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.

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.

The Discipline of Pediatric Urology: Prerogatives and Necessities.

To the Editor, The aim of this "position paper" is to describe the discipline of Pediatric Urology with its clinical and cultural competencies, represent the reasons for legitimizing its existence, and reinforce its importance in the "scenario" of the National Italian Healthcare System. The requisites and the educational requirements were defined by both the Italian Ministry of Health with the State-Regions Conference, and the European Union [...].

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 clinical management of non-obstructive kidney dysplasia: a consensus statement by the ERKNet Working Group on Kidney Malformations.

Kidney dysplasia is one of the most frequent causes of chronic kidney failure in children. While dysplasia is a histological diagnosis, the term 'kidney dysplasia' is frequently used in daily clinical life without histopathological confirmation. Clinical parameters of kidney dysplasia have not been clearly defined, leading to imprecise communication amongst healthcare professionals and patients. This lack of consensus hampers precise disease understanding and the development of specific therapies. Based on a structured literature search, we here suggest a common basis for clinical, imaging, genetic, pathological and basic science aspects of non-obstructive kidney dysplasia associated with functional kidney impairment. We propose to accept hallmark sonographic findings as surrogate parameters defining a clinical diagnosis of dysplastic kidneys. We suggest differentiated clinical follow-up plans for children with kidney dysplasia and summarize established monogenic causes for non-obstructive kidney dysplasia. Finally, we point out and discuss research gaps in the field.

Whole blood fatty acid profile of young subjects and adherence to the Mediterranean diet: an observational cohort study.

BACKGROUND: Relatively little is known about the physiological whole blood fatty acid composition in young people. Likewise, few studies have addressed the question of correlations between Mediterranean diet (MedDiet) adherence and blood fatty acids in childhood. METHODS: The fatty acid profile in whole blood from subjects, 46 days-19 years old (n = 152), without acute, chronic, or inflammatory diseases was analysed by gas chromatography. Dietary data was extracted from a 24-h recall in a subgroup of subjects (n = 60) into a modified Diet Quality Index for Children (KIDMED) questionnaire to evaluate MedDiet adherence. The cohort was divided into three age groups: < 2, 2- < 10, and 10-19 years. Kruskal-Wallis test and Bonferroni post hoc test were used to check for age group fatty acid differences. For correlations, Spearman's correlation coefficient and partial Spearman's correlation coefficient were used. RESULTS: Linoleic acid, EPA, DHA, palmitic acid, and total saturated fatty acids were stable over age groups. Dihomo-gamma-linolenic acid (DGLA), arachidonic acid (AA), total polyunsaturated FAs (PUFA), and total omega-6 PUFA increased from age group < 2 years; alpha-linolenic acid, total omega-3 PUFA, oleic acid, and total monounsaturated FAs decreased. Adherence to the MedDiet was at low-medium level in 91.7% of the subjects. In the age group 2- < 10 yrs., the degree of adherence correlated positively with total MUFA and PUFA balance, negatively with total PUFA, total n6-PUFA, AA/DHA, AA/EPA, and n6/n3. Age did not influence the correlations as to PUFA balance and AA/EPA. CONCLUSIONS: Increased FA proportions with age were seen in the n6-series of PUFA. The n3-FA species decreased or were stable. The vast majority of the subjects with dietary data, 92%, obtained a KIDMED score indicative of low-medium adherence to the MedDiet. The score correlated negatively with various n6-species, i.e. the MedDiet suppressed circulating n6-PUFA. Whole blood may be used to investigate FAs and MedDiet adherence correlations which may be applied in the study of health issues in childhood.

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.

Semi-closed-circuit vacuum-assisted MiniPCNL system in pediatric patients.

Surgical treatment of kidney stones has changed over the years. The use of Mini Percutaneous Nephrolithotomy (MiniPCNL) instrumentation is associated with a reduction of major complications but it lengthens the operative time (OT). This limit may be overcome by a semi-closed-circuit vacuum-assisted MiniPCNL system, characterized by a continuous inflow and a suction-controlled outflow. We present our initial experience in pediatric patients who underwent PCNL using a 12 Fr nephroscope and a 16-Fr-large nephrostomy sheath, equipped with a lateral arm connected to suction. We used Holmium laser lithotripsy and performed lapaxy by drawing back slowly the nephroscope inside the sheath until the opening of the lateral aspiration arm, without using other devices. The stone-free rate (SFR) was assessed at 4 weeks. We included 12 procedures in 8 patients with a median age of 119 months and a median weight of 27 Kg. The median OT was 108 min and the SFR was 80%. No intra-operative complications occurred, while minor post-operative complications occurred after 4/12 procedures. These preliminary data in pediatric population showed the employed system is a safe and effective approach to treat complex kidney stones with a satisfactory SFR and a low OT.

Substitution Phalloplasty in Patients With Bladder Exstrophy-Epispadias Complex: A Systematic Review of Techniques, Complications and Outcomes.

