Obstructive or non-obstructive megacystis: a prenatal dilemma.

Introduction: Diagnosis of prenatal megacystis has a significant impact on the pregnancy, as it can have severe adverse effects on fetal and neonatal survival and renal and pulmonary function. The study aims to investigate the natural history of fetal megacystis, to try to differentiate in utero congenital lower urinary tract obstruction (LUTO) from non-obstructive megacystis, and, possibly, to predict postnatal outcome. Materials and methods: A retrospective single-center observational study was conducted from July 2015 to November 2023. The inclusion criteria were a longitudinal bladder diameter (LBD) >7 mm in the first trimester or an overdistended/thickened-walled bladder failing to empty in the second and third trimesters. Close ultrasound follow-up, multidisciplinary prenatal counseling, and invasive and non-invasive genetic tests were offered. Informed consent for fetal autopsy was obtained in cases of termination of pregnancy or intrauterine fetal demise (IUFD). Following birth, neonates were followed up at the same center. Patients were stratified based on diagnosis: LUTO (G1), urogenital anomalies other than LUTO ("non-LUTO") (G2), and normal urinary tract (G3). Results: This study included 27 fetuses, of whom 26 were males. Megacystis was diagnosed during the second and third trimesters in 92% of the fetuses. Of the 27 fetuses, 3 (11.1%) underwent an abortion, and 1 had IUFD. Twenty-three newborns were live births (85%) at a mean gestational age (GA) of 34 ± 2 weeks. Two patients (neonates) died postnatally due to severe associated malformations. Several prenatal parameters were evaluated to differentiate patients with LUTO from those with non-LUTO, including the severity of upper tract dilatation, keyhole sign, oligohydramnios, LBD, and GA at diagnosis. However, none proved predictive of the postnatal diagnosis. Similarly, none of the prenatal parameters evaluated were predictive of postnatal renal function. Discussion: The diagnosis of megacystis in the second and third trimesters was associated with live births in up to 85% of cases, with LUTO identified as the main cause of fetal megacystis. This potentially more favorable outcome, compared to the majority reported in literature, should be taken into account in prenatal counseling. Megacystis is an often misinterpreted antennal sign that may hide a wide range of diagnoses with different prognoses, beyond an increased risk of adverse renal and respiratory outcomes.

The digital era of pediatric urological clinical care: telemedicine for management of lower urinary tract symptoms and nocturnal enuresis - a comprehensive tertiary center insight.

BACKGROUND: Lower urinary tract symptoms (LUTS) and nocturnal enuresis (NE) are complex conditions requiring a long-term follow-up. Telemedicine is an emerging technological tool in the surgical field, and its availability exponentially grew during the COVID-19 pandemic, expanding its application fields, optimizing technical aspects, reducing costs, and ensuring high-quality standards. This work describes our experience with telemedicine in a Division of Pediatric Urology for the follow-up of enuresis and LUTS. METHODS: A retrospective analysis of our telemedicine preliminary experience was conducted at Regina Margherita Children's Hospital in Turin, Italy. This study included all the patients aged <18 years who were monitored for enuresis and LUTS through telemedicine between September 1, 2021 and July 31, 2023. Clinical data and outcomes were analyzed, and patients and families were asked to voluntary fill an evaluation questionnaire on their satisfaction. Additionally, we focused on the post COVID-19 period, between September 2022 and July 2023, analyzing the data of two different patients' populations: the first one (G1) of patients choosing telemedicine outpatients visits (TOVs) and the second one (G2) of those choosing a face-to-face visit. RESULTS: One hundred five patients were enrolled. One hundred sixty-two TOVs were performed. The median age at first visit was 9.7±0.66 years (range 7-16 years). Diagnosis were: 77/105 (67%) NE and 28/105 (33%) LUTS. The average referred distance between the patients' residence and the hospital was 46.35±129.37 km (range 2-1300 kilometers) and the time taken to overcome it was 44.21±77.29 minutes (range 10-780 minutes). In 64/105 cases (61%) the follow-up was interrupted for total healing or symptoms resumption. Only two cases (2%) required the conversion to an in-person ambulatory consult, due to a social-linguistic barrier. 146/162 families (90%) filled the survey questionnaire at the end of each TOV, reporting in 94% of cases a high satisfaction level. In the comparative statistical analysis of the two patient groups, G1 (52 telemedicine office visits, [TOVs]) vs. G2 (25 face-to-face visits), the average referred distance was 17.78±7.98 km (range: 5-35 km) for G1, contrasting with 7.04±3.35 km (range: 2.5-14 km) for G2 (P=0.00001). Additionally, the waiting time before the visit was 3.96±2.90 minutes (range: 0-10 minutes) for G1, in contrast to 26.52±11.22 minutes (range: 5-44 minutes) for G2 (P=0.00001). Furthermore, a higher compliance with behavioral or pharmacological indications was observed in the G1 group, exhibiting lower adherence in only 12 out of 52 cases compared to 14 out of 25 cases in G2 (P=0.0091). CONCLUSIONS: Telemedicine is a proper solution and an effective tool to manage the therapeutic follow-up of NE and LUTS, ensuring suitable quality standards and reducing social costs, such as the loss of working days and transport costs. The implementation and complete integration of its use into the healthcare system should be the goals to pursue in order to take full advantage of all its potentials.

