Long-term psychosexual adjustment of adults born with classic bladder exstrophy.

BACKGROUND: In literature studies exploring long-term psychosexual development and intimacy of adults living with the complex genitourinary conditions associated with classic bladder exstrophy (BE) are scarce, with small sample sizes and lacking in methodology. OBJECTIVE: This study aims to examine areas of potential psychosexual distress in adults born with classic BE to develop targeted clinical interventions. STUDY DESIGN: The validated Sexrelation Evaluation Schedule Assessment Monitoring (SESAMO) questionnaire was administered to all BE patients aged ≥18 years operated on in our tertiary referral center during infancy. Z-scores were calculated for each area of interest, considering i) gender (female vs male); ii) committed partnership status (singles vs couples); iii) the voiding technique adopted to empty the bladder. RESULTS: A total of 33 (F:M 12:21; singles: couples 11:22) adults with BE were enrolled in the study at a median age of 39 (32-47) years. Overall, BE adults performed the worst regarding psychosexual identity (z-score:-1.282), pleasure (z-score:-0.915) and desire (z-score:-0.583); singles regarding relational attitude (z-score:-1.751) and imaginative eroticism (z-score:-0.806); couples regarding extramarital sexuality (z-score:-1.175) and sexual communication (z-score:-0.255). When it came to gender, females significantly performed worse than males regarding psychosexual identity (-1.645 vs -1.282; p-value:<0.0001) and areas of pleasure (-1.126 vs -0.359; p-value:<0.001). Single females performed worse than males regarding actual masturbation (-0.763 vs 0.583; p-value:<0.05) and better regarding relational attitude (-1.226 vs -1.751; p-value:<0.05). Females in stable relationships performed worse than males regarding actual masturbation (-1.645 vs 0.306; p-value:<0.05) and better regarding sexual intercourse (1.866 vs -0.565; p-value:<0.01). The voiding techniques used to empty the bladder did not show any influence on these results. CONCLUSION: Adults with BE have a greater likelihood of experiencing a wide range of psychosexual difficulties. Identifying the specific areas of psychological distress can help them cope with their medical experience and actual clinical condition and clinicians plan adequate psychological interventions.

The "V-I penoscrotal reconfiguration": A simple technique for the surgical treatment of congenital webbed penis.

OBJECTIVES: To describe a new penoscrotal reconfiguration technique, named "V-I penoscrotal reconfiguration" for the surgical reconstruction of a congenital webbed penis (CWP). METHODS: Twenty-one patients who underwent the "V-I penoscrotal reconfiguration technique" were included in this retrospective study. The CWP severity was assessed according to El-Koutby's classification. Demographic and clinical data, surgical data, and postoperative outcomes were scheduled and analyzed. Specifically, the postoperative follow-up included both physical and psychological assessments at 2 weeks, 1, 6, and 12 months after surgery. Parents' satisfaction degree was quantified by the Likert scale. RESULTS: CWP was grade 3 in 11 (52%) patients, 2 in five (24%), and 1 (24%) in five. Five (24%) CWP were isolated malformations, 11 (52%) were associated with phimosis, three (14%) with hypospadias, and two (10%) with hypospadias and phimosis. There were no postoperative complications and no cases of redo surgery. The cosmetic outcomes were excellent in all cases: the parents' satisfaction score was 4 in 17 (81%) cases and 3 (9%) in the other four cases. CONCLUSIONS: CWP may cause psychological distress and functional problems, especially during sexual intercourse. Its correction in childhood is advocated to prevent psychological and sexual issues. The "V-I reconfiguration technique" is simple, and easy with excellent cosmetic and functional outcomes.

Uncommon Haemorrhagic Cystitis of Infectious Origin: A Narrative Review for Urologists.

Purpose: Haemorrhagic cystitis may be due to different etiologies with infectious diseases representing an insidious cause to diagnose. The aim of this narrative review is to provide a comprehensive overview of less common but difficult-to-diagnose causes of infectious haemorrhagic cystitis of bacterial, mycobacterial, and parasitic origin, Moreover, we highlight possible diagnostic tools and currently available treatment options in order to give an updated tool for urologists to use in daily practice. Patients and Methods: The search engine PubMed was used to select peer-reviewed articles published from 1/Jan/2010 to 31/Aug/2022. Results: Bacteria, fungal, TB and schistosomiasis are uncommon causes of haemorrhagic cystitis burdened by high morbidity, especially if not promptly diagnosed. Conclusion: Because haemorrhagic cystitis ranges in severity from mild dysuria associated with pelvic discomfort to severe life-threatening haemorrhage, punctual diagnosis, and immediate treatment are essential to avoid further complications.

