INTRODUCTION: Urethral duplication in females is a rare entity with variable presentation and associated abnormalities. Several classification systems have been proposed; however, there exists a need to further delineate...INTRODUCTION: Urethral duplication in females is a rare entity with variable presentation and associated abnormalities. Several classification systems have been proposed; however, there exists a need to further delineate female urethral duplication. OBJECTIVE: We sought to propose a new simplified classification system for isolated female urethral duplication based on bladder neck anatomy and meatal configuration, focusing on clinical and surgical outcomes. STUDY DESIGN: A literature review of female urethral duplication up to August 2025 in the PubMed and OVID Databases was performed. We identified 39 articles with 44 patients; clinical characteristics and surgical outcomes were collected. One patient from our institution was included. RESULTS: Anatomic classification was based on bladder neck anatomy: separate bladder neck (A), common bladder neck (B) or lambda bladder neck (λ; single bladder neck, duplication extends from the common channel). The distal anatomy was defined by location of the urethral openings - anterior-orthotopic (1), anterior-posterior (2), orthotopic-posterior (3) and orthotopic-orthotopic (4). Incomplete duplicated urethra was identified with the subscript (i). All A1 and A2 reported urinary incontinence and the A bladder neck configuration subset of patients reported UTI in 36% versus 17% with B and 17% with λ. Despite similar bladder neck configuration, A2 (38%) configuration had more genital physical exam abnormalities and anomalies compared to A1 (0%). Surgical intervention was variable across groups - the preponderance of secondary and tertiary operations for type 2 distal anatomy may suggest increased surgical complexity to achieve "normal" voiding outcomes - 4/8 patients with A2 underwent a second surgery and third surgery, 1/4 with B2 and 4/7 with λ2. DISCUSSION: Our classification system, based on the bladder neck and distal anatomy, is defined after typical work-up. Despite small populations, there are meaningful differences driven both by the origin of the urethra and meatal location. The system reflects this by separating groups by plane, bladder neck configuration, distal meatal location, and includes the λ configuration. CONCLUSION: Urethral duplication in females is a rare entity, and management decisions must be individualized. To the best of our knowledge, we have compiled the largest collection of reported female urethral duplication with descriptions of clinical, surgical and associated abnormalities. Through this data, we propose a classification system based on bladder neck and distal anatomy, focused on meaningful clinical differences between groups that may help guide counseling and management decisions.
Lopez P, Pierucci UM, Blanc T
… +18 more, Bousquet M, Lauriot Dit Prevost A, Bidault-Jourdainne V, Clermidi P, Faure A, Branchereau S, Defert C, Suply E, Faraj S, Leclair MD, Chalhoub M, Chaussy Y, Louis D, Poli-Merol ML, Paye A, Lachkar AA, El-Ghoneimi A, Peycelon M
BACKGROUND: Prune Belly Syndrome (PBS) is a rare congenital disorder characterized by bilateral intraabdominal undescended testes, abdominal wall flaccidity, and urinary tract dilation. Surgical management of cryptorchid...BACKGROUND: Prune Belly Syndrome (PBS) is a rare congenital disorder characterized by bilateral intraabdominal undescended testes, abdominal wall flaccidity, and urinary tract dilation. Surgical management of cryptorchidism in PBS is technically challenging due to anatomical and urological anomalies. This study aimed to evaluate outcomes of different orchidopexy techniques, laparoscopic vs. open and one-stage vs. two-stage, in a national multicenter PBS cohort over 30 years. METHODS: We retrospectively reviewed 56 PBS patients treated between 1990 and 2023 in 22 French centers using data from the MARVU National Registry. Testicular position and size at last follow-up were compared across surgical techniques and staging approaches. RESULTS: A total of 87 orchidopexies were performed at a median age of 22 months; 62 (71%) were one-stage and 25 (29%) two-stage. Laparoscopy was used in 25 procedures (29%), more frequently in recent years. At a median follow-up of 7.5 years, 66 testes (76%) were located intrascrotally and 68 (79%) were of normal size. Testicular atrophy was observed in 15 cases (17%) and ectopic positioning in 18 (21%). No significant differences were found in anatomical outcomes based on surgical approach (intrascrotal position: 88% laparoscopic vs. 71% open, p = 0.11) or staging (atrophy: 19% one-stage vs. 12% two-stage, p = 0.54). Limitations include retrospective design and lack of hormonal or fertility data. CONCLUSIONS: Both one- and two-stage orchidopexies, whether laparoscopic or open, provide comparable anatomical outcomes in PBS-related cryptorchidism. Technique selection should be individualized. Prospective studies are needed to evaluate long-term endocrine and reproductive function.
