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Background and Clinical Significance: VACTERL association is a rare spectrum of congenital malformations that may involve the genitourinary system. We describe a challenging case of hypotonic, hyporeflexic, large-capacity bladder with bilateral obstructive megaureter in a boy with VACTERL syndrome, highlighting diagnostic and therapeutic challenges. Case Presentation: A 16-year-old boy with VACTERL syndrome, previously operated for esophageal atresia, Fallot’s tetralogy, Y-type urethral duplication, and bilateral vesicoureteral reflux, presented with breakthrough urinary tract infections, orchiepididymitis, and flank pain. Investigations revealed an enlarged bladder capacity (1000 mL), detrusor underactivity, high post-void residual volume, and bilateral hydronephrosis with megaureter. Obstruction of the bladder neck and neurological causes were excluded. After multidisciplinary discussion, bilateral ureteral reimplantation and limited reductive cystoplasty were performed. Histology revealed granulomatous foreign-body reaction due to previous bulking agent injection. Postoperative course was uneventful. At the three-year follow-up, the patient is asymptomatic with normal voiding and preserved renal function. Conclusions: This case illustrates the diagnostic and therapeutic challenges of managing late urological complications in a VACTERL patient with pre-existing urinary anomalies. The overlap of congenital and iatrogenic factors made the diagnostic pathway complex, requiring careful exclusion of neurogenic and mechanical causes. A tailored surgical strategy restored bladder function and preserved renal outcome.

BackgroundVery limited informations are currently available about the best approach to perform retroperitoneoscopic surgery. This multicentric international study aimed to compare the outcome of lateral versus prone approach for retroperitoneoscopic partial nephrectomy (RPN) in children.MethodsThe records of 164 patients underwent RPN in 7 international centers of pediatric surgery over the last 5 years were retrospectively reviewed. Sixty-one patients (42 girls and 19 boys, average age 3.8 years) were operated using lateral approach (G1), whereas 103 patients (66 girls and 37 boys, average age 3.0 years) underwent prone RPN (G2). The two groups were compared in regard to operative time, postoperative outcome, postoperative complications, and re-operations.ResultsThe average operative time was significantly shorter in G2 (99 min) compared to G1 (160 min) (p = 0.001). Only 2 lateral RPN required conversion to open surgery. There was no significant difference between the two groups as for intraoperative complications (G1:2/61, 3.3%; G2:6/103, 5.8%; p = 0.48), postoperative complications (G1:9/61, 14.7%; G2:17/103, 16.5%; p = 0.80), and re-operations (G1:2/61, 3.3%; G2:4/103, 3.8%; p = 0.85). Regarding postoperative complications, the incidence of symptomatic residual distal ureteric stumps (RDUS) was significantly higher in G2 (7/103, 6.8%) compared to G1 (1/61, 1.6%) (p = 0.001). Most re-operations (4/6, 66.6%) were performed to remove a RDUS .ConclusionsBoth lateral and prone approach are feasible and reasonably safe to perform RPN in children but the superiority of one approach over another is not still confirmed. Although prone technique resulted faster compared to lateral approach, the choice of the technique remains dependent on the surgeon’s personal preference and experience. Our results would suggest that the lateral approach should be preferred to the prone technique when a longer ureterectomy is required, for example in cases of vesico-ureteral reflux into the affected kidney moiety, in order to avoid to leave a long ureteric stump that could become symptomatic and require a re-intervention.

Background/Objectives: The Mitrofanoff appendicovesicostomy (MAV) is the gold standard for creating a continent catheterizable channel in children unable to perform clean intermittent catheterization (CIC) through the native urethra. Minimally invasive surgery has progressively replaced open techniques in pediatric urology, offering improved recovery and favorable cosmetic outcomes, and robotic-assisted Mitrofanoff has gained popularity in recent years. However, the high costs and limited availability of robotic systems create disparities in access to pediatric urologic reconstruction, particularly in low- and middle-income countries. In this context, the laparoscopic Mitrofanoff (MAV-L) and the laparoscopic-assisted Mitrofanoff (MAV-LA) represent practical, cost-effective alternatives, valuable in institutions without robotic platforms or in resource-limited settings. Recent evidence demonstrates that advanced laparoscopic approaches remain feasible even for complex urological procedures, supporting their continued relevance in the robotic era. Methods: We conducted a retrospective case series including seven male children (aged 9–12 years) who underwent MAV between 2018 and 2023. Peri-operative data included demographics, operative time, length of hospitalization, and complications. Functional and aesthetic outcomes were assessed during long-term follow-up. Statistical analysis accounted for the small sample size by using non-parametric tests where appropriate. Results: Three patients (43%) underwent MAV-L and four (57%) MAV-LA. Mean operative time appeared longer in MAV-L (273.3 ± 20.5 min) than in MAV-LA (203.8 ± 24.3 min; exploratory p = 0.019). Hospital stay was 9 ± 0.8 days vs. 7.5 ± 0.5 days (p = 0.026). During follow-up (43.3 ± 10.9 vs. 26.3 ± 5.4 months; p = 0.034), two complications occurred, both in the MAV-L group (stomal stenosis and channel leakage). All patients reported excellent continence, ease of catheterization, and high cosmetic satisfaction. Conclusions: Both laparoscopic and laparoscopic-assisted Mitrofanoff techniques are safe, feasible, and effective in children. Favorable cosmetic satisfaction was reported in the fully laparoscopic subgroup, based on subjective assessment. Importantly, these laparoscopic techniques are sustainable alternatives to robotic surgery, offering accessibility and high-quality reconstructive care even in centers with limited financial and technological resources.

