BACKGROUND: Hypospadias affects 1 in 200-300 male births. Parents are often concerned about penile adequacy beyond the urethral defect itself, yet few studies have systematically compared stretched penile length (SPL) in...BACKGROUND: Hypospadias affects 1 in 200-300 male births. Parents are often concerned about penile adequacy beyond the urethral defect itself, yet few studies have systematically compared stretched penile length (SPL) in hypospadias against population-based reference standards. OBJECTIVE: To evaluate SPL distribution patterns in boys with Types I and II hypospadias and compare them with established normative data. METHODS: The authors studied 876 consecutive boys aged 1-14 years with unoperated Types I (distal) and II (mid-shaft) hypospadias. Two observers independently measured SPL using the validated SPLINT technique. The SPL measurements were compared against age-matched normative data from 1276 Indian children. Exact binomial probability tests were used for percentile distributions, chi-square tests for subtype comparisons and t-tests for mean deviations. RESULTS: The cohort included 479 Type I and 397 Type II cases. SPL distribution showed a marked leftward shift: 71% fell below the 50th percentile (expected 50%, p < 0.001) and 41.5% below the 25th percentile. Lower percentiles were overrepresented, 20.7% were below the 10th percentile and 20.8% in the 10th-25th range. Upper percentiles were depleted: only 7.4% in the 75th-90th range and 1.7% above the 90th percentile (all p < 0.001). Mean SPL was reduced by 6.8% (95% CI: -8.18 to -5.42%) in Type I and 7.5% (95% CI: -9.05 to -5.92%) in Type II. The two subtypes showed no significant distributional difference (χ = 6.22, p = 0.18), suggesting that meatal position does not predict SPL reduction. CONCLUSIONS: Boys with distal and mid-shaft hypospadias show clinically meaningful SPL reduction that follows a continuous distribution rather than an all-or-none pattern. SPL reduction appears independent of meatal position. These findings support routine SPL assessment using population-specific references and can guide preoperative counselling.
INTRODUCTION: Circumcision is a common surgical intervention, and pain is the most common complaint. Poorly managed pain can increase morbidity and reduce patient and parent satisfaction. Ketorolac, a non-steroidal anti-...INTRODUCTION: Circumcision is a common surgical intervention, and pain is the most common complaint. Poorly managed pain can increase morbidity and reduce patient and parent satisfaction. Ketorolac, a non-steroidal anti-inflammatory drug (NSAID), provides analgesia and may be effective in reducing pain after circumcision in pediatric patients. STUDY OBJECTIVES: The primary objective was to determine the effect of pre-operative intravenous ketorolac versus normal saline placebo on parental perception of postoperative pain at 24 h. We also explored the effect of ketorolac on postoperative pain scores, incidence of bleeding, incidence of vomiting, and analgesic use. MATERIALS AND METHODS: A prospective, randomized, single-blinded trial was conducted at a single tertiary children's hospital. Patients aged 1-17 presenting for circumcision were included and randomized to either normal saline injectate or ketorolac (0.5 mg/kg, maximum 30 mg). Postoperative delirium and pain scores were recorded in the post-anesthetic care unit. Parents completed the parents' postoperative pain measure (PPPM) at 24 h. The CONSORT criteria and checklist were used to guide reporting of this randomized controlled trial. RESULTS: A total of 100 participants were included. 50 participants were in each group. Mean (SD) PPPM score 24 h after normal saline and ketorolac was 6.1 (3.4) and 5.5 (3.3), respectively for a mean difference of -0.55 points [95% CI: -1.9 to 0.83; p = 0.427]. The mean (95% CI) fixed effect size of ketorolac on post anesthetic care unit reported Face, Leg, Activity, Cry, Consolability score was -0.17 (-0.77 to 0.43, p = 0.585) points, while the mean (95% CI) effect size of ketorolac on numerical rating scale was -1.2 (-2.2 to -0.18, p = 0.026) points. DISCUSSION: Pre-operative intravenous ketorolac, compared to normal saline placebo, did not result in a difference in parental perception of postoperative pain at 24 h after circumcision surgery. As part of exploratory analyses, those receiving ketorolac had modestly lower mean immediate postoperative pain scores, likely not of clinical importance. Ketorolac has often been avoided due to concerns of increased bleeding risk, however it is important to consider that there is no substantial evidence to support this. This is the first study to our knowledge evaluating the effect of ketorolac in pediatric circumcision patients. While the PPPM score is a family centered outcome, it is an indirect measurement of pediatric pain. CONCLUSIONS: Pre-operative intravenous ketorolac was not associated with lower parental reported pain scores at 24 h compared to normal saline placebo after pediatric circumcision surgery. CLINICAL TRIAL REGISTRATION: NCT02973958.
