrocutaneous fistula within 12 months. An additional two cases of fistula were found in all proximal-type hypospadias patients at 24 months in the same group without statistical significance (p=0.07). The penile cosmetic satisfaction rate was not significantly different between the groups according to scores on the Pediatric Penile Perception Scale. CONCLUSIONS: Our randomized controlled trial did not show a significant decrease in the incidence of or a significant slowing of the progression of postoperative fistulas after TIP urethroplasty by the use of additional tunica vaginalis coverage. A tunica vaginalis flap is not routinely recommended but could have a selective role in proximal-type TIP urethroplasty with deficient dartos and subcutaneous tissue to cover the neourethra.

Best ultrasound parameter for prediction of adverse renal function outcome after pyeloplasty.

OBJECTIVE: To evaluate the clinical value of preoperative ultrasound parameters for post-pyeloplasty outcomes in pediatric patients with ureteropelvic junction obstruction. METHODS: The medical records of 187 pediatric and adolescent patients who underwent pyeloplasty as a result of ureteropelvic junction obstruction between 2010 and 2016 were retrospectively reviewed. The severity of hydronephrosis was measured by the Society for Fetal Urology grade, anteroposterior pelvic diameter, urinary tract dilation, hydronephrosis index, and the hydronephrosis area to renal parenchyma ratio at 3, 6 and 12 months. Adverse renal function outcome was defined as ≥10% decrease in postoperative differential renal function compared with preoperative values. RESULTS: Of the 187 patients, preoperative hydronephrosis was categorized as Society for Fetal Urology grade 3 in 26 patients (13.9%) and grade 4 in 161 patients (86.1%). No surgical failures, defined as requirement of repeat surgery or deterioration of hydronephrosis grade, were noted. The mean changes in Society for Fetal Urology grade, anteroposterior pelvic diameter, urinary tract dilation and hydronephrosis area to renal parenchyma ratio showed similar trends of recovery during the follow-up period. In total, 19 patients (10.2%) showed >10% decrease in differential renal function during follow up (mean 42 months). Multivariate logistic regression analysis showed that the hydronephrosis area-to-renal parenchyma ratio was the only significant prognostic factor for adverse renal function outcome (hazard ratio 1.806, 95% confidence interval 1.210-2.859, P = 0.005). Receiver operating characteristic analysis showed that the hydronephrosis area-to-renal parenchyma ratio was the most significant predictive value (area under the curve 0.711, 95% confidence interval 0.618-0.804, P = 0.006). CONCLUSIONS: Pediatric patients with high hydronephrosis area-to-renal parenchyma ratio values before surgery are more likely to show renal function decline after pyeloplasty.

Validity and reliability of a home-based, guardian-conducted video voiding test for voiding evaluation after hypospadias surgery.

Purpose: To investigate the validity and reliability of a home-based, guardian-conducted video voiding test for assessing postoperative voiding function after hypospadias surgery. Materials and Methods: In a single center, patients who had undergone urethroplasty by a single surgeon and postoperative uroflowmetry and video voiding tests conducted between 2008 and 2016 were retrospectively reviewed. Urinary stream was categorized into five grades by three pediatric urologists in a blinded manner. The primary outcome was statistical correlation across raters as measured by Spearman correlation coefficient to validate the interpretation of the video voiding test. The secondary outcome was the reliability of the voiding video test compared with maximum urinary flow rate assessed by uroflowmetry. Results: Thirty-one patients with hypospadias were enrolled. The patients' average ages were 12.3±3.2 months (range, 8-21 months) and 42.8±3.9 months (range, 35-48 months) at the time of surgery and voiding video tests, respectively. Hypospadias was anterior, penile, and proximal in 1 (3.2%), 18 (58.1%), and 12 (38.7%) patients, respectively. The number of patients with each voiding stream grade was as follows: very poor, 4; poor, 4; fair, 13; good, 4; and very good, 6. All intraclass correlation coefficients of the stream grade among the three observers were >0.95. Correlation between the maximum flow rate obtained by use of conventional uroflowmetry and the video voiding stream grade was validated (rho 0.778, p<0.001). Conclusions: The home-based guardian-conducted video voiding test is easy to perform and the present results demonstrate its validity and reliability for assessing patients' post-urethroplasty voiding pattern.

Utility of Society for Fetal Urology and Anteroposterior Pelvic Diameter Grading Systems for Estimating Time to Resolution of Isolated Hydronephrosis: A Single Center Study.