Background The coronavirus disease 2019 (COVID-19) is currently rare in children and they seem to have a milder disease course and better prognosis than adults. However, SARS-Cov-2 pandemic has indirectly caused problems in pediatric medical assistance. In view of this we wanted to draw a picture of what happened during health emergency and analyze future prospects for restarting. Methods We involved the Italian pediatric scientific societies institutionally collected in the Italian Federation of Associations and Scientific Societies of the Pediatric Area (FIARPED); We sent a questionnaire to all scientific societies about the pediatric care activity during the COVID-19 emergency and future perspectives for the phase of post-containment. Results The analysis of the questionnaires showed significant decrease of:admission, outpatient visits and specialist consultancy activities during the COVID-19 emergency, primarily linked to the fear of infection. Instead it was increased the serious degree of diseases admitted. Most of scientific societies maintained the relationship with chronic patients through some form of telemedicine, reporting a strong positive opinion about this modality. Finally showed the need to give life a new approach for hospitalizations and outpatient visits through a greater use of telemedicine, educational programs on families and a more decisive role of family pediatricians. Conclusions Our study highlighted many aspects that can be improved in pediatric care. We think that It will be necessary a new shared strategy to improve the management and continuity of care for pediatric patients, primarily developing a network of collaboration between families, family pediatrician and hospitals and by enhancing the use of new methods of telecommunications .

In the last 20 years, endoscopic injection (EI) has affirmed as a valid alternative to open surgery for management of pediatric vesicoureteral reflux (VUR). This study aimed to investigate and discuss some debated aspects such as indications, bulking agents and comparison, techniques of injection and comparison, predictive factors of success, use in specific situations. EI is minimally invasive, well accepted by patients and families, with short learning curve and low-morbidity profile. It provides reflux resolution rates approaching those of open reimplantation, ranging from 69 to 100%. Obviously, the success rate may be influenced by several factors. Recently, it is adopted as first-line therapy also in high grade reflux or complex anatomy such as duplex, bladder diverticula, ectopic ureters. The two most used materials for injection are Deflux and Vantris. The first is absorbable, easier to inject, has lower risk of obstruction, but can lose efficacy over time. The second is non-absorbable, more difficult to inject, has higher risk of obstruction, but it is potentially more durable. The two main techniques are STING and HIT. To date, the ideal material and technique of injection has not yet clearly established, but the choice remains dependent on surgeon’s preference and experience.

Background Minimal access adrenal surgery (MAAS) for adrenal pathologies is the standard for many pediatric surgical centers. However, the literature offers few reports and minimal evidence from small case series. The aim of this study was to evaluate the outcomes of pediatric MAAS through a multi-center data analysis. Method Pediatric patients who underwent MAAS between January 2002 and December 2013 were retrospectively included. Data analysis was conducted using Spss software (Welch's t-test, X-square, Fisher tests, multiple regression model). Results Six European centers participated, 68 patients were included with mean age of 5.2 years (2 months–16 years). Lesion volume was of 18.1 cc (0.78–145.6), with a mean diameter of 2.8 cm (1.1–6.5). Localization was 50% left-sided masses, 45.6% right-side masses, and 4.4% bilateral. Histological examination revealed 36 neuroblastomas, 15 adenomas, nine pheochromocytomas, three ganglioneuromas, two ganglioneuroblastomas, one bilateral hyperplasia, one adrenocortical carcinoma, an alveolar sarcoma, and a calcification. Surgical access was transperitoneal in 63 (92.6%) and retroperitoneal in 5 (7.4%). Mean operative time was 170 ± 87 min (285 ± 30 min for bilateral lesions). Mean hospital stay was 4.2 ± 2.5 days. Complications included blood loss requiring transfusion in five patients (7.4%) and a diaphragmatic tear. Infiltration of surrounding structures correlated with intraoperative complication rate ( p  = 0.027) and operative time ( p  < 0.01). No mass rupture, conversion, or post-operative complications were observed. Median follow-up was 52 months (1–161). Two recurrences occurred in patients with pheochromocytoma. Age, weight, symptoms, characteristics at imaging, chemistry, volume, or histology, did not influence operative time, hospital stays, or complication rate. Conclusions Pediatric MAAS was safe adopted for masses up to 145.6 cc, with a very low rate of complication. Conversion to open is not necessary even in the presence of infiltrations. MAAS should represent the first-line treatment for selected cases in centers experienced in laparoscopy.

