The central question of nephron-sparing surgery in unilateral non-syndromic Wilms tumour sits at a crossroads between surgery, oncology, and nephrology. There has been a significant paradigm shift in ...paediatric oncology towards reducing toxicity and addressing long-term treatment-related sequalae amongst childhood cancer survivors. After paediatric nephrectomy and 30–50 years of follow-up, 40% of patients will have chronic kidney disease, including 22% with hypertension and 23% with albuminuria. It is difficult to predict which patients will progress to develop hypertension, reduced glomerular filtration rate, albuminuria, and a higher cardiovascular risk. For these reasons, nephron-sparing surgery when it is technically feasible must be considered. To decrease the incidence of positive surgical margins (viable tumour present at a resection margin), incomplete lymph node sampling, and complications, these procedures should be performed at specialist and experienced reference centres. Based on the impacts of individual treatment pathways, survivors of childhood WT need to be followed through adulthood for early detection of chronic kidney disease, hypertension, and prevention of cardiovascular events.
CLOVES syndrome (congenital lipomatous overgrowth, vascular malformations, epidermal naevi, scoliosis/skeletal and spinal syndrome) is a genetic disorder that results from somatic, mosaic ...gain-of-function mutations of the PIK3CA gene, and belongs to the spectrum of PIK3CA-related overgrowth syndromes (PROS). This rare condition has no specific treatment and a poor survival rate. Here, we describe a postnatal mouse model of PROS/CLOVES that partially recapitulates the human disease, and demonstrate the efficacy of BYL719, an inhibitor of PIK3CA, in preventing and improving organ dysfunction. On the basis of these results, we used BYL719 to treat nineteen patients with PROS. The drug improved the disease symptoms in all patients. Previously intractable vascular tumours became smaller, congestive heart failure was improved, hemihypertrophy was reduced, and scoliosis was attenuated. The treatment was not associated with any substantial side effects. In conclusion, this study provides the first direct evidence supporting PIK3CA inhibition as a promising therapeutic strategy in patients with PROS.
Prenatal therapy for LUTO (Lower Urinary Tract Obstruction) is debated due to mixed results regarding postnatal renal function following fetal cystoscopy or vesicoamniotic shunting. Current ...literature is, however, limited by the inability to determine the cause of the obstruction using plain sonography and the lack of selection criteria for fetuses who may benefit from prenatal therapy. Fetal cystoscopy may serve as a diagnostic tool and would offer a more "physiologic" treatment for bladder outlet obstruction. However, it carries additional technical issues due to inappropriate instrumentation.
Les résultats du traitement prénatal des obstacles sous-vésicaux sont souvent mitigés en termes de bénéfices sur la fonction rénale. Devant le diagnostic prénatal de mégavessie fœtale persistante, ...aucun signe échographique ne permet actuellement de déterminer avec certitude la cause de l’obstacle. La cystoscopie fœtale, qui consiste en l’exploration endoscopique de la vessie, pourrait permettre de déterminer la nature de l’obstacle et de le traiter éventuellement, en maintenant le cycle de réplétion/vidange vésicale. L’absence de critères de sélection des fœtus candidats à la cystoscopie fœtale et les difficultés techniques de sa réalisation limitent cependant son utilisation. Nous présentons dans cette revue les principes de cette intervention, les résultats obtenus et les limites, ainsi que les axes de recherches actuels.
Prenatal therapy for LUTO (Lower Urinary Tract Obstruction) is debated due to mixed results regarding postnatal renal function following fetal cystoscopy or vesicoamniotic shunting. Current literature is, however, limited by the inability to determine the cause of the obstruction using plain sonography and the lack of selection criteria for fetuses who may benefit from prenatal therapy. Fetal cystoscopy may serve as a diagnostic tool and would offer a more “physiologic” treatment for bladder outlet obstruction. However, it carries additional technical issues due to inappropriate instrumentation.
Robotic surgical oncology in children calls for experienced surgeons in minimally invasive surgery (MIS) and a solid oncological background. The aim of this review was to analyze the current state of ...robotic-assisted laparoscopy in pediatric tumor resection, assess the necessary framework of minimally invasive surgical oncology and describe future developments of the robotic technology.
A literature search of the MEDLINE/PubMed database was conducted, using the terms "robotic surgery", "pediatric" or "children" and "oncology" or "tumor". All relevant English-language studies published between 2008 and 2022 were reviewed.