BACKGROUND: Emphysematous pyelonephritis (EPN) is a rare, life-threatening gas-producing renal infection. Literature on pediatric EPN remains scarce. OBJECTIVE: To describe a case of pediatric EPN and systematically revi...BACKGROUND: Emphysematous pyelonephritis (EPN) is a rare, life-threatening gas-producing renal infection. Literature on pediatric EPN remains scarce. OBJECTIVE: To describe a case of pediatric EPN and systematically review the data on clinical presentations, diagnostic modalities, management strategies, and prognosis in pediatric emphysematous pyelonephritis. METHODS: A comprehensive search in PubMed, Embase and Web of Science using defined search terms was done, along with the Joanna Briggs Institute (JBI) critical appraisal checklist for quality assessment. RESULTS: Of 100 records, 20 studies (n = 21) were included in the review. The median (IQR) age of presentation was 48 (8-138) months. The major risk factor was reflux/obstructive nephropathy (52.3 %), and E. coli was the common organism. Management included antibiotics (47.6 %), minimally invasive procedures (33.3 %), or nephrectomy (19 %). We additionally report an adolescent girl with Type 1 diabetes mellitus and class 3A right-sided EPN. She improved with antibiotics and percutaneous nephrostomy (PCN) insertion. CONCLUSION: Obstructive or reflux nephropathy is a major risk factor for pediatric EPN. Antibiotics are the mainstay for treatment with supportive management. Refractory cases may require percutaneous drainage or partial/total nephrectomy.
INTRODUCTION: We previously published our modified testicular traction technique, including an intracorporeal suture, gubernacular preservation, and minimal dissection. We used to fix the testis to a mobile part of the a...INTRODUCTION: We previously published our modified testicular traction technique, including an intracorporeal suture, gubernacular preservation, and minimal dissection. We used to fix the testis to a mobile part of the abdominal wall to enhance lengthening with respiratory movement rather than fixing near a bony landmark. This study compares our approximately 7-day traction period to the traditional 6-week period adopted earlier. PATIENTS AND METHODS: Patients with intra-abdominal testes who underwent intracorporeal placement of a traction suture, as a modification of staged traction laparoscopic-orchiopexy, were prospectively divided into two groups across five centers in three Arabian Gulf countries between February 2018 and October 2024. In Group 1, the interval between the two stages was approximately 7 days, whereas in Group 2, the waiting interval was 6 weeks. RESULTS: Group 1 included 92 testes, while group 2 included 58 testes. All patients tolerated both stages of surgery well without complications. During the second stage, four testes were identified as detached from the anterior abdominal wall in Group 2. Two individuals required a repeat of the initial stage, while the other two were sufficiently long to be placed in the scrotum without tension. The mean operative time was marginally prolonged in n Group 2, attributed to the time required for testicular adhesiolysis. At 1-year follow-up, a successful outcome was observed in all cases. CONCLUSION: Short interval modified staged traction orchiopexy demonstrated comparable efficacy to the original staged traction orchiopexy. Additionally, the operative time for the second stage of traction was reduced, with a shorter interval, attributed to simpler dissection. It may also be associated with a lower likelihood of repeating the first stage.