JJ stent dislodgement in the distal ureter: how to recover it in preschool children?

Double-J ureteral stents are usually placed after various urological procedures. The dislodgement of their distal ringlet is a rare complication, whose retrieval is arduous in younger children, due to the small ureteral caliber. We propose our innovative endoscopic approach to recover the dislodged JJ stent. Under 8-9.8 Ch cystoscopy, the ureteral meatus is gently cannulated with a 00.18″ guidewire, then a balloon catheter Passeo 18 3-4 mm (Biotronik, Lake Oswego, OR, USA) is coaxially inserted. A pneumatic dilatation of the vesical-ureteral junction is performed up to 8 atmospheres for 5 minutes under direct vision. Consequently, the ureteral meatus allows the cystoscope passage, and the JJ-stent can be recovered thanks to endoscopic grasping forceps. A mono-J stent is then left in place for 24 hours. Four patients aged 8 months - 4 years have been successfully treated with this approach after that JJ migration was found intraoperatively or during ultrasonography. No intra- or postoperative complications occurred. Postoperative hospital stay was prolonged for one day. During 29.5 medium follow-up no clinical or ultrasonographic signs of vesical-ureteral reflux ensued. Our cystoscopic approach is effective and safe to ensure a prompt endoscopic JJ retrieval without changing neither surgical approach nor the anesthesiological support. We believe that all the pediatric urology centers should know the procedure and have small size balloon catheter available.

Retrograde Intrarenal Surgery for Renal Stones: Is It a Safe and Effective Option in Preschool Children?

INTRODUCTION: Few studies in the literature describe the Retrograde Intra-Renal Surgery (RIRS) outcome in preschool children. We evaluated the feasibility, stone-free rate and complications of RIRS in preschool children at two European tertiary care centres of Pediatric Urology. MATERIAL AND METHODS: The retrospective study includes all children undergone RIRS for stones <25 mm from 2017 to 2022. Patients were divided into Group 1 <5 years (G1) and Group 2 >5 years (G2). Semirigid ureterorenoscope 4.5-6.5 Ch and a 7.5 Fr flexible ureteroscope with a 9.5/11 Ch ureteral access sheath (UAS) were used. Stone-free rate (SFR) was evaluated at 3 months. Fischer/Chi-square test for qualitative data and Mann-Whitney for quantitative data were used for statistical analysis. RESULTS: 63 patients underwent RIRS, 19 G1-patients, median age 3.55 ± 1.06 years (range 1.5-5 years), and 44 G2-patients, median age 11.25 ± 2.95 (range 6-17 years) (p < 0.00001). Intraoperative complications occurred in 1 case in G1(5%) and 3 in G2(7%) (p = 1): two minor ureteric injuries in G2 were treated by a prolonged JJ-stent. Postoperative fever was reported in 3 cases in G1 (16%) and 4 in G2(9%) (p = 0.42), while post-operative hematuria in 4 G1-patients (21%) and in 7 G2-patients (16%) (p = 0.72). SFR was 84.2% in G1 and 88.6% in G2. At an average follow-up of 15.05 ± 4.83 months in G1 and 19.95 ± 10.36 months in G2, reintervention for residual stones was necessary in 3 cases in G1(16%) and in 6 cases in G2(14%) (p = 1). CONCLUSIONS: In a European country with low-volume pediatric stone centers, RIRS is a promising therapeutic option in young children as it offers acceptable stone-free rate and a low incidence of high-grade complications. LEVEL OF EVIDENCE: III.

Prechemotherapy Transperitoneal Robotic-Assisted Partial Nephrectomy (RAPN) for a Wilms Tumor: Surgical and Oncological Outcomes in a Four-Year-Old Patient.