Indocyanine Green (ICG)-Guided One-Stage Delayed Bladder Closure and Radical Soft-Tissue Mobilization (Kelly Procedure) For Bladder Exstrophy Repair: The First Experience.

The vascular supply of the pelvic structures and the external genitalia can be easily injured during the one-stage delayed bladder closure and radical soft-tissue mobilization (Kelly procedure) for bladder exstrophy surgical repair. Aiming to help surgeons assessing and confirming tissue perfusion and viability, indocyanine green (ICG)-based laser angiography was incorporated into the operative approach to reduce the risk of ischemic injuries. The EleVision IR system (Medtronic Ltd) was adopted to confirm the identification of the vascular pedicles and assess the tissue perfusion in real-time in a 5-month-old with bladder exstrophy undergoing the one-stage delayed bladder closure and radical soft-tissue mobilization (Kelly procedure). ICG (0.15 mg/kg) was intravenously administered at 6 key steps during surgery with the ability to be re-dosed every 15 minutes. ICG-based laser angiography helped to confirm the correct identification of the vascular structures during surgery and to assess tissue perfusion in real-time. Blood flow did not change considerably after initial dissection or upon approximating the pubis symphysis. At the end of the procedure, good penile perfusion was shown, proving that no direct injury or substantial compression of the pudendal vessels had occurred following the mobilization and the reconstructive phase. ICG-based laser angiography proved to be safe, effective, and easy to employ and should be considered as a reasonable adjunct for tissue perfusion assessment and operative decision-making in patients undergoing bladder exstrophy Kelly repair.

Indocyanine Green (ICG)-Guided Onlay Preputial Island Flap Urethroplasty for the Single-Stage Repair of Hypospadias in Children: A Case Report.

First described by Duckett in 1981, and initially employed for the surgical correction of mid-penile hypospadias, the onlay preputial island flap urethroplasty has progressively gained increasing popularity, extending its indication to proximal forms. However, with the complexity of the penile anomaly, the rate of postoperative complications related to poor tissue perfusion (including skin and glans dehiscence, urethral stenoses, and fistulas) has also increased. Conventionally, the visual assessment of the onlay preputial island flap is the only option available to establish the appropriate tissue vascularization during surgery. To this end, we have first introduced the EleVision IR system (Medtronic Ltd., Hong Kong, China) to assess the vascular perfusion of the preputial island flap in a 13-month-old boy undergoing the onlay urethroplasty for the surgical correction of a mid-shaft hypospadias. This was possible 80 s after the intravenous injection of indocyanine green (ICG, 0.15 mg/kg, Diagnostic Green GmbH, Munich, Germany). ICG-based laser angiography helped define the proximal resection margin of the preputial flap, and proved to be safe, effective, and easy to employ. This innovative intraoperative imaging modality can be considered a useful adjunct for tissue perfusion evaluation and intraoperative decision-making during the onlay preputial island flap urethroplasty in children.

The definition of ideal training of a urology resident from two different perspectives: trainees vs professors. Is there agreement in their idea of good training?

Introduction: At the end of their residency program, urology trainees should reach the minimum skills required to be able to work by themselves and within a team. To achieve this objective, it is fundamental that the training involves not only surgical activities, but also theoretical, academic, and relational ones. What is the perfect balance between these activities within the ideal urological training? This study aims to evaluate the concordance in different concepts of good urological training between different perspectives (trainees vs professors). Material and methods: Between January and December 2020 the same survey was distributed via email to 967 urology trainees and urology tutors. The survey investigated 5 educational fields: theoretical, clinical, surgical, relational, and simulation. For each field, specific questions investigated the importance of different activities and the training outcomes considered fundamental to be reached by a resident. The questions were evaluated by responders through a Likert 10-point scale. Results: The survey was completed by 155 trainees (58.9%, Group A) and 108 tutors (41.1%, Group B) from 26 different countries. Relative to the tutors, residents assigned statistically significantly lower scores to prostate biopsy (median score 9.11 vs 9.24), robotic simulator training (5.66 vs 5.93), on-call duties with consultants (6.85 vs 7.99), as well as all aspects of relational training (e.g., proper dialogue with colleagues: 7.95 vs 8.88). Conversely, residents assigned statistically significantly higher scores, albeit below sufficiency, to the performance of robotic prostatectomy as a first operator (4.45 vs 4.26). Finally, no discrepancies between residents' and tutors' scores were recorded regarding the remaining items of clinical training (e.g., urodynamics, outpatient clinic, ward duties) and surgical training (e.g., major open, laparoscopic and endoscopic surgical training; all p values >0.05). Conclusions: There was partial concordance between trainees and tutors regarding the activities that should be implemented and the skills that should be achieved during a urological residency. The residents aimed for more surgical involvement, while the tutors and professors, although giving importance to surgical and theoretical training, considered clinical practice as the fundamental basis on which to train future urologists.