BACKGROUND: Penile anthropometry is essential for diagnosing genital anomalies. However, as these biometric values vary ethnically, population-specific standards are necessary. AIM: To establish the first penile anthropo...BACKGROUND: Penile anthropometry is essential for diagnosing genital anomalies. However, as these biometric values vary ethnically, population-specific standards are necessary. AIM: To establish the first penile anthropometric nomograms for boys in Qatar. STUDY DESIGN: This prospective cross-sectional study included 210 healthy boys in Qatar (aged 6 months to 12 years) undergoing elective circumcision at Hamad Medical Corporation (October 2023-October 2024). We recorded demographic data and measured stretched penile length (SPL), glans diameter at the coronal sulcus (GDCL), and mid-penile diameter (MPD). Age-specific nomograms were generated, and associations between SPL, age, and BMI were analyzed using SPSS v27. RESULTS: Mean SPL, GDCL, and MPD for infants (6-12 months) were 3.75 ± 0.4 cm, 1.36 ± 0.1 cm, and 1.28 ± 0.1 cm, respectively. For boys (1-12 years), values were 5.53 ± 0.9 cm, 1.71 ± 0.2 cm, and 1.61 ± 0.21 cm. SPL showed a biphasic growth pattern: a gradual increase from 3.75 ± 0.4 cm (6 months-5 years) to 4.90 ± 0.9 cm (6-10 years), followed by rapid growth to 7.72 ± 1.3 cm at 11-12 years. SPL correlated with height age but not with body mass index. DISCUSSION: This study provides the first population-specific nomograms for penile anthropometry of healthy boys in Qatar. The observed biphasic growth pattern is consistent with global trends but shows distinct ethnic variations. Limitations include the lack of data for infants under six months. Compared internationally, our results align with Middle Eastern cohorts but differ from Asian and South American populations, underscoring the need for local reference standards. These findings have direct clinical applicability, providing essential reference data to prevent misdiagnosis of conditions like micropenis. CONCLUSIONS: We successfully established an age-specific nomogram for penile biometrics of boys in Qatar. These normative data provide region-specific reference values to support the clinical assessment of disorders of sexual development and to optimize surgical decision-making in hypospadias repair.
BACKGROUND: Legislation restricting surgical interventions in children with differences of sex development (DSD) has intensified debate about female genital restoration surgery (FGRS) in patients with congenital adrenal...BACKGROUND: Legislation restricting surgical interventions in children with differences of sex development (DSD) has intensified debate about female genital restoration surgery (FGRS) in patients with congenital adrenal hyperplasia (CAH). Long-term patient-reported outcomes are needed regarding the optimal timing of surgery. OBJECTIVE: We aimed to assess health-related quality of life (HRQoL), gender identity, and family satisfaction regarding surgical timing and outcome in females with CAH following genital restoration surgery. STUDY DESIGN: Cross-sectional survey of CAH patients who underwent surgery between January 2007 to December 2016 at our institution. Validated instruments (KINDL questionnaire for HRQoL, Utrecht Gender Dysphoria Scale, UGDS) and structured telephone interviews were employed. RESULTS: Data on HRQoL was available for 25 patients (self- and/or parent-reported) out of 56 eligible patients (45% response rate). Median age at first surgery was 6 months (range: 3-137 months). Patients' age at time of participation ranged from 2 to 28 years. All had 21-hydroxylase deficiency (92% salt-wasting form) with Prader grades ranging from II-V. Wound dehiscence requiring secondary suturing occurred in 15% of patients with primary surgery at our center, while only one (4%) patient developed vaginal stenosis after early primary vaginoplasty. Two additional patients (8%) with prior outside surgery required vaginal revision after FGRS at age 12. HRQoL scores were comparable to healthy reference populations across most age groups. Children aged 3-6 and adolescents and young adults showed no significant difference from reference values, while children aged 7-13 showed a slight elevation. None of five patients ≥14 years demonstrated gender dysphoria (all UGDS scores <40, threshold ≥40). 14 patients and families were also interviewed by telephone. All preferred early surgical timing. No family expressed regret about the decision or preferred delayed surgery. DISCUSSION: This study provides validated intermediate-term patient- and parent-reported outcomes after FGRS in CAH. Key limitations include the small sample size, single-center design, and young age of most patients. Selection bias may exist, though participating families included those who underwent revision surgery. The absence of a non-operated comparison group reflects current clinical reality, as nearly all CAH patients with urogenital sinus underwent surgical correction. CONCLUSIONS: Females with CAH reported normal HRQoL and a comfortable female gender identity after early FGRS. The patients and their families expressed a preference for early surgery. However, there is need for longer-term follow-up to assess sexual function and reproductive outcomes as well comparison of outcomes with a non-operated group.