PURPOSE: We aimed to compare the Society for Fetal Urology and anteroposterior pelvic diameter classification systems in predicting the time to resolution of isolated hydronephrosis. MATERIALS AND METHODS: We retrospectively reviewed isolated hydronephrosis cases prenatally detected and postnatally diagnosed between 1994 and 2018. Other urinary tract anomalies and vesicoureteral reflux were excluded. Baseline grades for both systems were collected. Anteroposterior pelvic diameter was classified as grade 1 to 4 over a scale of 5 mm. Resolution was defined by an anteroposterior pelvic diameter of less than 5 mm with Society for Fetal Urology grade I hydronephrosis. Time to resolution was analyzed using Kaplan-Meier curves. RESULTS: The study population consisted of 831 patients (1,028 renal units). Median followup was 26 months (range 6 to 260), and 559 units (54.3%) reached resolution during a median followup of 9 months (1 to 133). Resolution rates at 48 months were 81.7%, 65.6%, 37.6% and 5.2% for Society for Fetal Urology grades I through IV disease and 80.0%, 41.2%, 13.1% and 2.5% for anteroposterior pelvic diameter grades 1 through 4, respectively. Discrepancies between these grades were present in 481 units (46.8%). Society for Fetal Urology grades II to IV categorized as anteroposterior pelvic diameter grade 1 showed a superior cumulative rate of resolution (84.7%) vs anteroposterior pelvic diameter grades 2 to 4 categorized as Society for Fetal Urology grade I (53.2%, log-rank test p <0.001). CONCLUSIONS: Hydronephrosis cases with anteroposterior pelvic diameter grades 2 to 4 but categorized as Society for Fetal Urology grade I have an inferior cumulative rate of resolution than vice versa. Society for Fetal Urology grades have little influence on resolution when anteroposterior pelvic diameter is less than 10 mm. Therefore, anteroposterior pelvic diameter is more useful in predicting the time to resolution.

Genomic Landscape of Young-Onset Bladder Cancer and Its Prognostic Implications on Adult Bladder Cancer.

Due to the rare occurrence of young-onset bladder cancer (YBC), its genomic characteristics remain largely unknown. Twenty-nine biopsy-proven YBC cases were collected using a nation-wide search for bladder cancer diagnosed at 20 years or younger. Whole exome sequencing and RNA sequencing were carried out in 21 and 11 cases, respectively, and compared with those of adult bladder cancer (ABC) cases obtained from public databases. Almost all YBCs were low grade, non-invasive papillary tumors. YBC had a low mutation burden and less complex copy number alterations. All cases harbored putative driver mutations. Mutations were most commonly found in HRAS (10 cases), with a preference for exon 5. FGFR3 gene fusions were noted with various partner genes (7 cases). The alterations on HRAS and FGFR3 occurred in a mutually exclusive manner. Others included KRAS mutations (2 cases), chromosomes 4p and 10q arm-level deletions (1 case), and ERCC2 mutation (1 case). There were no point mutations in TP53 and FGFR3. The gene expression profiles of YBC were similar to those of the ABC group with good prognosis. None of the YBCs and ABCs with YBC-like mutations showed progression to muscle-invasive tumors. Our results suggest that bladder cancer with YBC-like mutations represents an indolent bladder tumor, regardless of age.

Impact of de novo vesicoureteral reflux on transurethral surgery outcomes in pediatric patients with ureteroceles.

Purpose: We aimed to determine the impact of de novo vesicoureteral reflux (VUR) on postoperative urinary tract infection (UTI) and renal function in pediatric patients with ureteroceles. Materials and Methods: We retrospectively reviewed the medical records of 34 patients (36 renal units) with ureteroceles that were treated endoscopically. Perioperative radiologic and clinical data regarding de novo VUR, UTI, and renal function were analyzed. Logistic regression analysis was used to identify factors that predicted unfavorable surgical outcomes, such as primary surgical failure, postoperative UTI, and deterioration of renal function. Results: Of the 36 renal units, 22 had a duplex system (61.1%). Preoperative VUR was noted in 9 units (25.0%), including 3 units without renal duplication. Endoscopic surgery successfully decompressed the ureterocele and hydronephrosis in 28 units (77.8%). De novo VUR developed in 18 renal units (50.0%) postoperatively. The absence or presence of de novo VUR was not related to unfavorable surgical outcomes in univariate or multivariate analyses. Even after selection for the 28 renal units without preoperative VUR, the occurrence of de novo VUR had no predictive value for unfavorable surgical outcomes. Moreover, among the 14 renal units without renal duplication, de novo VUR had no predictive value for any of these adverse outcomes. Conclusions: After endoscopic ureterocele puncture, de novo VUR is not significantly associated with postoperative UTI or deterioration in renal function in the long term. It may not, therefore, be necessary to reconstruct lower urinary tract routinely to correct de novo VUR after endoscopic puncture of the ureterocele.