BACKGROUND AND OBJECTIVE: Antibiotic resistance is a challenge in contemporary world. Urethroplasty for hypospadias is one of the most common urological surgeries performed around the world, yet, there is still no consen...BACKGROUND AND OBJECTIVE: Antibiotic resistance is a challenge in contemporary world. Urethroplasty for hypospadias is one of the most common urological surgeries performed around the world, yet, there is still no consensus on the use of antibiotics pre- and post-operatively. Our objective was to analyze the effectiveness of antibiotic prophylaxis and therapy before, during, and after hypospadias repair in children. METHODS: A prospective randomized trial was carried out including patients with coronal hypospadias who underwent urethroplasty performed by one surgeon. Urethral catheters were used in all cases for 10 days. Study participants were randomly assigned in a 1:1:1 ratio to receive a single intraoperative administration of antibiotics (Group I); an intraoperative antibiotic prophylaxis and antibiotic therapy for 10 days until the removal of urethral catheter (Group II); no antibiotic administration (Group III). Randomization was performed using computer-generated permuted blocks with randomly varying block sizes, prepared by an independent statistician. The results were analyzed using the analysis of variance (ANOVA). The following criteria were compared: postoperative functional complications, such as: urethral fistula, stenosis, diverticulum; wound infection symptoms: hyperemia of surgical site, pain during palpation, and symptomatic urinary tract infection. RESULTS: A total of 300 patients were included in the study. Two patients (2%) in Group I, two patients (2%) in Group II, and four patients (4%) in Group III had urethral fistulas, requiring surgery 6 months after primary repair, yet without statistical difference between groups. We found no significant difference in frequency of symptomatic UTIs between three groups (p = 0.182). CONCLUSION: There is no effect of antibiotic prophylaxis and therapy on the frequency of postoperative surgical and infectious complications after urethroplasty for coronal hypospadias repair in children.
OBJECTIVE: To investigate the utility of the External Genitalia Score (EGS) in the diagnosis of disorders of sex development (DSD) and decision-making regarding gender assignment in affected patients. METHODS: A retrospe...OBJECTIVE: To investigate the utility of the External Genitalia Score (EGS) in the diagnosis of disorders of sex development (DSD) and decision-making regarding gender assignment in affected patients. METHODS: A retrospective cohort study was conducted, enrolling 114 DSD patients aged <2 years (88 reared as males, 26 reared as females) treated at our hospital between April 2005 and June 2023, alongside 40 hypospadias patients aged <2 years who underwent surgery at our institution from January to July 2023. Demographic data (age) and EGS assessments of external genitalia were collected for all participants. Statistical analyses included independent samples t-tests, Mann-Whitney U tests and Receiver Operating Characteristic (ROC) curve analysis. Specifically, EGS scores were compared between the hypospadias group and the male-reared subgroup of the DSD cohort; additionally, EGS scores were contrasted between male-reared and female-reared DSD subgroups. RESULTS: The mean age was 20.3 months in the hypospadias group, 17.9 months in the male-reared DSD group, and 18.8 months in the female-reared DSD group. EGS ranged from 5.5 to 11.5 (median 10.5) in the hypospadias group and from 1 to 12 (median 4.75) in the DSD group. ROC curve analysis was performed to compare EGS scores between the hypospadias group and the male-reared DSD subgroup. The optimal diagnostic threshold was determined by maximizing the Youden index (sensitivity + specificity - 1), which balances sensitivity and specificity. A cut-off value of ≤8.50 was identified as indicative of DSD; clinically, patients with an EGS score <9 should be prioritized for DSD screening. Further comparison between male-reared and female-reared DSD subgroups yielded a threshold of 4.00. Clinically, an EGS score ≤4 may suggest a preference for female gender assignment. DISCUSSION: The EGS scale is a reliable, valid, and clinically feasible tool for characterizing external genitalia in DSD patients. An EGS score of 9 can serve as an indicator for initiating detailed sex development evaluation in hypospadias patients. While gender assignment in DSD is a complex, multifactorial process, EGS scores showed a significant association with the sex of rearing in our cohort. In settings where major determinants are balanced, EGS may serve as an adjunctive descriptive parameter rather than a standalone decision-making tool.