BACKGROUND: We report Bladder Turn-in & Epispadias Repair (TIER) as a first step of two-staged Bladder Exstrophy repair. TECHNIQUE: Circumferential incision was made around the bladder and urethral plate. Penis was deglo...BACKGROUND: We report Bladder Turn-in & Epispadias Repair (TIER) as a first step of two-staged Bladder Exstrophy repair. TECHNIQUE: Circumferential incision was made around the bladder and urethral plate. Penis was degloved and corporal bodies were mobilised. The epispadiac urethra and spongiosa were dissected off the corporal bodies leaving the frenulum and prepuce attached to hemi-glans. Perineal muscle was identified by muscle stimulator and wrapped around the bladder neck. Bladder was closed leaving reimplantation/bladder neck repair to second stage. The neourethra was tubularised and meatus fashioned within the glans. No osteotomy/radical mobilisation was done; rectus muscles were mobilised from periosteal attachment for midline closure. Ventral skin flaps were used for penile skin cover. RESULTS: Between 2020 and 2024, eleven male infants (median age 3-months) were operated. None had glans loss, bladder neck fistula or renal damage. 1/11 (9%) had superficial dehiscence. The mean bladder capacity was 150 ml; 5/11 (45.45%) completed second stage: reimplantation & bladder neck repair; 4/5 had dry period >2 h. None had recurrent urine infections or upper tract deterioration (median follow-up: 3 years). CONCLUSION: This pilot study reports two-stage TIER where bladder closure/with perineal muscle approximation and epispadias repair are performed at first stage enabling bladder growth from modest outlet resistance allowing staged ureteric reimplantation in a safe setting. IRB approval: IEC-NI/24/Feb/92/41.
BACKGROUND AND OBJECTIVE: Vesicoureteral reflux (VUR) is a common condition in pediatric urology, yet important uncertainties persist regarding risk stratification, imaging strategies, and prevention of long-term renal d...BACKGROUND AND OBJECTIVE: Vesicoureteral reflux (VUR) is a common condition in pediatric urology, yet important uncertainties persist regarding risk stratification, imaging strategies, and prevention of long-term renal damage. Emerging technologies may help address these challenges. This review provides a forward-looking overview of recent advances in artificial intelligence (AI) and immunomodulation that may influence future management of pediatric VUR. METHODS: A forward-looking literature review was performed using the PubMed database (January 2000-March 2025), focusing on studies addressing AI, immunomodulation, or vaccination in the context of VUR and urinary tract infections. Criteria of inclusion were the relevance to pediatric VUR, the novelty of the proposed concept, the potential clinical implications and, for the AI literature, the existence of a clinical evaluation of the algorithm on a dataset from patients. KEY FINDINGS AND LIMITATIONS: AI-based models show promising performance in supporting clinical decision-making, including prediction of the need for voiding cystourethrography, automated grading of VUR, estimation of recurrent urinary tract infection risk and prediction of chemoprophylaxis. These tools may facilitate more individualized diagnostic and therapeutic strategies, although current evidence is largely retrospective and requires prospective validation. Immunization and immunomodulatory approaches aim to reduce infection burden and modulate inflammatory pathways associated with renal scarring. While early experimental and adult clinical data are encouraging, pediatric-specific evidence remains limited, and clinical applicability in children with VUR is not yet established. CONCLUSION: Artificial intelligence and immunologically targeted strategies represent complementary, emerging approaches that may contribute to more personalized management of pediatric VUR. At present, both should be regarded as exploratory tools whose clinical impact will depend on further validation and appropriately designed pediatric studies.