Pediatric vulvar lichen sclerosus: Does it resolve or does it persist after menarche?

BACKGROUND AND OBJECTIVES: Although data regarding the rates of remission and progression of the disease are still scarce, it is generally now acknowledged that pediatric vulvar lichen sclerosus (pVLS) can persist beyond puberty. Recent studies reveal that this condition may persist in as many as 75% of cases. The present study aims to answer the following query: how does pVLS evolve after menarche? METHODS: This observational retrospective study conducted on premenarchal girls diagnosed with pVLS in our institution between 1990 and 2011 describes 31 patients who returned for multidisciplinary clinical evaluation following menarche. RESULTS: The mean follow-up time was 14 years. At the post-menarche clinical examination, patients were classified as follows: 58% were still affected by VLS, 16% presented with a complete remission of disease, and 26% were completely asymptomatic although with persistent clinical signs of VLS. CONCLUSIONS: In our series, pVLS persists following menarche in the majority of patients. These findings suggest the importance of a long-term follow-up even among patients who report resolution of symptoms following menarche.

Radiation Exposure and Surgical Outcomes after Antegrade Sclerotherapy for the Treatment of Varicocele in the Paediatric Population: A Single Centre Experience.

INTRODUCTION: Antegrade sclerotherapy (Tauber) effectively treats varicocele. However, fluoroscopy exposes young males to ionizing radiation. We aimed to evaluate radiation exposure and surgical outcomes after the Tauber procedure. MATERIALS AND METHODS: We retrospectively analysed data from 251 patients. Dose area product (DAP) and fluoroscopy time were recorded. The effective dose was calculated with the PCXMC software. Descriptive statistics and linear regression tested the association between clinical predictors and radiation exposure. RESULTS: Median (IQR) age and body mass index (BMI) were 14 (13-16) years and 20.1 (17.9-21.6) kg/m². Five (2.1%) patients developed clinical recurrence and two (0.81%) developed complications. Median fluoroscopy time and DAP were 38.5 (27.7-54.0) s and 89.6 (62.5-143.9) cGy*cm2. The effective dose was 0.19 (0.14-0.31) mSv. Fluoroscopy time was higher in patients with collateral veins (41 (26-49) s vs. 36 (31-61) s, p = 0.02). The median amount of sclerosing agent (SA) used was 3 (3-4) ml. DAP was higher when SA > 3 mL was used (101.4 (65-183) cGy*cm2 vs. 80.5 (59-119) cGy*cm2; p < 0.01). At univariable linear regression, age, BMI, operative time and SA > 3 mL were associated with higher DAP (all p < 0.01). At multivariable linear regression, only BMI (beta 12.9, p < 0.001) and operative time (beta 1.9, p < 0.01) emerged as predictors of higher DAP, after accounting for age and SA > 3 mL. CONCLUSIONS: The Tauber procedure is safe and associated with low effective doses. Operative time and the patient's BMI independently predict a higher radiation dose.

A Unique Case of Inflatable Penile Prosthesis (IPP) Implantation and Mesh Phalloplasty in a Patient Born with Congenital Fusiform Megalourethra.

We present the first case of a patient born with congenital fusiform megalourethra who underwent the successful placement of an inflatable penile prosthesis (IPP) and mesh phalloplasty due to primary erectile dysfunction (ED). After an extensive psychosexual assessment and a preoperative MRI scan, an IPP was successfully implanted. The procedure was carried out through a penoscrotal approach, which offered excellent exposure to the crura and the proximal ends of the corpora cavernosa. Following the incision and the dilatation of both corpora cavernosa, the penile cylinders were inserted and a mesh phalloplasty was performed, to replace the erectile tissues lacking in the distal corpora cavernosa. After reservoir and pump placement, the device was tested, and no mechanical issues were recorded. The postoperative course was uneventful, and, at the 2-year follow-up, the patient reported highly satisfactory results, with valid functional erections.