Pubertal outcomes and sex of rearing of patients with ovotesticular disorder of sex development and mixed gonadal dysgenesis.

PURPOSE: Patients with ovotesticular disorder of sex development (DSD) and mixed gonadal dysgenesis (MGD) usually present with asymmetric gonads and have wide phenotypic variations in internal and external genitalia. The differential diagnosis of these conditions is based on karyotype and pathological findings of the gonads. This study investigated the clinical features at presentation, karyotype, sex of rearing, and pubertal outcomes of patients with ovotesticular DSD and MGD. METHODS: The study comprised 23 patients with DSD who presented with asymmetric gonads. The presenting features, karyotype, sex of rearing, and pubertal outcomes were reviewed retrospectively. RESULTS: All 23 patients presented with ambiguous genitalia at a median age of 1 month (range, 1 day-1.6 years). Müllerian duct remnants were identified in 15 of 23 patients (65.2%). Fourteen patients were diagnosed with ovotesticular DSD, whereas the other 9 were diagnosed with MGD. Eight of 14 patients (57.1%) with ovotesticular DSD were raised as males, while 7 of 9 patients with MGD (77.8%) were assigned as males. One male-assigned patient with ovotesticular DSD changed to female sex at age 20 years. CONCLUSION: Patients with ovotesticular DSD and MGD manifest overlapping clinical presentations and hormonal profiles. It is difficult to determine the sex of rearing and predict long-term pubertal outcomes. Therefore, long-term follow-up is required to monitor spontaneous puberty, sex outcome, and urological and gynecological complications.

Does epithelioid angiomyolipoma have poorer prognosis, compared with classic angiomyolipoma?

Purpose: Classic angiomyolipoma (AML) is common benign kidney tumor. However, some studies have claimed that epithelioid angiomyolipoma (EAML) has malignant potential. We compared the patient characteristics and prognosis of EAML and classic AML to demonstrate predicting factors and poorer prognosis of EAML. Materials and Methods: The medical records of 231 patients who were diagnosed with EAML (n=27, 11.7%) or classic AML (n=204, 88.3%), were reviewed. All patients underwent computed tomography (CT) scans before operation or needle biopsy. We assessed the age, sex, tumor size, body mass index, comorbidities, and Hounsfield unit (HU) according to each CT phase. We defined the unfavorable group as patients with recurrence, metastasis and death due to tumor progression. Logistic regression analysis was used to predict EAML. Results: EAML patients were younger (41.2 years vs. 49.1 years, p=0.001), predominantly male (55.6% vs. 28.4%, p=0.005), and had a larger tumor (7.5 cm vs. 4.2 cm, p<0.001). The median pre-contrast HU was not significantly different between EAML and classic AML (29.9±23.7 vs. 14.7±41.0, p=0.071). In multivariable analysis, younger age (odds ratio [OR], 0.96; p=0.032), male sex (OR, 3.33; p=0.013), and tumor larger than 4 cm (OR, 3.8; p=0.009) were significant predictive factors. Five patients (18.5%) had unfavorable outcomes, two patients had lymph node metastasis, and three patients had lung metastasis. Conclusions: Patient and tumor characteristics can be helpful in determining the type of AML preoperatively. Younger age, male sex, and larger tumor mass may increase the possibility of diagnosing EAML. EAML has malignant potential and requires careful follow-up.

Urethral defect due to periurethral abscess treated with a tunica vaginalis flap: A case report.