BACKGROUND: The establishment of a multidisciplinary spina bifida (SB) clinic in 2006 resulted in a review of the literature and an audit of renal outcomes based on then management practices. The audit showed 17% new ons...BACKGROUND: The establishment of a multidisciplinary spina bifida (SB) clinic in 2006 resulted in a review of the literature and an audit of renal outcomes based on then management practices. The audit showed 17% new onset renal scarring over a mean 5.8-year follow-up period. This prompted the development of a local protocol based on risk stratification combining serial ultrasound and non-invasive bladder function assessments, with invasive urodynamic studies reserved for high-risk patients. OBJECTIVE: This study sought to assess the impact of a risk stratified protocol on renal scarring and continence outcomes in children with SB. METHODS: A single centre, retrospective case review of SB patients treated after the introduction of the protocol was conducted. Electronic medical records were used to access patient demographics, continence status and the results of investigations and adherence to the local management protocol. Management that deviated from the protocol was deemed non-adherence. Renal scarring was determined by the presence of scarring on DMSA renogram. Continence was defined as having no urinary incontinence or no more than a single episode of incontinence in a month in patients above the age of 5. For statistical analysis, descriptive statistics in percentages were used, for comparisons of dichotomous variables the Students t-test was performed and to calculate statistical significance a Fisher exact test was done. RESULTS: 167 SB patients were identified with a mean follow up of 56 months. 141 patients were considered adherent to the protocol, 26 were non-adherent. In the protocol adherent group 5 patients (3.5%) developed renal scarring compared with 6 patients (23%) managed out of protocol (p = 0.002). Overall, 49/108 patients were continent either self-voiding 8/108 (7%), with urethral CIC 19/108 (20%) and 22/108 (45%) of them required bladder augmentation. Urinary continence improved with age with 26% continence at age 10, 64% continence by age 15 and 90% continent above 15 years of age. CONCLUSION: A management approach based on risk stratification resulted in incidence of renal scarring that is better than historical controls and comparable to published outcomes. Social continence was achieved in 90% of SB patients by 15 years of age. Hostile bladder changes can be readily identified using non-invasive assessment methods. An expectant treatment approach based on risk stratification is associated with good long term renal outcome and utilises invasive urodynamic resources for SB patients at high risk of renal injury or to address urinary continence in the older child.
INTRODUCTION: The intestinal microbiome holds promise as a tool for prognostics, diagnostics, and interventions in patients with congenital anomalies of the kidney and urinary system. The goal was to characterize the neo...INTRODUCTION: The intestinal microbiome holds promise as a tool for prognostics, diagnostics, and interventions in patients with congenital anomalies of the kidney and urinary system. The goal was to characterize the neonatal rat intestinal microbiome in response to partial upper and lower urinary obstruction using 16S rRNA Illumina sequencing. METHODS: Neonatal Sprague Dawley rats were operated on day three of life. Partial ureteral obstruction was performed in upper urinary tract obstruction (UUTO) via suture ligation. Partial urethral obstruction was created in lower urinary tract obstruction (LUTO) via clip-applier technique. The intestinal microbiome was evaluated via 16S rRNA gene amplicon sequencing. Alpha and beta diversity, single taxa abundance, and unique indicator species analysis was performed using R Statistical Software 4.2.0. RESULTS: At 21 days post-operatively, body mass was higher in LUTO compared to UUTO (p = 0.003). Beta diversity clustered significantly between groups. LUTO had elevated Firmicutes:Bacteroidota ratios compared to UUTO (p = 0.014). A unique LUTO indicator was the absence of CAG-302 (p = 0.014). Lactobacillus_B was significantly downregulated in UUTO (p = 0.033). All unique indicators of UUTO derived from the Lachnospiraceae family. CONCLUSION: This study evaluated the neonatal response of the gastrointestinal microbiome to urinary obstruction. LUTO resulted in high-risk microbiome profiles consistent with obesity and metabolic syndrome. UUTO rats had microbiome profiles consistent with CKD models, which modulate short chain fatty acids and uremic toxins. Such patterns in the intestinal microbiome may be used as a prognostic model to detect children at high-risk for organ damage secondary to obstructive uropathy.