Purpose A preliminary experience on in vivo bladder wall regeneration in a subset of patients born with exstrophy–epispadias complex is reported. The objective was to improve bladder capacity and compliance without bowel augmentation. Methods Five patients (3 males, 2 females), mean age 10.4 years, presenting poor bladder capacity and compliance after complete exstrophy repair, underwent bladder augmentation using small intestinal submucosa (SIS) scaffold. Ultrasonography, cystoscopy with cystogram, assessment of bladder volume and compliance and bladder biopsy were performed before surgery (T0), at 6 (T1) and 18 months (T2) follow-up. Histology was compared with normal bladder specimens. Wilcoxon test was adopted for statistics. Results Bladder capacity and compliance resulted increased (+30%) at T1 ( p  < 0.05) and remained stable at T2, despite dry intervals did not changed significantly. Bladder biopsy at T1 showed no evidence of SIS, but normal transitional mucosa and sero-muscular layer containing smooth muscle fascicles, small nerve trunks and vessels within abundant type-3 collagen. Muscle/collagen ratio was decreased compared with controls at T1 and T2 ( p  < 0.05). No kidney damage, bladder diverticula, or stones were observed at 3 years follow-up. Conclusions Bladder regeneration was feasible in these patients, but bladder capacity and compliance was poorly increased to obtain significant clinical benefit. Histology showed poor muscle components. The acellular matrix grafting failed to provide long-term effective results in terms of continence achievement.

Posterior urethral valves represent the most common cause of bladder outlet obstruction in infancy that impairs renal and bladder function. Long-term outcome of patients with previous PUV is evaluated. Patients over 18 years of age, treated from 1982 to 1995 before the age of 3 years were considered. Previous surgery, renal function, bladder activity, urinary incontinence, and fertility/sexual activity were evaluated. Clinical interview, creatinine clearance, uroflowmetry with ultrasound post-void urine residue, and self-administered questionnaire were recorded. Out of 45 identified records, 24 patients (53.3%) accepted to be enrolled (age 18–34 years, mean 23 years). The mean follow-up was 19.5 years (16–30 years). Out of the 21 excluded patients, 20 did not reply to the clinical interview and 1 died at age of 6 years. All the 24 patients had early endoscopic section of PUV; nine also received transient ureterocutaneostomy or vesicostomy. Ureteroneocystostomy was performed in five patients and ureterocystoplasty with unilateral nephrectomy in two. At follow-up chronic renal failure was detected in 13 patients (54.1%) and 9 (37.5%) had arterial hypertension. End-stage renal disease developed in five patients (20.8%): three had successful renal transplantation and two were in dialysis. Lower urinary tract symptoms were present in seven patients (29.1%). No significant fertility deficit and sexual dysfunction were observed in 23 patients, while 1 patient was azoospermic. No paternity was reported so far. Long-term outcome of patients with previously treated PUV is mandatory. Kidney, bladder, and sexual functions should be monitored till adulthood to verify any modified behaviour.

There is evidence that tissue-specific stem cells reside in certain adult tissues. Their specific properties remain elusive, because they are rare in parent tissues and heterogeneous; furthermore, technical difficulties have been encountered in their identification and the characterization of their progeny. The aim of this study was to isolate stem/progenitor cells from the human bladder. We have devised a method for isolating stem/progenitor cells from the human bladder. This is based on the enzymatic digestion of fresh surgical bladder specimens, followed by culture of cells in the presence of EGF and bFGF. We also used markers that identified and finally characterized these cells. Spheroids with self-replicative potential were obtained from all bladder specimens. The isolated population contained a subset of CD34+ CD45- cells. These spheroids represent a predominant functional type of stem/progenitor cells within the human bladder. This envisage their potential use for the treatment of animal models in pediatric surgery.

Purpose To compare the outcome of laparoscopic and retroperitoneoscopic approach for partial nephrectomy in infants and children with duplex kidneys. Methods Data of 102 patients underwent partial nephrectomy in a 5-year period using MIS procedures were analyzed. Fifty-two children underwent laparoscopic partial nephrectomy (LPN), and 50 children underwent retroperitoneoscopic partial nephrectomy (RPN). Median age at surgery was 4.2 years. Statistical analysis was performed using χ 2 test and Student’s t test. Results The overall complications rate was significantly higher after RPN (15/50, 30 %) than after LPN (10/52, 19 %) [ χ 2  = 0.05]. In LPN group, complications [4 urinomas, 2 symptomatic refluxing distal ureteral stumps (RDUS) and 4 urinary leakages] were conservatively managed. In RPN group, complications (6 urinomas, 8 RDUS, 1 opening of remaining calyxes) required a re-operation in 2 patients. In both groups no conversion to open surgery was reported. Operative time (LPN:166.2 min vs RPN: 255 min; p  < 0.001) and hospitalization (LPN: 3.5 days vs RPN: 4.1 days; p  < 0.001) were significantly shorter in LPN group. No postoperative loss of renal function was reported in both groups. Conclusions Our results demonstrate that RPN remains a technically demanding procedure with a significantly higher complications and re-operation rate compared to LPN. In addition, length of surgery and hospitalization were significantly shorter after LPN compared to RPN. LPN seems to be a faster, safer and technically easier procedure to perform in children compared to RPN due to a larger operative space and the possibility to perform a complete ureterectomy in refluxing systems.