Case Report: effect of lumasiran treatment in a late preterm baby with antenatal diagnosis of primary hyperoxaluria type 1.

Background: Primary hyperoxaluria type 1 (PH1) is a rare disease with autosomal recessive transmission, characterized by increased urinary excretion of oxalate, resulting in chronic kidney disease secondary to recurrent urolithiasis, nephrocalcinosis, and accumulation of oxalate in various organs and tissues (systemic oxalosis). Since 2020, an innovative pharmacological approach, namely, lumasiran, has been added to the therapeutic armamentarium (dialysis and liver-kidney transplantation). The purpose of this paper is to describe the effect of lumasiran initiated at 10 days of life in a newborn with prenatally diagnosed PH1. A female fetus was prenatally diagnosed with hyperoxaluria type 1, based on family history and genetic testing. Her brother had the onset of the disease at 2 months of age and underwent liver and kidney transplantation at 13 months and 8 years of age, respectively. The baby was born late preterm at 36 weeks + 4 days of gestation via spontaneous labor, and lumasiran for compassionate use was started on the tenth day of life. At 20 months of age, the baby showed normal urinary oxalate values and kidney function, while the plasma oxalate level was under the threshold of oversaturation. There were no signs of systemic oxalosis. Conclusions: Early use of lumasiran in young infants, who do not yet show signs of the disease, represents a therapeutic challenge for the pediatric nephrologist. The ability of the drug to act on the hepatocyte of the newborn and the most appropriate dosage to be used in these very young babies have yet to be clarified.

Fluorescence-Guided Surgery and Novel Innovative Technologies for Improved Visualization in Pediatric Urology.

Fluorescence-guided surgery (FGS), three-dimensional (3D) imaging technologies, and other innovative devices are rapidly revolutionizing the field of urology, providing surgeons with powerful tools for a more complete understanding of patient-specific anatomy. Today, several new intraoperative imaging technologies and cutting-edge devices are available in adult urology to assist surgeons in delivering personalized interventions. Their applications are also gradually growing in general pediatric surgery, where the detailed visualization of normal and pathological structures has the potential to significantly minimize perioperative complications and improve surgical outcomes. In the field of pediatric urology, FGS, 3D reconstructions and printing technologies, augmented reality (AR) devices, contrast-enhanced ultrasound (CEUS), and intraoperative magnetic resonance imaging (iMRI) have been increasingly adopted for a more realistic understanding of the normal and abnormal anatomy, providing a valuable insight to deliver customized treatments in real time. This narrative review aims to illustrate the main applications of these new technologies and imaging devices in the clinical setting of pediatric urology by selecting, with a strict methodology, the most promising articles published in the international scientific literature on this topic. The purpose is to favor early adoption and stimulate more research on this topic for the benefit of children.

Pediatric Urolithiasis: Current Surgical Strategies and Future Perspectives.

New technological innovations and cutting-edge techniques have led to important changes in the surgical management of pediatric urolithiasis. Miniaturized technologies and minimally invasive approaches have been increasingly used in children with urinary stones to minimize surgical complications and improve patient outcomes. Moreover, the new computer technologies of the digital era have been opening new horizons for the preoperative planning and surgical treatment of children with urinary calculi. Three-dimensional modeling reconstructions, virtual, augmented, and mixed reality are rapidly approaching the surgical practice, equipping surgeons with powerful instruments to enhance the real-time intraoperative visualization of normal and pathological structures. The broad range of possibilities offered by these technological innovations in the adult population finds increasing applications in pediatrics, offering a more detailed visualization of small anatomical structures. This review illustrates the most promising techniques and devices to enhance the surgical treatment of pediatric urolithiasis in children, aiming to favor an early adoption and to stimulate more research on this topic.

Definition, diagnosis and management of fetal lower urinary tract obstruction: consensus of the ERKNet CAKUT-Obstructive Uropathy Work Group.