INTRODUCTION: Most studies in pediatric urology have been limited to single-center retrospective designs with inherent limitations. Ideally, pediatric urology research would be collaborative, cross-institutional, and gui...INTRODUCTION: Most studies in pediatric urology have been limited to single-center retrospective designs with inherent limitations. Ideally, pediatric urology research would be collaborative, cross-institutional, and guided by shared priorities supported by funding mechanisms that enable rigorous study designs. OBJECTIVE: To promote collaborative research in the highest-priority areas of the field, we aimed to engage pediatric urologists to establish a shared clinical research agenda through a structured, consensus-based process. STUDY DESIGN: We employed a 3-phase approach. First, we surveyed frontline pediatric urologists in the United States to elicit research questions addressing gaps in clinical knowledge. Next, we reviewed and refined responses to inform a three-round modified Delphi prioritization with a 22-member working group. Questions meeting consensus in the final Delphi round were advanced to the final phase where an in-person 11-member workshop developed a preliminary research agenda. This agenda was subsequently finalized through electronic feedback and endorsement by the full working group. RESULTS: Eighty-eight pediatric urologists submitted 256 questions during the elicitation phase. Overlapping or redundant submissions were consolidated, resulting in 64 unique questions that advanced to prioritization. Following three Delphi rounds, this list was narrowed to 10, and the consensus workshop endorsed 6 final priorities. DISCUSSION: We describe a structured, consensus-based process to establish clinical research priorities for pediatric urology. Through three phases of elicitation, prioritization, and a consensus workshop, we engaged the pediatric urology community to identify six clinical research priorities. Next steps include targeted dissemination to major funders and professional societies to guide future research and funding priorities. The initial phase of this study focused on clinician input and was limited in stakeholder diversity due to lack of representation from allied health professionals and researchers who are not practicing urologists and other specialists in urologic disease (e.g., nephrology, infectious diseases) as well as lack of patient or family perspectives. The next phase will include further refinement of these broad priorities into actionable research questions through an iterative, multi-stakeholder process involving affected patients and families as well as interdisciplinary providers and methodological panelists. CONCLUSION: This clinician-prioritized research agenda reflects the most pressing clinical research gaps in pediatric urology in the United States, identified through broad engagement with pediatric urologists. Future work should ensure alignment of clinician-identified priorities with those of patients and caregivers. The ultimate goal is to direct collaborative, multi-institutional, and multinational efforts that strengthen the evidence base, inform clinical guidelines, and improve patient care in pediatric urology.