RATIONALE: Periurethral abscess is a life-threatening disease, and the occurrence of a urethral defect with periurethral abscess is a rare finding. In this case, the patient had a lengthy urethral defect from the bulbous urethra to the membranous urethra accompanied by periurethral abscess that developed within a short time. Herein, we report a case of a pedicle-sparing tunica vaginalis flap utilized in urethral reconstruction which degenerated due to fibrotic changes and soft tissue defects in the urethral bed. PATIENT CONCERNS: The patient was a 36-year-old man with fever and lower urinary tract symptoms who had been treated with antibiotics and anti-inflammatory drugs for urinary tract infections 3 days before admission. Purulent necrosis was formed by the urethral abscess, and a long-length urethral defect was formed in the bulbous urethra. DIAGNOSIS: Based on the initial computed tomography and laboratory findings, empirical antibiotics were administered to treat a lower urinary tract infection. On the 7th day of hospitalization, ultrasonography was performed due to the sudden swelling of the scrotum, and the patient was diagnosed with a periurethral abscess that was 10 × 3 cm in size. INTERVENTION: Initial urinary diversion, wide debridement, and a large amount of abscess drainage were performed. Necrosis of the urethral ventral part caused a urethral defect that was 5 cm in size. After treatment with antibiotics, long-term disinfection and intermittent debridement were conducted and urethral reconstruction was performed using a tunica vaginalis flap with preserved vascular structure. OUTCOMES: No complications occurred until 6 months after urethral reconstruction. LESSONS: Urethral reconstruction using a tunica vaginalis flap is a good method for selected patients. Pedicle-sparing tunica vaginalis is an advantageous material for resolving urethral defects, especially when the surrounding circulation conditions are poor.

Clinicopathological Characteristics of Urinary Bladder Tumors in Korean Patients 20 Years or Younger.

BACKGROUND: To investigate the clinicopathological characteristics of urinary bladder tumors, a rare malignancy, in patients 20 years or younger. METHODS: Using a retrospective chart review among patients who received bladder surgery at 2 institutions between July 1996 and January 2013, we analyzed the clinicopathological characteristics of urinary bladder tumors in 21 pediatric patients (male:female = 4.25:1.00; mean age, 12.1 years). RESULTS: Pathology revealed 9 urothelial tumors, 6 rhabdomyosarcomas, 1 low-grade leiomyosarcoma, 1 large cell neuroendocrine carcinoma, 1 inflammatory myofibroblastic tumor, and 3 cases of chronic inflammation without tumors (including 1 xanthogranulomatous inflammation). Urothelial tumors (mean patient age, 16.0 years) were benign or low-grade; and only transurethral resection of the bladder tumor was necessary for treatment. Patients with rhabdomyosarcomas (mean age, 5 years) underwent radiotherapy (if unresectable) or transurethral resection of the bladder tumor (if resectable), after chemotherapy. Of these patients, 2 underwent radical cystectomy, with the remaining patients not receiving a cystectomy. With the exception of one patient, all patients are currently alive and recurrence-free. CONCLUSION: Urothelial tumors were the most commonly found pediatric bladder tumor, with embryonal rhabdomyosarcoma being the second most common. Urothelial tumors are common in relatively older age. Since urothelial tumors in children typically have a good prognosis and rarely recur, transurethral resection of the bladder tumor is the treatment of choice. Rhabdomyosarcomas are common in younger patients. Since rhabdomyosarcoma is generally chemosensitive, chemotherapy and radiotherapy are the treatment of choice for bladder preservation in these patients.

Long-term Outcomes of Endoscopic Anti-reflux Surgery in Pediatric Patients with Vesicoureteral Reflux: Urinary Tract Infection, Renal Scarring, and Predictive Factors for Success.

BACKGROUND: To report the long-term outcomes of endoscopic surgery (ES) in pediatric patients with vesicoureteral reflux in terms of success rate, urinary tract infection, and renal function. METHODS: We retrospectively reviewed the records of 73 pediatric patients (110 ureters) who underwent ES for vesicoureteral reflux. Ultrasonography was performed 1, 3, and 12 months postoperatively. Voiding cystourethrography was performed 3 months postoperatively and repeated after 1 year if vesicoureteral reflux persisted. Success was defined as the absence of reflux at the first voiding cystourethrography. Renal scans were performed at least 12 months postoperatively. Renal function deterioration was defined as a new scar or a greater than 5% decrease in function. RESULTS: The median follow-up duration was 24 (12-118) months. The overall success was 65.6%, while it was 78.9%, 87.0%, 62.5%, 37.5%, 66.7% for grades I, II, III, IV, and V, respectively. In multivariate analyses, significant predictive factors for success were vesicoureteral reflux grade (odds ratio [OR], 0.28; P < 0.001) and mound detection at the first postoperative ultrasonography (OR, 13.53; P < 0.001). Renal function deterioration was found in 8 (15.3%) ureters and was less common in those with successful surgeries than in those with failures (9.5% vs. 40.0%; P = 0.035). No significant predictive factor for renal function deterioration or urinary tract infection was found. CONCLUSION: Successful short-term outcomes of ES are expected in low-grade vesicoureteral reflux, especially when a mound is detected by postoperative ultrasonography. However, unpredictable long-term renal deterioration warrants continued follow-up.