BACKGROUND: Wilms tumor (WT) is the most frequent renal tumor in children. The SIOP-UMBRELLA Guidelines allow for nephron-sparing surgery (NSS) in syndromic patients, as well as in cases of small (<300 mL) non-syndromic unilateral WTs, without lymph node involvement, and with a substantial expected remnant renal function, following neoadjuvant chemotherapy. We present a case of prechemotherapy transperitoneal robot-assisted partial nephrectomy (RAPN) for a unilateral, non-syndromic Wilms tumor. METHODS: A four-year-old child presented with a solid mass measuring 3.6 cm in diameter involving the upper right renal pole, incidentally detected during an abdominal echotomography. CT scan and abdominal MRI revealed no local infiltration or lymph node involvement, suggesting that the exophytic mass could be easily resected via an NSS robotic approach. Preoperative imaging did not strongly suggest WT. A virtual 3D reconstruction of the tumor was performed. RESULTS: After the oncologic board approval, a robot-assisted partial nephrectomy with an intraperitoneal approach was performed. Histopathological analysis confirmed the diagnosis of WT. The patient subsequently received 10 doses of vincristine as adjuvant chemotherapy. A 28-month follow-up showed no tumor recurrence. CONCLUSIONS: Intraperitoneal RAPN may be an option for selected WT and warrants consideration as a challenging but advantageous approach.

Intravesical Agents in the Treatment of Bladder Clots in Children.

Bladder blood clots represent an infrequent urinary condition in children. They usually result from hematuria with many underlying causes, such as urinary tract infections and urethral/bladder traumas. Treatment options for clot removal include trans-urethral or suprapubic bladder irrigation and, if unsuccessful, endoscopic management under general anesthesia with a resectoscope. In younger male children, however, the repeated passage of a trans-urethral resectoscope may be challenging and traumatic, due to the small lumen diameter. Eventually, an open surgical approach can be required in many patients. Few anecdotal non-surgical approaches have been proposed for the management of bladder blood clots in children. This review aims to summarize the conservative techniques described in the literature with the instillation of intravesical agents, analyzing the different strategies and their advantages.

Testicular torsion during the COVID-19 pandemic: Results of a multicenter study in northern Italy.

INTRODUCTION: The literature reported an increased avoidance of the Emergency Department (ED) during COrona VIrus Disease 19 (COVID-19) pandemic, causing a subsequent increase of morbidity and mortality for acute conditions. Testicular torsion is a surgical emergency, which can lead to the loss of the affected testicle if a delayed treatment occurs. As testicular loss is time-related, outcome was hypothesized to be negatively affected by the pandemic. OBJECTIVE: The aim is to investigate whether presentation, treatment and outcomes of children with testicular torsion were delayed during COVID-19. STUDY DESIGN: Medical records of pediatric patients operated for testicular torsion of six Paediatric Surgical Units in Northern Italy between January 2019 and December 2020 were retrospectively reviewed. Patients were divided as for ones treated during (dC) or before the pandemic (pC). To reflect possible seasonality, related to lockdown restrictions, winter and summer calendar blocks were also analysed. For all cohorts, demographic data, pre-operative evaluation, operative notes and post-operative outcomes were reviewed. Primary outcomes were referral time, time from diagnosis to surgery and ischemic time, while secondary outcomes were orchiectomy and atrophy rates. Statistic was conducted as appropriate. RESULTS: A total of 188 patients with acute testicular torsion were included in the study period, 89 in the pre-COVID-19 (pC) period and 99 during COVID-19 (dC). Time from symptom onset to the access to the Emergency Department (T1) was not different among the two populations (pC: 5,5 h, dC: 6 h, p 0.374), and similarly time from diagnosis to surgery (pC: 2,5 h, dC: 2,5 h, p 0.970) and ischemic time (pC: 8,2 h, dC: 10 h, p 0.655). T1 was <6 h in 46/99 patients (46%) pC and 45/89 patients (51%) dC (p = 0.88, Fisher's exact test). Subgroup analysis accounting for different lockdown measures, confirm the absence of any difference. Orchiectomies rate was 23% (23/99) dC and 21% (19/89) pC (p = 0.861, Fisher's exact test) and rate of post-operative atrophy was 9% dC (7/76) and 14% pC (10/70), p = 0,44, Fisher's exact test. DISCUSSION: Despite worldwide pediatric ED accesses reduction, we reported that neither ischemic time nor the long-term outcomes in children with testicular torsion increased during the COVID-19 pandemic. In the available literature, few studies investigated the topic and are controversial on the results. Similarly to our findings, some studies found that timing and orchiectomy rates were not significantly different during the pandemic, while others reported a correlation to pandemic seasonality. Furthermore, in the recent pediatric literature it has been reported a delayed testicular torsion diagnosis due to shame in informing parents. Strengths of this study are the large numerosity, its multicentric design and a long study period. Its main limitation is being retrospective. CONCLUSIONS: We reported our large cohort from one of the most heavily COVID-19-affected regions, finding that referral, intra-hospital protocols and ischemic time in testicular torsion were not increased during to the pandemic, as well as orchiectomy rate and atrophy.

