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PurposeTransition in urology is defined by the process that allows an adolescent or a young adult with a congenital or acquired urogenital anomaly to assume increasing responsibility for their own health care and to become the primary decision maker in their care.MethodsA review of the literature regarding transitional care for lifelong urologic congenital anomalies was performed with the aim of reporting expert opinion when data are non-existent. This review focuses on special considerations for adolescents and young adults with spina bifida, bladder exstrophy, anorectal malformations and differences of sexual development.ResultsUrologic goals during the transition from childhood to adulthood continue to include attention to the preservation of renal function and optimization of lower urinary tract function. Additional concerns include care to decrease long-term surgical complications (especially after augmentation cystoplasty), to monitor for malignancy, to prepare for sex activity and fertility, and to help the adult patient in decision making. Transition aims to maximize quality of life and independence by ensuring uninterrupted appropriate care through a multidisciplinary approach which varies by geographical location and healthcare setting. Barriers include patient and family factors as well as provider and system related factors. A dedicated team is an important element of successful transition.

PurposeWe have studied outcome of double-face preputial island flap (DFPIF) technique in severe types of hypospadias: penoscrotal, scrotal and perineal.MethodsWe have used DFPIF in 75 boys at a median age of 1.1 years (1.0–1.5). The meatus was penoscrotal, scrotal or perineal after de-gloving the penis. The inner face of the foreskin was used for urethroplasty and the outer face for ventral skin covering. Modifications were added: proximal anastomosis was protected by a spongioplasty; in case of urethral plate transection, we anastomosed on onlay proximal and distal segments of the flap (onlay-tube-onlay) and the tubularized part was sutured to corpus cavernosa. FU was scheduled at one month then every 3 months for a year then annually. At each consultation, the surgeon filled out a detailed cosmetic and functional sheet including flowmeter.ResultsThirty-four patients had onlay preputial flap repair with urethral plate preservation. Forty-one had the onlay-tube-onlay technique. All children had a curvature, 19 had a significant residual curvature after dissection, corrected by dorsal plication (n = 9) and ventral lengthening (n = 10). Median FU was 4.2 years (2.7–6.5). 36 children (48%) had complications and needed redo surgery: 12 fistulas, 11 diverticula, 7 meatal stenosis, 3 strictures and 2 residual curvatures. All children but three voided within the normal limits for their age.ConclusionDFPIF remains a good option for a one-stage repair of severe hypospadias. After a median of 1.8 procedures, the final success rate was 96%. The healthy well-vascularized ventral skin allows safe redo surgery when needed.

A major complication of feminizing genitoplasty in children is the loss of clitoral sensation with serious impact at adult life. We suggest a new method to evaluate the surgical results during childhood based on the bulbocavernosus or clitoro-perineal reflex (CPR). The afferent pathway of CPR implies the intact sensory receptors on the clitoral glans. Girls with congenital adrenal hyperplasia who were followed-up medically without surgery or who underwent feminizing genitoplasty with or without clitoroplasty were included (2002–2018). All clitoroplasties were standardized reduction clitoroplasty with preservation of neurovascular bundles associated with vaginoplasty and vestibuloplasty. Standardized examinations were prospectively performed including the CPR starting at one year postoperatively. The reflex was triggered by gentle touch of the glans by a cotton swab. Contraction of the perineal muscles was considered positive. Thirty-two children were operated at a median age of 8.6 months (5.8–12.1). Median follow-up (FU) was 3.9 years (1.3–6.4). Twenty-four patients had clitoroplasties: 17 were tested for CPR at one-year FU, and all had a positive test. Eight girls had genitoplasty without clitoral surgery, two of them were tested and were positive. Ten patients were managed without surgery, two of them were tested for the CPR and were positive. The reflex was always triggered easily and repeated at least twice during the FU. The clitoro-perineal reflex is a simple, non-invasive and reproducible test in early childhood and may serve as an early evaluation tool of clitoral innervation after feminizing genitoplasty. These results need to be confirmed at long term and completed at adult life.

This study investigated relationships between neuroblastomas (NBs) imaging phenotypes, tumor genomic profile and patient outcome. This IRB-approved retrospective observational study included 133 NB patients (73 M, 60 F; median age 15 months, range 0-151) treated in a single institution between 1998 and 2012. A consensus review of imaging (CT-scan, MRI) categorized tumors according to both the primarily involved compartment (i.e., neck, chest, abdomen or pelvis) and the sympathetic anatomical structure the tumors rose from (i.e., cervical, paravertebral or periarterial chains, or adrenal gland). Tumor shape, volume and image-defined surgical risk factors (IDRFs) at diagnosis were recorded. Genomic profiles were assessed using array-based comparative genomic hybridization and divided into three groups: \"numerical-only chromosome alterations\" (NCA), \"segmental chromosome alterations\" (SCA) and \"MYCN amplification\" (MNA). Statistical analyses included Kruskal-Wallis, Chi2 and Fisher's exact tests and the Kaplan-Meier method with log-rank tests and Cox model for univariate and multivariate survival analyses. A significant association between the sympathetic structure origin of tumors and genomic profiles was demonstrated. NBs arising from cervical sympathetic chains were all NCA. Paravertebral NBs were NCA or SCA in 75% and 25%, respectively and none were MNA. Periarterial NBs were NCA, SCA or MNA in 33%, 56% and 11%, respectively. Adrenal NBs were NCA, SCA or MNA in 16%, 36% and 48%, respectively. Among MNA NBs, 92% originated from the adrenal gland. The sympathetic anatomical classification was significantly better correlated to overall survival than the compartmental classification (P < .0003). The tumor volume of MNA NBs was significantly higher than NCA or SCA NBs (P < .0001). Patients with initial volume less than 160 mL had significantly better overall survival (P < .009). A \"single mass\" pattern was significantly more frequent in NCA NBs (P = .0003). The number of IDRFs was significantly higher in MNA NBs (P < .0001). Imaging phenotypes of neuroblastomas, including tumor origin along the sympathetic system, correlate with tumor genomic profile and patient outcome.