Long-term outcomes of kidney and bladder function in patients with a posterior urethral valve.

We investigated long-term functional changes in the kidney and bladder of patients with posterior urethral valve (PUV) who underwent fetal intervention or postnatal surgery.We retrospectively reviewed the medical records of 28 consecutive patients treated for PUV at our institution. Detailed data on medical and surgical histories, particularly on pre- and postnatal treatment modality, including fetal vesicoamniotic shunt, endoscopic valve ablation, and vesicostomy, were collected and analyzed. Long-term renal function was evaluated based on serum levels of creatinine (sCr), estimated glomerular filtration rate (eGFR), and renal scans. Voiding function was evaluated in urodynamic tests.Vesicoamniotic shunting was performed in 12 (42.8%) patients. Although the mean initial sCr was significantly higher in patients in whom a fetal shunt was placed than in others (2.04 vs 1.17 mg/L, P = .038), the sCr at long-term follow-up was not significantly different between them (0.64 vs 0.40 mg/L, P = .186). The mean maximum detrusor pressure was significantly lower in patients with a fetal shunt than in others (37.7 vs 73.0 cm H2O, P = .019). Postnatal vesicostomy was performed in 14 patients, and primary valve ablation was performed in 13 patients. The mean initial sCr was higher in patients in the vesicostomy group than in the primary valve ablation group (2.08 vs 0.86 mg/L, P = .014). However, no significant differences were found in sCr (0.9 vs 0.3 mg/L, P = .252) or GFR (59.1 vs 68.5 mL/min/1.73 m, P = .338) at long-term follow-up. Bladder capacity was greater and residual urine volume was less in the vesicostomy group than in the primary valve ablation group, but without statistical significance.Vesicostomy is more beneficial in the recovery of renal function and is not inferior in terms of bladder function, even in patients with severe PUV disorder. It is a reliable surgical option that can spare renal function and guarantee adequate bladder function in the long term.

Predictors of Renal Functional Improvement After Pyeloplasty in Ureteropelvic Junction Obstruction: Clinical Value of Visually Assessed Renal Tissue Tracer Transit in 99mTc-mercaptoacetyltriglycine Renography.

OBJECTIVE: To determine the clinical value of visually assessed renal tissue transit time (TTT) in 99mTc-mercaptoacetyltriglycine (99mTc-MAG3) renography for patients undergoing pyeloplasty. MATERIALS AND METHODS: Medical records of 164 patients who underwent dismembered pyeloplasty were retrospectively reviewed. Baseline and postoperative renal ultrasonography and 99mTc-MAG3 renography were performed. Two urologists blinded to clinical data evaluated the renography and classified TTT as timely or delayed based on visualization of the tracer in the kidney pelvis between 2 and 10 minutes. Renal functional change after pyeloplasty was compared between patients in the timely and delayed groups. RESULTS: A total of 126 patients (median age, 9 months) were evaluated after excluding patients with bilateral ureteropelvic junction obstruction, a single functioning kidney, duplicated ureter, or <3 months of follow-up. There were no differences between 89 patients with timely TTT and 37 patients with delayed TTT in mean preoperative hydronephrosis grade (3.7 vs 3.8) and pelvic diameter (3.1 cm vs 3.4 cm). Although the pre- and postoperative mean values of differential renal function (DRF) were significantly higher in the timely group than in the delayed group (47.2% vs 38.3% and 47.9% vs 44.6%), DRF change was greater in the delayed group (6.3% vs 0.6%). In multivariate analysis, delayed TTT was the only significant predictor of >5% improvement in renal function after pyeloplasty. CONCLUSION: Delayed TTT in 99mTc-MAG3 renography was a significant predictor of renal functional improvement after pyeloplasty in ureteropelvic junction obstruction. Because substantial improvement of renal function is anticipated, we recommend immediate pyeloplasty in patients with delayed TTT and decreased DRF.