INTRODUCTION: Approximately 30% of all male childhood cancer survivors receive gonadotoxic therapy increasing their risk for infertility. The only current option for potential fertility preservation in prepubertal males...INTRODUCTION: Approximately 30% of all male childhood cancer survivors receive gonadotoxic therapy increasing their risk for infertility. The only current option for potential fertility preservation in prepubertal males is through testicular tissue cryopreservation (TTC), available through clinical trials. Our aim was to evaluate our experience and identify barriers to enrollment in the TTC study. METHODS: A retrospective review was conducted to describe a single center experience with TTC, including an assessment of factors impacting the decision to enroll or not enroll in the TTC trial. RESULTS: A total of 61 patients were found to be eligible for enrollment in the TTC trial, with 23 patients (34%) choosing to enroll and undergo testicular tissue cryopreservation including 19 (83%) oncology patients. In patients who underwent TTC there were no unexpected delays in starting disease directed therapy. For families that declined enrollment, many did not see fertility preservation as a priority at the time of counseling. (n = 17, 44%). Other barriers included concerns about post-operative complications and a feeling of being overwhelmed at the time of counseling. DISCUSSION: Despite being the only preservation option for pre-pubertal boys facing risk of infertility, TTC enrollment was not pursued by a majority of eligible patients and families. Identifying potential barriers or misconceptions, particularly in regard to post operative complication risks, offer opportunities for improved counseling and education. Early introduction of the Fertility Navigation team allows for procedural coordination success and access for patients that are interested. Success is not measured in how many patients proceed with TTC, rather that all patients have the opportunity for counseling and discussion. CONCLUSION: TTC can be offered and completed safely without delays in disease-directed treatment timelines. A Fertility Navigation Program allows for equal opportunity for education and enrollment on TTC protocol which should be the goal for pediatric institutions, though is not pursued by many patients. A majority of those who declined the study did not see fertility as a priority at the time of treatment while others were concerned regarding peri-operative risks.
PURPOSE: Prune Belly Syndrome (PBS) is a rare congenital malformation complex characterized by cryptorchidism, urinary tract abnormalities and deficiency of the abdominal wall musculature. Although advances in neonatal a...PURPOSE: Prune Belly Syndrome (PBS) is a rare congenital malformation complex characterized by cryptorchidism, urinary tract abnormalities and deficiency of the abdominal wall musculature. Although advances in neonatal and reconstructive care have substantially improved survival, the long-term risk of malignancy in this population remains poorly defined. We report a novel case of early-onset metastatic bladder carcinoma in a patient with PBS and perform a systematic review of all tumors described in association with the syndrome. METHODS: A systematic literature review was performed according to PRISMA guidelines and registered in PROSPERO (CRD420251072086). Databases searched through April 2025 included PubMed, Embase, Web of Science, ClinicalTrials.gov, Cochrane, and SciELO. Reported tumor cases in PBS patients were identified and classified as either PBS-associated or not. We additionally describe a new case of invasive bladder carcinoma. RESULTS: Seventeen studies comprising 20 patients met inclusion criteria. Tumors directly related to the genitourinary tract accounted for half of the cases. Bladder carcinoma was the most frequent neoplasm (35%), followed by hepatoblastoma (25%). Bladder tumors occurred at markedly younger ages and often presented as advanced diseases. Our index case involved a 33-year-old man with PBS who developed metastatic muscle-invasive urothelial carcinoma and died shortly after diagnosis. DISCUSSION: Improved survival in PBS has led to recognition of new late complications, including malignancy. Tumorigenesis may be influenced by chronic urinary stasis, infection, catheterization, cryptorchidism, and underlying genitourinary developmental abnormalities. These findings should be interpreted as hypothesis-generating given the limited number of reported cases. CONCLUSION: Although uncommon, malignancies in PBS may occur earlier and behave more aggressively than in the general population. Given the current level of evidence, careful long-term follow-up with clinical awareness is warranted, rather than routine invasive screening for all patients.