Thoracoscopic excision of asymptomatic antenatally diagnosed mediastinal bronchogenic cysts: A case report.

Bronchogenic Cysts (BCs) are benign congenital malformations commonly located in the mediastinum. In recent years the development of antenatal diagnosis has changed the BCs management allowing an earlier minimally invasive approach. We report a case of an asymptomatic 8-months-old girl with antenatal diagnosis of subcarinal posterior mediastinal BC. Thoracoscopic excision of the cyst was successfully performed. The management of antenatally diagnosed BCs is discussed. Thoracoscopic treatment of BCs is safe and effective with the advantage of a reduced morbidity compared to thoracotomy.

Feasibility of a Standardized Management for Primary Spontaneous Pneumothorax in Children and Adolescents: A Retrospective Multicenter Study and Review of the Literature.

Introduction: The management of primary spontaneous pneumothorax (PSP) in children still remains controversial. The current literature shows a lack of scientific evidence supporting a standardized management. Materials and Methods: A multicenter retrospective study was performed, patients admitted for PSP in five Pediatric Surgical Units over a 9-year period (from 2008 to 2017) were included. Patient data were reviewed for each case. Management and outcomes were statistically analyzed. Results: Overall 159 patients (135 males and 24 females) were included in this study. During the first hospital admission, 62/159 patients (39%) were conservatively managed with oxygen therapy; 95/159 patients (60%) required a chest drain insertion; 2/159 patients (1%) underwent surgery because of a bilateral pneumothorax. Thoracoscopy was performed in 75/159 (47%) patients after the second hospital admission or for persistent air leak during the first access. Postoperatively, 8/75 (11%) patients developed recurrent pneumothorax requiring chest drain insertion in 3/8 (37%) cases or aspiration in 1/8 (13%), whereas 4/8 (50%) patients were conservatively managed. All of the patients but one, were confirmed to be affected by emphysematous-like changes at histology. Conclusions: Despite the limitations of being a retrospective study, we suggest that the early surgical management in children with PSP is feasible and safe and it seems to significantly reduce the recurrence rate. To the best of our knowledge there are no pediatric guidelines for the management of PSP, therefore, we support the need for prospective studies to create the evidence-based pillars for correct and standardized management of this condition.

Circumcision with Glubran(®) 2 in children: experience of Italian Center.

BACKGROUND: Circumcision is one of the most common surgical procedures in the world. Despite it is known its wide prevalence for religious and medical reasons in children, it remains a controversial practice in paediatric age. To date, there is no described the gold standard technique to circumcise paediatric patients. We started to use glue for circumcision about 2 years ago. We designed this prospective study with the aim to compare two surgical techniques, which were used in our hospital to perform circumcision in children. The implication for practice was the understanding if there were differences between these approaches related to patient's and parents benefits to manage this condition and benefits for surgeon and hospital in term of saving money and time. METHODS: This is a randomized, single-blind one-center study. It was conducted at the Department of Paediatric Surgery of Siena. Data were collected between March 2011 and December 2012. Study's population involved all patients who required circumcision. Two randomizes groups: group one which involved patients who underwent circumcision using sutures and group two, which involved patients who underwent circumcision using surgical glue (Glubran(®) 2). Two exclusion criteria were used: the redo-circumcision and the allergy or hyper-sensibility to cyanoacrylate (main component of glue). RESULTS: We report 99 patients who underwent circumcision with Glubran(®) 2 in comparison with a group of children circumcised with sutures (vycril rapide). We measured three outcomes (operating time, postoperative pain and assessment of cosmetic), which, even if not all statistically significant, allowed us to draw any conclusions about the use of glue in circumcision. CONCLUSIONS: Traditional circumcision is performed using a standard sleeve technique with sutures for the approximation of the skin edges. However, since some years a tissue adhesive as N-butyl-2-cyanoacrylate (NBCA) (Glubran(®) 2) is used in many centers to circumcise children. Based on our results we can conclude that, glue (Glubran(®) 2) application is an excellent alternative to circumcision in paediatric age for a faster surgery, less postoperative pain and good early cosmetic.