Although concerns have been raised regarding the use of MIS in surgical oncology, current literature reports similar oncological outcome if surgeons comply with the oncologic principles. The benefits of MIS have been established for robotic surgery in adult studies, including a shorter time to adjuvant chemotherapy. Surgical feasibility should be assessed based on tumor characteristics, preoperative imaging focusing on vascular involvement and surgeon's experience until clear guidelines are issued. The difficulties in establishing eligibility criteria for robotic resection of pediatric tumors lie in the great variability of indications, heterogeneity in tumor histology with their own surgical specificities, and wide range of age and weight, as shown by the literature review we performed. Between 2008 and 2022, 31 studies reported 171 cases with three studies including at least ten patients. The most reported procedure was adrenalectomy (41 cases). Current research in pediatric surgical oncology focuses on intraoperative locoregional treatment, improved vision with fluorescence and dyed-loaded specific probes and the many possibilities of enhancement software using the robotic console.
The robotic technology allows the surgeon to push the boundaries of conventional laparoscopy. Specific surgical guidelines are necessary.
Experimentally recorded point cloud data, such as those generated by single-molecule localization microscopy, are continuously increasing in size and dimension. Gaining an intuitive understanding and ...facilitating the analysis of such multidimensional data remains challenging. Here we report a new open-source software platform, Genuage, that enables the easy perception of, interaction with and analysis of multidimensional point clouds in virtual reality. Genuage is compatible with arbitrary multidimensional data extending beyond single-molecule localization microscopy.
This study aimed to report a multi-institutional experience with robot-assisted laparoscopic surgery (RALS) for treatment of urinary tract stones in children. The medical records of 15 patients (12 ...boys), who underwent RALS for urolithiasis in 4 international centers of pediatric urology over a 5-year period, were retrospectively collected. The median patient age was 8.5 years (range 4–15). Eleven/fifteen patients (73.3%) had concurrent uretero–pelvic junction obstruction (UPJO) and 2/15 patients (13.3%) had neurogenic bladder. Stones were in the renal pelvis in 8/15 (53.3%), in the lower pole in 3/15 (20%), in the bladder in 2/15 (13.3%), and in multiple locations in 2/15 (13.3%). One patient (6.6%) had bilateral multiple kidney stones. The median stone size was 10.8 mm (range 2–30) in upper tract location and 27 mm (range 21–33) into the bladder. Eleven patients with concomitant UPJO underwent simultaneous robot-assisted pyelolithotomy and pyeloplasty in 12 kidney units. Two patients with isolated staghorn stones received robot-assisted pyelolithotomy. Robot-assisted cystolithotomy was performed in two patients with bladder stones. The median operative time was 131.8 min (range 60–240). The stone-free rate was 80% following initial surgery and 100% after secondary treatment. Clavien 2 complications (hematuria, infections) were recorded in 5/15 patients (33.3%). Three/fifteen patients (20%) with residual renal stones were successfully treated using ureterorenoscopy (Clavien 3b). RALS was a feasible, safe and effective treatment option for pediatric urolithiasis in selected cases such as large bladder stones, bilateral kidney stones, staghorn stones or concomitant anomalies such as UPJO requiring simultaneous pyeloplasty.
Since bladder neck dextranomer/hyaluronic acid copolymer (Deflux) injections can improve urinary incontinence of various etiologies, we hypothesized that incontinent children with bladder ...exstrophy-epispadias complex would benefit from dextranomer/hyaluronic acid copolymer. We aimed to analyze dextranomer/hyaluronic acid copolymer efficacy and predictors of treatment success in bladder exstrophy-epispadias complex.
Incontinent bladder exstrophy-epispadias complex children aged >4 years undergoing dextranomer/hyaluronic acid copolymer injections in our hospital between October 1997 and January 2021 were included. Medical history, continence, and bladder capacity before injections were recorded. Postoperatively, patients were categorized as "dry," "significantly improved," or "failure." Postoperative complications, bladder emptying mode, and bladder capacity were reported. Failure-free survival was estimated by Kaplan-Meier models.
Altogether, 58 patients (27 male epispadias, 9 female epispadias, 22 bladder exstrophy) underwent 105 injections at median age of 8.5 (interquartile range 5.8-12) years. Previous bladder neck reconstruction had been performed in 38 (66%) and 33 (57%) received multiple injections. Complications occurred in 9%. Five-year failure-free survival was 70% (standard error 9.1) in males epispadias compared to 45% (9.0) in females and exstrophy patients (
= .04). Previous bladder neck reconstruction associated with improved 5-year failure-free survival in females and exstrophy patients (58%, SE 11) but not in male epispadias (75%, SE 11).
Dextranomer/hyaluronic acid copolymer injections provided satisfactory and lasting continence in over half of bladder exstrophy-epispadias complex patients. While injections were successful in male epispadias regardless of bladder neck reconstruction timing, results were better in bladder exstrophy and female epispadias after previous bladder neck reconstruction.
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