INTRODUCTION AND OBJECTIVE: Traditionally, patients with high anorectal malformations (ARM) are considered to have a worse associated urological functional prognosis and are actively managed in pediatric urology units. H...INTRODUCTION AND OBJECTIVE: Traditionally, patients with high anorectal malformations (ARM) are considered to have a worse associated urological functional prognosis and are actively managed in pediatric urology units. However, the evidence supporting this risk-based stratification remains limited. This study aimed to determine whether the height of the malformation or the presence of associated anomalies influences the risk of lower urinary tract dysfunction (LUTD) in a large cohort of children with ARM, in order to assess the need for systematic urological surveillance. MATERIALS AND METHODS: We conducted a retrospective cohort study of ARM patients followed by the "Multidisciplinary URIMAR clinic" (pediatric colorectal and urology) from January 2017 to December 2024. Demographic and clinical data, as well as current bladder function, were collected from medical records. Patients with cloacal malformations were excluded. Patients were divided into groups according to the presence of dysraphism and height of ARM. Bivariate analyses were performed using LUTD symptom variables (DVISS), urodynamic tests (both invasive and non-invasive), and treatment requirements. RESULTS: From a general registry of 108 patients with ARM, a total of 58 (63.8% male) patients met inclusión criteria. Spinal anomalies and congenital anomalies of kidney and urinary tract (CAKUT) were present in 27.5% and 41.3% of patients, respectively. Urinary symptoms were reported in 41.4% of patients, most commonly urinary hesitancy (19%), daytime incontinence (15.5%), and urinary urgency (12.1%). Urodynamic abnormalities were found in 55.1%, with plateau curve being the most frequent finding (93%) followed by significant postvoid residue (40.6%) and electromyographic activity (15.6%). Notably, 72% of asymptomatic patients showed abnormal flowmetry. No significant differences in the prevalence of urinary symptoms (p = 0.392), urodynamic abnormalities (p = 0.501), or pharmacological requirements (p = 0.512) were observed according to the height of the malformation. Similarly, no significant differences were found according to the presence of spinal anomalies in the prevalence of urinary symptoms (p = 0.52) or urodynamic abnormalities (p = 0.448). However, patients with ARM and spinal defects were more likely to require clean intermittent catheterization (p = 0.02) and showed a tendency toward requiring anticholinergic drugs (p = 0.1). DISCUSSION: LUTD was observed across all anatomical subtypes of ARM, including patients with low malformations and no spinal dysraphism. Although the severity of LUTD was magnified by the presence of spinal anomalies, no anatomical subtype guaranteed normal bladder function. Notably, almost 50% of asymptomatic patients with a normal spine exhibited abnormal uroflowmetry patterns. These findings challenge the traditional risk stratification based on malformation height and support the universal indication for urological follow-up in all patients with ARM, regardless of anatomical subtype or the presence of spinal defects. CONCLUSION: LUTD constitutes a fundamental concern for the entire population of children with ARM, regardless of the height of malformation. Although patients with associated spinal anomalies face a disproportionately higher risk, the absence of such anomalies does not rule out the possibility of LUTD. These findings support the implementation of systematic and mandatory urological surveillance programs for all ARM patients to ensure early detection and intervention.
Penile agenesis is an extremely rare congenital anomaly that poses major challenges for urological reconstruction. The appendix, due to its consistent vascular supply and mobility, represents a promising option for compl...Penile agenesis is an extremely rare congenital anomaly that poses major challenges for urological reconstruction. The appendix, due to its consistent vascular supply and mobility, represents a promising option for complex urethral reconstructions. This case report describes a 10-year-old boy with penile agenesis who initially underwent perineal urethrostomy and colostomy, followed by neophalloplasty using a scrotal flap and Vantris injections to enhance neophallus volume. Later, urethral reconstruction was performed using a pedicled appendiceal graft harvested robotically and implanted into the neophallus with microsurgical precision, preserving vascularity. The procedure was successful, and the patient is awaiting the next stage for urethral anastomosis. This study presents a minimally invasive technique employing the appendix as a neourethra, combining functional and aesthetic outcomes in the challenging context of penile agenesis.