PurposeTo evaluate the impact of the COVID-19 pandemic-related lockdown on management and outcomes of children with acute appendicitis.MethodsA retrospective cohort study was conducted, including children treated for acute appendicitis (January 20th–May 11th, 2020). The data regarding the severity of appendicitis and outcome were collected and compared for two time periods, before and after the nationwide lockdown (March 17th, 2020).ResultsThe number of cases of acute appendicitis increased by 77% during the lockdown (n = 39 vs. n = 69, p = 0.03). During the lockdown, children treated for appendicitis were older (11.1 vs. 8.9 years, p = 0.003), and were more likely to live more than 5 km away from our institution (77% vs. 52%, p = 0.017). Less children had previously consulted a general practitioner (15% vs. 33%, p = 0.028), whereas more children were transferred from other hospitals (52% vs. 31%, p = 0.043). There was no difference in terms of length of hospital stay, rate of postoperative intra-abdominal abscess, ER visits, and readmissions between both periods. Three children (4%) were diagnosed with COVID-19 and appendicitis.ConclusionsDespite an increase in the number of children with appendicitis managed at our hospital during the COVID-19-related lockdown, management, and outcome remained similar. Although our pediatric center was strongly affected by this pandemic, maintaining our prior practice strategies for acute appendicitis avoided the occurrence of collateral damage for those children.

Introduction Mastering paediatric laparoscopic surgery competency (PLSC) is technically challenging. The present study aimed to determine whether the inter-academic PLSC degree (IAD_PLSC) practical training program enables trainees to improve their skills. Methods This retrospective study included trainees enrolled in the IAD_PLSC program in 2021 and 2022 which included two separate 12 h-sessions. Trainees practiced to perform intracorporeal laparoscopic knots (ILK) using 5- and 3-mm instruments on a pelvi-trainer simulator; Peg Transfer (PT) was used as a dexterity test; final marks added the adapted Objective Structured Assessment of Technical Skills (OSATS) score (out of 40) and time to complete ILK. ILK was considered a success in case the OSATS score > 27/40. Results A total of 43 trainees completed the IAD_PLSC program. N  = 37 (86.0%) mastered the ILK (OSATS score > 27/40) using 5-mm instruments at the end of session 1 and N  = 33 (76.7%) at the end of session 2 ( p  = 0.41). There was no significant improvement in the median [interquartile range, IQR] OSATS score (32 [30–36] vs. 32 [28–34], p  = 0.19), but the median [IQR] knot completion time improved significantly (184 s [161–268] vs. 166 s [128–218], p  = 0.002). There was a significant correlation between the knot completion time and PT time at the end of session 1 (ρ = 0.58, 95% confidence interval, CI [0.31;0.76], p  < 0.0002) and session 2 (ρ = 0.78, 95%CI [0.62;0.87], p  < 0.0001). When using 3-mm instruments, N  = 34 (79.1%) of trainees mastered the ILK at the end of session 2. The median [IQR] OSATS score significantly improved between the end of the two sessions (22 [21–24] vs. 31 [28–33], p  < 0.0001), but there was no significant correlation between OSATS score or knot completion time and PT time, at the end of both sessions. Conclusion A nation-wide training program in paediatric laparoscopic surgery provides valuable practical training of intracorporeal suturing for residents in pediatric surgery and urology.

Posterior urethral valves (PUV) are congenital anomalies characterized by the persistence of mucosal folds in the urethra, leading to various degrees of obstruction. They are the most common cause of lower urinary tract obstruction in fetuses and children, with a severe prognosis, as one-third of affected children develop end-stage renal disease before adulthood. The French National Diagnostic and Care Protocol (NDCP) aim to provide healthcare professionals with guidelines for the optimal diagnostic and therapeutic management of PUV from the fetal stage to adolescence. The guidelines emphasize early diagnosis through prenatal ultrasound and the importance of a multidisciplinary approach involving pediatric urologists, nephrologists, and other specialists. It outlines prenatal interventions such as vesico-amniotic shunting and postnatal surgical options like endoscopic valve ablation to alleviate obstruction and preserve renal function. Long-term follow-up is crucial for monitoring renal function, managing bladder dysfunction, and preventing complications such as urinary tract infections and chronic kidney disease. The guidelines also identify off-label pharmaceuticals and necessary specialty products not typically covered by insurance. By standardizing care pathways and promoting consistent, high-quality care, the guidelines aim to improve the prognosis and quality of life for children with PUV, setting a benchmark for managing this rare condition in pediatric urology.