BACKGROUND: Males born with bladder exstrophy-epispadias complex generally have a shorter phallus, split corpora with dorsal curvature, and a scarred and flattened glans, so substitution phalloplasty is often required. AIM: The aim of this study was to review the techniques, complications, and outcomes of substitution phalloplasty in bladder exstrophy-epispadias complex patients to determine the ideal surgical procedure and gauge the risks and benefits for the patient. METHODS: A systematic review of the literature was performed using PubMed/MEDLINE and the Cochrane Library with the following terms: ("phalloplasty"); (("epispadias") OR ("bladder exstrophy") OR ("cloacal exstrophy")). We included only full-text articles reporting data about techniques and outcomes of substitution phalloplasty in patients with bladder exstrophy-epispadias complex. OUTCOMES: To determine whether patients with bladder exstrophy-epispadias complex might benefit from substitution phalloplasty. RESULTS: We selected 7 studies involving 47 patients. All the studies were characterized by a low level of evidence and a heterogeneous approach during treatment and outcome assessment. The free radial forearm flap was the most commonly performed technique (89%) with an overall complication rate of 15%. Urethroplasty was performed in 22 of 47 (47%) patients, and in most cases (20/22) a "tube-within-the-tube" technique was performed simultaneously with the phalloplasty (20/47). Urethroplasty complications were recorded in 12 of 22 (54%) patients with 6 fistulae and 6 stenoses. A penile prosthesis was implanted in 32 of 47 (68%) patients and complications occurred in 8 of 32 (25%) patients with 6 erosion. Aesthetic, sexual, and psychological outcomes were satisfactory, but none of the studies used validated instruments for the final assessment. CLINICAL IMPLICATIONS: It was not possible to formulate any recommendations based on a high level of evidence regarding substitution phalloplasty in patients with bladder exstrophy-epispadias complex. STRENGTH & LIMITATION: To our knowledge, this is the first review to address bladder exstrophy-epispadias complex patients only. The limitations are mainly represented by the small number of cases because of the rarity of this disease and by the fact that no studies used validated instruments. CONCLUSION: Substitution phalloplasty in patients with bladder exstrophy-epispadias complex can achieve good functional, aesthetic, psychological, and sexual outcomes. It requires multiple procedures and carries a high complication rate. Multicentric studies including the assessment of patients by means of a validated questionnaire which investigates both sexual function and psychosexual satisfaction are required. Berrettini A, Sampogna G, Gnech M, et al. Substitution Phalloplasty in Patients With Bladder Exstrophy-Epispadias Complex: A Systematic Review of Techniques, Complications, and Outcomes. J Sex Med 2021;18:400-409.

Redo Laparoscopic Pyeloplasty in Infants and Children: Feasible and Effective.

Purpose: To determine the feasibility and effectiveness of redo laparoscopic pyeloplasty among patients with failed previous pyeloplasty, specifically examining rates of success and complications. Materials and Methods: We retrospectively reviewed the charts of all patients, who underwent redo laparoscopic pyeloplasty from 2006 to 2017. This included patients who underwent primary pyeloplasty at our institution and those referred for failures. Analysis included demographics, operative time, complications, length of hospital stay, complications, and success. Success was defined as improvement of symptoms and hydronephrosis and/or improvement in drainage demonstrated by diuretic renogram, especially in those with persistent hydronephrosis. Descriptive statistics are presented. Results: We identified 22 patients who underwent redo laparoscopic pyeloplasty. All had Anderson-Hynes technique except two cases in which ureterocalicostomy was performed. Median (IQR) follow-up was 29 (2-120) months, median time between primary pyeloplasty and redo laparoscopic pyeloplasty was 12 (7-49) months. The median operative time was 200 (50-250) min, and median length of hospital stay was 3 (2-10) days. The procedure was feasible in all cases without conversion. During follow-up, all but two patients demonstrated an improvement in the symptoms and the degree of hydronephrosis. Ninety-one percent of patients experienced success and no major complications were noted. Conclusions: Redo laparoscopic pyeloplasty is feasible and effective with a high success rate and low complication rate.

Persistent Abnormalities of Fatty Acids Profile in Children With Idiopathic Nephrotic Syndrome in Stable Remission.

Steroid-sensitive nephrotic syndrome is an immunological disorder mediated by still poorly defined circulating factor(s) that target the podocyte and damage the filtration barrier. Fatty acids (FA) have several biological roles and, in particular, are strictly involved in cell to cell communication, inflammatory processes and regulation of lymphocyte pools. Studies of FAs during INS have been mainly focused on biochemical changes during the phase of proteinuria; while no information is available about FA profile in patients with idiopathic nephrotic syndrome (INS) on stable remission. Aim of this study is to assess differences in blood FA profile between pediatric patients with INS during the phase of stable remission. Blood fatty acid profile of 47 pediatric patients on stable remission and 47 matched healthy controls were evaluated with gas chromatography. Patients with INS on stable remission had significantly higher levels of PUFA and omega-6 than controls (40.17 vs. 37.91% and 36.95 vs. 34.79%), lower levels of SFA and MUFA. Considering the single fatty acids, levels of omega-6 18:2n6 linoleic acid and omega-6 20:4n6 arachidonic acid were significantly higher in patients with INS than in controls (23.01 vs. 21.55%, p-value 0.003 and 10.37 vs. 9.65%, p-value 0.01). Moreover, patients with INS showed lower levels of SFA 14:0 (0.74 vs. 0.92%) and 18:0 (10.74 vs. 11.74%) and MUFA 18:1n9 oleic acid (18.50 vs. 19.83%). To the best of our knowledge this is the first study assessing FAs profile in children with INS in stable remission. In a population of 47 patients, we were able to demonstrate a higher blood level of linoleic and arachidonic acid, and consequently of omega-6 and PUFA, compared to controls. Persistently higher than normal levels of either linoleic or arachidonic acid, could be viewed as candidate biomarker for a state of risk of relapse in children with idiopathic nephrotic syndrome.