Pediatric Thoracoscopy and Bronchial Blockers: The Continued Search for the Ideal One-Lung Ventilation.

INTRODUCTION: Thoracoscopic surgical procedures in small infants and children require a growing demand for lung isolation in pediatric anesthesia practice. MATERIALS AND METHODS: Between January 2006 and September 2014, 16 children underwent thoracoscopic procedures that needed one-lung ventilation using a bronchial blocker. RESULTS: The thoracoscopic procedure was performed at a mean age of 99 months (range, 6-186 months) and a mean weight of 33 kg (range, 7-68 kg) without any conversion to open surgery in any case. We were able to achieve one-lung ventilation in all patients. The quality of lung deflation was excellent in all patients except in 1 due to malposition of the bronchial blocker, whereas dislodgement of this device occurred in 2 cases. CONCLUSIONS: The use of a bronchial blocker provided a highly effective means of one-lung ventilation for children undergoing the thoracoscopic approach.

Sternal reconstruction by extracellular matrix: a rare case of phaces syndrome.

Congenital defects of the sternum are rare and due to a failure of midline development and fusion of the sternal bones. Surgical correction of a sternal cleft should be preferred during infancy for functional reasons. Chest wall reconstruction represented a complex problem in the last decades. We report our successful outcome of sternal reconstruction in a rare case of PHACES syndrome, in which the patient was submitted to reconstruction of the sternum and complete closure of the thoracic defect by the employ of an extracellular matrix XCM Biologic tissue matrix. We promote the use of extracellular matrix in surgical reconstruction of chest defects for its maneuverability, plasticity, tolerability and the possibility of growing with the children's chest getting a good compliance and optimal cosmetic results.

Serum levels of inhibin B in adolescents after varicocelelectomy: A long term follow up.

INTRODUCTION: To study the impact on adult's fertility of serum inhibin B levels in adolescent patients with idiopathic varicocele after minimally invasive surgical correction and to compare fluctuation of pituitary-testis hormonal values and testicular volumes. MATERIALS AND METHODS: A case-control study was carried out on a group adolescent patients (n=60) affected by idiopathic left varicocele (group V) and compared with control adolescents (n=40) in the Paediatric Surgery Section of Siena (from June 1993 till September 2013). Inhibin B levels and testicular volume before (T0) and after at 6 and 12 months from surgery (T1 and T2) were evaluated. RESULTS: A positive correlation between testicular growth at T1 and T2 (P<0.001) was found. Linear regression analysis showed a positive correlation between inhibin B levels and testicular volume (expressed as the sum of the right and left values) (P<0.0001). CONCLUSIONS: Inhibin B levels are a valid marker for studying the effects of varicocele on the testicular function and confirm the necessity of early surgical correction for preventing the trophic testicular damage and male infertility.

Thoracoscopic thymectomy in children: our preliminary experience.

INTRODUCTION: The aim of this study is to present a preliminary series of six children affected by an anterior mediastinal mass, resulting in a thymoma. We treated this pediatric population by a video-assisted thoracoscopic thymectomy. PATIENTS AND METHODS: We performed a retrospective study from January 2000 to January 2012 of all children affected by an anterior mediastinal mass, resulting in a thymoma. Data included sex, age at surgery, clinical and radiological features, surgical procedure, follow-up, and complications. RESULTS: Video-assisted thoracoscopic thymectomy was performed in six children (five boys and one girl). Four patients presented with dyspnea and/or thoracic pain treated with analgesic and corticosteroid therapy without any benefits. Two patients were asymptomatic. Preoperatively, exams included radiological imaging, blood, and immunological test. All 6 patients were treated with a thoracoscopic approach; of these patients, 5 were treated with a thoracoscopic right-side thymectomy and 1 with a left-side approach. Patients were treated by a three-trocar technique, and thymectomy was performed using Valleylab (now Covidien) (Boulder, CO) LigaSure™. The mean operative time was 120 minutes. The mean hospital stay was 5 days. In all cases histological findings revealed no malignancy. The mean follow-up was 38 months. DISCUSSION: Thoracoscopic thymectomy facilitated the goal of early thymectomy. The right-side approach is preferred because it gains a good visualization of the venous anatomy for dissection. The left side allows a good extended resection of both the thymus and perithymic fat. Thoracoscopic thymectomy should be consider a valid, less invasive alternative to the most radical open approach.