BACKGROUND: Antegrade continence enema (ACE) and catheterizable bladder channel (Mitrofanoff) procedures are routinely performed in pediatric urology patients diagnosed with a neurogenic bladder and bowel. Stomal stenosi...BACKGROUND: Antegrade continence enema (ACE) and catheterizable bladder channel (Mitrofanoff) procedures are routinely performed in pediatric urology patients diagnosed with a neurogenic bladder and bowel. Stomal stenosis is a common surgical complication of these procedures, occurring in approximately 10-30% of stomas. Our frustration with this complication prompted us to modify our suturing technique during stomal construction to attempt to decrease the incidence of stomal stenosis. METHODS: We compared the rates of stomal stenosis between patients with neurogenic bladder who underwent the creation of an ACE or Mitrofanoff channel using the historical techniques (prior to April 2018) versus the current technique (from April 2018 to December 2020). Our current technique for stoma creation consists of suturing full thickness bowel to only the dermal layer of the skin using interrupted 5-0 polydioxanone interrupted sutures with the knots buried. Statistics were performed using Fisher's exact t-test, with p-values <0.05 considered significant. RESULTS: There were no significant differences in demographics between patients in the 2 cohorts. Stomal stenosis occurred in 25 of 98 stomas (25.5%) after undergoing either an ACE or Mitrofanoff procedure using the historical techniques, with a median patient follow-up of 122.6 months for ACE cohort and 165.8 for Mitrofanoff cohort. The incidence of stomal stenosis was significantly decreased in the current technique, with one of the 31 stomas (3.2%) experiencing stenosis (p = 0.022), with a median follow-up of 78.4 months for ACE cohort, and 66.5 months for Mitrofanoff cohort. These follow-up durations exceed the upper limits of time-to-stenosis in the historical stomas. Stomas in the current cohort have a minimum follow-up of 4.5 years and a maximum follow-up of 7 years. CONCLUSIONS: Our current suturing technique has significantly reduced the incidence of stomal stenosis in our patients. The technique is straightforward and flexible and can be applied to any stoma placed in any position. Only one of the patients with stomas created with the current suturing technique have developed stomal stenosis, with follow-up exceeding the median time to development of stenosis of our historical cohort.
Mohammadi Ganjaroudi N, Torabinavid P, Hojjat A
… +7 more, Neishabouri A, Moaveni AK, Mahjoub FE, Shiravi Khoozani M, Lotfi B, Hekmati P, Kajbafzadeh AM
PURPOSE: To evaluate whether underlying histological alterations in hydrocele sac wall can provide an explanation of difference in abdominoscrotal hydrocele (ASH) and communicating hydrocele (CH) behavior. The aim of thi...PURPOSE: To evaluate whether underlying histological alterations in hydrocele sac wall can provide an explanation of difference in abdominoscrotal hydrocele (ASH) and communicating hydrocele (CH) behavior. The aim of this study is to assess the immunohistopathologic characteristics of CH and ASH sac walls in pediatric patients. METHODS: In a retrospective case-control study, patients diagnosed with ASH or CH underwent surgical management at a tertiary pediatric center. Samples of ASH or CH wall sac were obtained for histopathologic evaluations, especially for contractile units and fibromuscular components. These assessments comprised Hematoxylin and eosin (H&E) and immunohistochemical (IHC) staining for smooth muscle actin (SMA), Myogenin and Caldesmon (H-CALD) to identify and quantify the presence of cells with striated or smooth muscle origins. All patient's imaging, clinical and demographic data were evaluated. All statistical analysis performed using SPSS software and p-value of <0.05 considered as the level of significance. RESULTS: Among 38 patients in our case-control study, 14 (36.84%) patients referred with bilateral hydrocele. The intra-operative observation of all ASH cases indicated no patent processus vaginalis. The IHC evaluations of ASH and CH specimens demonstrated that there is a significantly higher expression of SMA (median 5.73% versus 0.56%, P-Value <0.001), H-CALD (median 9.28% versus 0.18%, P-Value <0.001) in comparison to those of CH. CONCLUSION: The higher expression of smooth muscle and myofibroblast origins in the ASH wall suggests the potential of consistent contractility, which likely affects the secretion-reabsorption imbalance. Performing histologic studies can enhance our understanding of ASH pathogenesis and clinical characteristics.