INTRODUCTION: Lower urinary tract dysfunction (LUTD) significantly impacts children's health-related quality of life (HRQoL), but key determinants of this impairment remain incompletely understood within a patient-center...INTRODUCTION: Lower urinary tract dysfunction (LUTD) significantly impacts children's health-related quality of life (HRQoL), but key determinants of this impairment remain incompletely understood within a patient-centered framework. METHODS: This retrospective cross-sectional study enrolled 272 children with LUTD. Clinical symptoms were assessed using the Dysfunctional Voiding Scoring System (DVSS) and the Overactive Bladder Symptom Score (OABSS), and HRQoL via the Pediatric Quality of Life Inventory (PedsQL™) 4.0 Generic Core Scales. RESULTS: The mean total PedsQL score was 83.66 ± 16.07, with the School Functioning domain being the most impaired (73.75 ± 20.94). Univariate analysis identified older age (≥11 years), daytime urinary leakage, overactive bladder (OAB) symptoms, and abnormal DVSS scores as factors associated with reduced HRQoL (all P < 0.05); multivariate regression further confirmed only older age and abnormal DVSS were independent influencing factors (both P < 0.001). A significant negative correlation existed between the total DVSS score and the total PedsQL score (r = -0.390, P < 0.01), indicating that greater symptom severity predicts poorer overall HRQoL. CONCLUSION: This study indicates that in children with LUTD, the severity of voiding dysfunction (quantified by DVSS) is inversely correlated with HRQoL. Voiding dysfunction impairs multiple domains of daily functioning, including physical well-being, emotional adjustment, social interaction, and school performance. Shifting from a symptom-focused to a patient-centered care model and integrating routine HRQoL assessment into standard clinical management can optimize clinical practice for pediatric LUTD.
INTRODUCTION: Historically, children with ureteroceles presented symptomatically and were managed surgically. It is unclear if this changed in the modern medical era of prenatal imaging and shared decision making. We aim...INTRODUCTION: Historically, children with ureteroceles presented symptomatically and were managed surgically. It is unclear if this changed in the modern medical era of prenatal imaging and shared decision making. We aimed to describe the presentation and management of ureteroceles during initial urological evaluation of children in the era of widespread prenatal ultrasonography. PATIENTS AND METHODS: We retrospectively reviewed records of children (<18 years old [yo]) initially evaluated at our center with a ureterocele (2011-2020). We analyzed demographics, renal anatomy, initial presentation for evaluation, and initial management with non-parametric statistics. Febrile urinary tract infections (fUTIs, ≥ 38 °C) were classified as 1) urosepsis (positive urine culture admitted to pediatric intensive care), 2) documented (positive urine culture) or 3) family-reported. RESULTS: We identified 188 children (65% female). Median age at presentation was 1.2 months old (mo) (IQR 18 days-4.4 mo). Antenatally-detected congenital anomalies of the kidney and urinary tract (aCAKUT) were noted in 143 (76%) children with a confirmed postnatal diagnosis of ureterocele. Overall, 129/188 (69%) children presented without symptoms and 59 (31%) presented with symptoms. fUTI was the most common symptomatic presentation (46/188, 24%): urosepsis (6 children), documented (30), and family-reported (10). Children with aCAKUT presented earlier than those without aCAKUT (27 days vs. 1.6 yo, p < 0.0001). They were also less likely to present with symptoms (11% vs. 96%, p < 0.0001), including fUTIs (7% vs. 78%, p < 0.0001). In total, 108 children (57%) were initially managed with transurethral incision, 73 (39%) were observed, and 7 (4%) had reconstructive surgery. Asymptomatic children with aCAKUT (42%) and symptomatic children without aCAKUT (37%) were more likely to be observed than symptomatic children with aCAKUT (7%, p = 0.02). Among 143 children with aCAKUT, those on antibiotic prophylaxis were less likely to present with a history of a fUTI compared to those not on prophylaxis (4/106 vs. 6/37, 4% vs. 16%, p = 0.02). COMMENT: We present a large observational study describing clinical and anatomical characteristics of children presenting with ureteroceles in a medical era of ubiquitous prenatal ultrasonography. Our retrospective study was limited by incomplete documentation of all antenatal ultrasonography and adherence with antibiotic prophylaxis. Long-term clinical outcomes will be the focus of future work. CONCLUSION: In contrast to historical cohorts, most children presented to urologists with asymptomatic ureteroceles diagnosed with aCAKUT. Most children without aCAKUT presented with a fUTI. Overall, 39% of children were initially observed, indicating an increased use of observation in the modern medical era.