BackgroundLaparoscopic Nissen fundoplication (LNF) and gastrostomy tube (GT) placement may be performed concomitantly in children with gastro-esophageal reflux disease (GERD) and failure to thrive. We aimed to evaluate the rate and risk factors for LNF failure in children undergoing concomitant LNF/GT.MethodsA retrospective multi-institutional cohort study was conducted, reviewing patients that underwent LNF (2005–2014). Data collected included patient demographics, comorbidities, and type of GT (laparoscopy- or endoscopy-assisted). The primary outcome measure was LNF failure. Data was compared using contingency tables or Mann–Whitney tests, when appropriate. An exploratory analysis by Kaplan–Meier survival and Cox proportional hazards analysis was performed to determine predictors of time to LNF failure after LNF/GT.ResultsOf 189 children that underwent LNF, 99 (52%) had a concomitant GT (55% laparoscopy-, 45% endoscopy-assisted). LNF failed in 15% after LNF/GT and in 17% after LNF alone (p = 0.84), at a median age of 23 months (IQR 8–41). Using univariate analysis, we found that a younger age at the time of surgery (p = 0.05), prematurity (p = 0.0018), esophageal atresia (p = 0.01), and endoscopy-assisted GT (p = 0.02) were potential predictors of LNF failure after LNF/GT. After multivariate regression analysis, prematurity (p = 0.007) remained significantly associated with LNF failure after LNF/GT. No predictive factors for LNF failure after LNF alone were identified.ConclusionsConcomitant GT insertion and LNF is a common practice, as half of the children that undergo LNF also received GT insertion. Children born preterm or with esophageal atresia comprise a fragile population at high-risk of LNF failure after LNF/GT. Prospective, multicentric studies are needed to evaluate the best GT technique to use in children undergoing LNF.

PurposeRetrocaval ureter (RCU) is a rare congenital anomaly and published data on pediatric laparoscopic management are poor. The aim of this study was to report our experience of retroperitoneal laparoscopic approach for management of RCU in children.MethodsA retrospective review of data from patients treated for RCU between 2002 and 2018 in our institution was performed. All patients were positioned in a flank position and underwent a three-port (5-mm optical trocar and two 3-mm trocars) laparoscopic retroperitoneal ureteroureterostomy. Anastomosis was made by 6/0 absorbable sutures. A JJ stent was always inserted.ResultsFive patients with a median age of 94 months (5–152) were operated on and followed up for a median time of 103 months (46–201). Median operating time was 200 min (160–270). No conversion and no transfusion occurred. Median hospital stay was 2 days (1–4). Ureteral stent was removed after 52 days (47–82). Complications included pyelonephretis (N = 1). In all cases, hydronephrosis decreased postoperatively.ConclusionsRetroperitoneal laparoscopic approach for RCU is safe and effective in children. Our video demonstrates different patients with specific surgical details to show how to manage these children. The global vision of the upper tract by laparoscopy leads to optimal management of these children even if the anomaly was not detected preoperatively.

Purpose Post-procedural urinary tract infections (ppUTIs) following voiding cystourethrography (VCUG) vary widely, with rates from 0 to 42%, though recent studies suggest rates typically below 5%. Verifying urine sterility before VCUG is traditionally done but questioned. This study assessed the 7-day ppUTI rate post-VCUG without prior urine sterility confirmation and identified associated risk factors. Methods A retrospective review of VCUG cases in children under three years at a pediatric hospital over two years was conducted. Exclusions included neuropathic bladder, bladder exstrophy, pre-VCUG urine cultures, and lost-to-follow-up cases. Achieving a ppUTI rate below 5% would support safe VCUG practice without pre-urine culture. Results Of 318 VCUGs performed on 300 children, 248 (78%) were males (8% circumcised) with a median age of 5 months. Retrograde VCUG was more common than suprapubic cystography (63% vs. 37%). Before the test, 33.6% received antibiotics, mostly prophylactically. Hydronephrosis was present in 66.4%, and 69% had a history of UTI. VCUG results were abnormal in 43% of cases: 85% had vesicoureteral reflux (VUR), 10% had posterior urethral valves (PUV), and 28% had other abnormalities. The 7-day ppUTI rate was 3.8%, with 67% of ppUTI cases having abnormal VCUG results versus 41% without ppUTI (p = 0.06). No significant risk factors for ppUTI were identified. Conclusions Omitting systematic urine culture before VCUG was not associated with a high ppUTI rate, even in children with pre-existing urologic conditions or a history of UTI, indicating that VCUG can be safely performed without prior urine sterility confirmation. No risk factors for ppUTI were identified.