BACKGROUND: Toilet training and continence are major developmental milestones, but children with neurogenetic conditions often experience extreme difficulties, sometimes never achieving full continence. These challenges...BACKGROUND: Toilet training and continence are major developmental milestones, but children with neurogenetic conditions often experience extreme difficulties, sometimes never achieving full continence. These challenges impose ongoing burden on caregivers, especially as children age and require toileting assistance outside the home. SATB2-associated syndrome (SAS) is a rare genetic disorder in which such functional issues have not been systematically studied. We sought to assess bladder and bowel function in individuals with SAS and compare symptoms burden to normative data. METHODS: Caregivers completed a survey that included the Vancouver Symptom Score (VSS) for Dysfunctional Elimination Syndrome. VSS scores were analyzed and compared to published norms. Additional data included toilet training milestones, continence status, urinary tract infections, and bowel treatment history. RESULTS: Of the 32 individuals with SAS represented in the caregiver-reported survey, over half (53%) were not toilet trained, and only 22% were fully trained for both day and night (mean age at toilet training 6.9 years). The SAS group (n = 32) had a median total VSS of 16.0 (range: 6-30), indicating clinically significant dysfunction, while control data (n = 49) reported a median of 5.0 (range: 0-12, p < 0.001), with the greatest differences observed in daytime wetting, nighttime wetting, wetting extent, and fecal soiling. CONCLUSIONS: Individuals with SAS experience markedly elevated elimination symptom burden. Findings support the need for early screening and intervention to promote continence and reduce caregiver strain. A clinical pathway is proposed for screening, evaluation, and management of elimination dysfunction in SAS.
OBJECTIVE: This study aimed to investigate whether the maximal cystometric capacity (MCC) in children with spina bifida (SB) is indeed lower, as predicted by the Palmer formula, and to evaluate the impact of different bl...OBJECTIVE: This study aimed to investigate whether the maximal cystometric capacity (MCC) in children with spina bifida (SB) is indeed lower, as predicted by the Palmer formula, and to evaluate the impact of different bladder filling rates on urodynamic parameters. MATERIALS AND METHODS: This prospective, randomized, two-sequence crossover-controlled study included 70 children aged 3-18 years with spina bifida under regular follow-up. In Group 1, the first two bladder fillings were performed at the ICCS-recommended rate, and the third at 75% of that rate (Palmer formula). In Group 2, the sequence was reversed. Urodynamic parameters, including maximal cystometric capacity (MCC), bladder compliance, detrusor activity, filling pressures, and detrusor leak point pressure (DLPP), were analyzed across fillings. RESULTS: Cystometric bladder capacity was lower during fillings performed at the Palmer-adjusted rate compared with those at the ICCS-recommended rate. The proportion of reduced compliance significantly decreased in Group 1 (p = 0.046) but remained unchanged in Group 2. A significant positive correlation was observed between expected bladder capacity (EBC) and measured MCC in both groups (ρ ≈ 0.5-0.6). The highest correlation and agreement were found in Group 1 during the third filling at the Palmer rate (ICC = 0.606). No significant intra- or intergroup differences were observed in detrusor pressure, end-filling pressure, DLPP, or overactive bladder prevalence. CONCLUSION: Bladder filling rate was associated with differences in both cystometric capacity and bladder compliance in children with spina bifida. Fillings performed according to the Palmer formula (approximately 75% of the ICCS-recommended rate) were associated with capacities that more closely approximated age-expected values and with modest differences in bladder compliance. Conversely, faster filling rates did not produce similar benefits. These findings suggest that slower filling strategies may improve measurement consistency and agreement with expected bladder capacity estimates. However, the magnitude and direct clinical impact of these differences should be interpreted cautiously, particularly in light of the potential influence of sequence-related effects.
Conventional circumcision techniques may result in bleeding, limited control of the mucosal collar, and the need for dressings. We describe a modified bipolar technique enabling near-bloodless dissection with precise muc...Conventional circumcision techniques may result in bleeding, limited control of the mucosal collar, and the need for dressings. We describe a modified bipolar technique enabling near-bloodless dissection with precise mucosal trimming. Following bipolar division of the preputial skin, the inner mucosa is separated using two forceps and retracted proximally, creating a protective plane for safe skin adjustment. The mucosal collar is trimmed with scissors. The wound is closed with sutures and 2-octyl cyanoacrylate, avoiding dressings. This technique has been performed in 55 consecutive cases (median age: 2 years and 2 months; range: 0 days-17 years and 5 months) with no reported complications over a median follow-up of 2 years and 7 months (range: 3 months-4 years and 1 month).