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.

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.

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 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.

A Case of Hypotonia-Cystinuria Syndrome With Genito-Urinary Malformations and Extrarenal Involvement.

Hypotonia-Cystinuria syndrome (HCS) is a rare disease, caused by a mutation in two contiguous genes (SLC3A1 and PREPL) localized on chromosome 2p21, and it is characterized by both renal involvement with cystine stones and nervous involvement with hypotonia. We here describe a 2 years old child with HCS associated with other clinical features as congenital anomalies of kidney and urinary tract (primary obstructed megaureter, POM), cryptorchidism and cardiac involvement (patent foramen ovale with atrial septum aneurysm). To the best of our knowledge, cryporchidism and POM have never been reported before in patients with HCS. Moreover, a cardiac involvement has been described only in another case of HCS that, interestingly, presents the same genetic abnormalities as our patient. The diagnosis of HCS can be difficult because neurological signs are aspecific and kidney stones are commonly absent during the first months of life. A better understanding of the complete clinical scenario associated with HCS can help clinicians suspect, diagnose and treat HCS earlier with a positive influence on both neurological and renal outcome.

Retrograde intrarenal surgery using ureteral access sheaths is a safe and effective treatment for renal stones in children weighing <20 kg.

INTRODUCTION: Surgical treatment of pediatric kidney stones has changed dramatically in recent years because of the miniaturization of surgical instruments and the availability of intracorporeal lithotripters. The retrograde intrarenal surgery (RIRS) technique is nowadays considered an effective and safe procedure but studies in very young children are lacking and use of a ureteral access sheath (UAS) has also been debated. OBJECTIVE: To assess safety and efficacy of RIRS using UAS in children weighing < 20 kg. MATERIALS AND METHODS: Data from 13 children weighing <20 kg who underwent RIRS for renal stones were collected. A Double-J stent was positioned 14 days before surgery. Demographics, stone location, stone number and composition, stone-free status (SFS) and complications were evaluated. Descriptive statistics were applied to describe the cohort. RESULTS: Thirteen patients, age 3.91 ± 1.8 years (mean ± SD), underwent 16 RIRS. Mean patient weight and stone burden were 14.88 ± 3.81 kg (range 10-20 kg) and 15.5 ± 3.8 mm (median 16 mm), respectively. A UAS was used in 15 out of 16 (93.8%) procedures. SFS was achieved in 81.3% of cases after the first procedure and 100% after auxiliary procedures. Postoperative urinary tract infections with fever (Clavien II) were observed in two (12.5%) patients. Hydrocalyx (Clavien IIIb) was noted in one (6.3%) patient. Patients with stones located in the lower polar calices (p = 0.024) and with mixed composition (p = 0.036) had a greater prevalence of complications than those with calculi of other compositions located in other sites. After a mean follow-up of 22.4 months no cases of ureteral strictures or vesicoureteral reflux were observed (Table). DISCUSSION: The current findings support previous evidence showing safety and efficacy of RIRS with use of the UAS in pre-stented very young children. In our experience RIRS seems to be an effective and safe option with a stone-free rate of 81.3% and only 1 severe complication (Clavien-Dindo grade IIIb) and no long-term complications. This study is, to date, the first to examine the safety and efficacy of RIRS using UAS for the treatment of intrarenal stones in a selected cohort of pre-school patients weighing <20 kg. CONCLUSIONS: RIRS using UAS is an effective and safe procedure for treating kidney stones in pre-school patients weighing <20 kg without complications at both short and long term follow up. Stones of mixed composition located in the lower polar calices are associated with a greater risk of postoperative complications.

Hyperechogenic Bowel: Etiologies, Management, and Outcome according to Gestational Age at Diagnosis in 279 Consecutive Cases in a Single Center.

Introduction The aim of the study was to investigate perinatal outcome of fetuses with hyperechogenic bowel (HB) in relation to gestational age at diagnosis. Materials and Methods This is a retrospective observational study of fetal HB cases from 2002 to 2012. Patients were divided into three groups according to trimester at diagnosis. For each group, data from fetal ultrasound examination, fetal medicine investigations, intrapartum cares, and neonatal outcome were obtained. Results A diagnosis of HB was made in 279 fetuses among them 17 (6%) during the first trimester, 186 (67%) during the second trimester, and 75 (27%) during the third trimester. A significant prevalence of maternal comorbidities was noticed in group 1 (12%: p = 0.02). A chromosomal defect was identified in 13% of the fetuses without difference among the three groups. HB was associated with prenatal infection in 11.5% (n = 32) of the cases, with an equal distribution between groups 2 and 3. Intrauterine growth retardation was noticed in 23% (n = 64) of the cases with a slightly high prevalence in groups 1 (35%). HB was the only ultrasonographic intestinal soft marker in 80% (n = 223) of the fetuses, two-third of them were detected during the first and the second trimesters (p = 0.001). However, HB was associated with bowel dilation in 33% of the cases diagnosed during the third trimester (p = 001). Ultrasonographic extraintestinal anomalies were identified in 30% of the fetuses with a higher prevalence in group 1 (59%). HB resolved spontaneously in 55 (19.7%) cases-without difference among the three groups. In group 1 we recorded a significant prevalence of intrauterine demise (23.5%, p = 0.004). Two hundred twenty-seven (81.3%) pregnancies resulted in live-born neonates; among them gastrointestinal anomalies were noticed in 12.5% with a significant prevalence in group 3 (36%) compared with 6 and 5.4% in groups 1 and 2, respectively (p = 0.001). Extraintestinal anomalies were confirmed in 27% of the cases, whereas postnatal mortality rate was of 7% without differences between the three groups. Conclusion Detection of HB during the first trimester is associated with an increased risk for maternal comorbidities, intrauterine growth retardation, and adverse pregnancy outcome. Moreover, if HB is detected during the second trimester, it is associated with a favorable prognosis. Otherwise, HB detected during the third trimester is associated with a significant risk of gastrointestinal anomaly.

Incidental Diagnosis of Giant Cell Tumor After Urachal Remnant Removal in a Thalassemic Child.

Urachal anomalies are relatively uncommon; they usually present in childhood as urachal cysts, urachal sinuses, diverticula, and patent urachus. The malignant involvement of the urachus, although rare in children, is commonly found in adulthood when adenocarcinoma accounts for the majority of the cases, representing up to 20-30% of all adenocarcinomas of the bladder. Urachal tumors of variable histology have been reported in children and adolescents as single case report. We present the first case of a 4-year-old thalassemic boy, with an incidental finding of giant cell tumor arising in an urachal remnant.

Vacuum assisted closure for the treatment of complex wounds and enterocutaneous fistulas in full term and premature neonates: a case report.

BACKGROUND: The Vacuum Assisted Closure (VAC) system has become an effective treatment for acute and chronic wound defects. Although its use has been reported in wound care of children and premature infants, the management of the device in this population has not been well established. CASE PRESENTATION: We report the satisfactory results in two neonates (one full-term and one preterm) with complex wounds secondary to major abdominal surgery. In the premature baby an enterocutaneous fistula was also present. Complete epithelialization of the wounds was achieved in both patients within a few weeks thus avoiding any further surgical procedure. CASE PRESENTATION: The use of VAC system in neonates is safe and effective in the management of complex wounds and should be considered as a first line treatment in the event of a major dehiscence.