Fetal lower urinary tract obstruction (LUTO) is associated with high mortality and postnatal morbidity caused by lung hypoplasia and impaired kidney function. Specific diagnostic features that can guide clinical approach and decisions are lacking; thus, the European Reference Network for Rare Kidney Diseases established a work group to develop recommendations regarding the clinical definition, diagnosis and management of prenatally detected LUTO. The work group recommends the use of antero-posterior diameter of renal pelvis as the most reliable parameter for suspecting obstructive uropathies and for suspecting prenatal LUTO in the presence of fetal megacystis. Regarding prenatal and postnatal prognosis of fetuses with LUTO, the risk of fetal and neonatal death depends on the presence of oligohydramnios or anhydramnios before 20 weeks' gestation, whereas the risk of kidney replacement therapy cannot be reliably foreseen before birth. Parents of fetuses with LUTO must be referred to a tertiary obstetric centre with multidisciplinary expertise in prenatal and postnatal management of obstructive uropathies, and vesico-amniotic shunt placement should be offered in selected instances, as it increases perinatal survival of fetuses with LUTO.

Semi-closed-circuit vacuum-assisted mini percutaneous nephrolithotomy in the pediatric population: the initial experience of two tertiary referral centers.

BACKGROUND: Percutaneous nephrolithotomy (PCNL) is the gold-standard for complex renal stones treatment in the pediatric population. While the miniaturization of PCNL reduces the risk of bleeding, it can hinder surgical and functional outcomes. The aim of the study was to assess the safety and feasibility of semi-closed-circuit vacuum-assisted Mini-PCNL (vmPCNL) in pediatric patients. METHODS: From January 2017 to December 2018, we prospectively collected data on consecutive vmPCNLs from two European tertiary referral centers. The procedure was performed with the ClearPetra® (Well Lead Medical Co., Ltd., Guangzhou, China) access sheath equipped with a lateral arm connected to the aspiration system (pressure setting ~120-150 cmH2O) by a 200 Ml plastic stone collector. Pre-, intra- and postoperative data and costs were analyzed. The stone-free rate (SFR) was defined as absence of residual fragments >4 mm with either ultrasound or kidney, ureter, and bladder X-ray. RESULTS: Eighteen vmPCNLs were performed in 16 renal units of 13 children. The median age was 119 months (IQR: 97-160) and the weight was 29 Kg (IQR: 25-40). The median cumulative stone size was 32 mm (22-46) with 8 (44.4%) cases of staghorn stones. The OT was 128 min (IQR: 99-167). The basketing was unnecessary in 6/18 (33%) cases. Neither intra-operative complications nor blood transfusions occurred. Postoperative fever was observed in 5/18 (27.8%) cases; in one case a double J ureteral stent was placed for concomitant hydronephrosis. The SFR was 81.3% (13/16), rising to 93.8% (15/16) after ancillary procedures. The materials costs of a vmPCNL (734.8 €) were comparable to mini-PCNL using a reusable set (710.7 €). CONCLUSIONS: The vmPCNL seems to be sustainable, safe and feasible for kidney stones treatment in the pediatric population.

Comparison of Cosmetic Results in Children >10 Years Old Undergoing Open, Laparoscopic or Robotic-Assisted Pyeloplasty: A Multicentric Study.

PURPOSE: Our goal was to compare surgical scars assessed by a validated patient reported outcome questionnaire in children undergoing open (OP), laparoscopic (LP), or robotic-assisted (RALP) pyeloplasty. Our secondary aim was to assess the influence on the outcomes of variables such as gender or body mass index (BMI). MATERIALS AND METHODS: We conducted an observational, cross-sectional, multicentric study of patients undergoing primary pyeloplasty between age 10 and 18 years at 5 tertiary Italian institutions during the period January 2010 to December 2019. Of 227 eligible patients 114 (50%) participated. OP was performed in 37 (32%), LP in 30 (26%) and RALP in 47 (41%), After a median (IQR) followup of 5.2 (2.3-7.8) years, scars were measured and assessed by a validated Patient Scar Assessment Questionnaire. Scores were compared among techniques and in accordance with several variables. RESULTS: The median length of the surgical scar at followup was significantly larger (p <0.0001) after OP (8.1 cm vs 1.8 cm for LP and 2.0 cm for RALP), where scar length correlated with BMI (p=0.04). Ninety patients (79%) had a Patient Scar Assessment Questionnaire score within the first quartile, the most favorable. During followup, 43 (38%) participants reported scar-related symptom. Symptoms were generally more common after OP (54% vs 30% for LP and 30% for RALP, p=0.06) and scar hyperesthesia was significantly more frequent after OP (p=0.01). CONCLUSIONS: Perception of the cosmetic outcomes in pre-adolescents and adolescents after pyeloplasty was generally good. LP achieved the best cosmetic results. OP was more commonly associated with scar-related symptoms and the size of the incision paralleled BMI.