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Background: In recent years, evolution of surgery has led to laparoscopy and then to single port surgery. In pediatric age, few papers have been published about single port procedures; in particular, no one has described the use of the Octoport device (Frankenman International Ltd., Suzhou, China). We present our experience using a new device., Methods: A retrospective analysis of first 300 cases was performed collecting the data of all patients treated with Octoport device from October 2017 to September 2021. Epidemiological data, diagnosis, operative times, and complications were analyzed. Postoperative pain was compared with standard laparoscopy., Results: A total of 300 procedures were performed during the study period. The age range was 1-17 years. The conversion rate was 3.6% (11 patients) including both conversion to traditional laparoscopy and to laparotomy. Pain management was comparable to traditional laparoscopy. The complication rate was 3.6%, in one case leading to re-do surgery. All the cases in our Unit were successfully completed, with complications mainly related to the original pathology rather than to the technique itself., Conclusions: The learning curve for Octoport use proved to be functional as for standard laparoscopy. In this study, surgical indications for the use of single port laparoscopy were defined, discerning favorable and unfavorable procedures. A proven superiority of this technique over traditional laparoscopy is yet to be defined, but Octoport has proved to be a safe and easy tool to reduce invasiveness of procedures in pediatric surgery with better cosmetic results.

Occult spinal dysraphism (OSD) comprises different forms of failure in embryogenic development that can lead to genitourinary, spinal, or lower limb alterations, thus determining progressive neurological deterioration. The correct management of children harboring OSD represents a significant issue during their life up to adulthood. However, patients often have to entertain individual consultations with each specialist. We settled on a multidisciplinary team comprising pediatric neurosurgeons, urologists, neurologists, orthopedists, and other supporting physicians. We present the results of such actions by analyzing a series of 141 children with OSD subjected to neurosurgical procedures, evaluating the impact of multidisciplinary management on outcomes. We also evaluated the specific actions according to the different ages of OSD patients from birth to adulthood to provide a schematic plan that could represent a basis for establishing and disseminating the need for a multidisciplinary approach in OSD management. The multidisciplinary team allows all consultants to see the patient together, covering specific aspects of history and examination pertinent to their management. Offering a one-stop service prevents coordination issues between the different medical teams, avoids delays or cancellations of the various appointments, optimizes cost-effectiveness, and improves efficiency and parents' satisfaction.

Esophageal atresia (EA) is the most common congenital esophageal malformation. An improvement in survival led to a focus on functional outcomes and quality of life (QoL). We analyzed the long-term outcomes and QoL of patients submitted to surgery for EA. Perinatal characteristics, surgical procedures, gastrointestinal and respiratory current symptoms and QoL were investigated. Thirty-nine patients were included. Long Gap patients had a higher rate of prematurity and low birth weight. The prevalent surgical procedure was primary esophageal anastomosis, followed by gastric pull-up. Twenty-four patients had post-operative stenosis, while gastroesophageal reflux (GER) required fundoplication in eleven cases. Auxological parameters were lower in Long Gap patients. The lowest scores of QoL were in the Long Gap group, especially in younger patients, which was the group with the highest number of symptoms. In the long term, the QoL appeared to be more dependent on the type of esophageal atresia rather than on associated malformations. Surgical management of GER was indicated in all patients with Long Gap EA, supposedly due to the prevalence of gastric pull-up for this type of EA. The assessment of QoL is part of surgeon's management and needs to be performed in each phase of a child's development.

Background: Ongoing innovation in laparoscopy lead pediatric surgeons to consider a mini-invasive approach for inguinal hernia correction. We review cases and evolution of surgical technique in a pediatric center., Methods: A retrospective study included patients that underwent inguinal hernia repair between 01/01/2011 and 30/06/2019. Surgical techniques are compared. Outcomes considered: surgery duration, intraoperative and postoperative complications., Results: 664 patients were included. 187 patients underwent laparoscopy(group A), 477 underwent open surgery(group B). Throughout time from 2011 to 2019, there has been an increase in laparoscopy, accounting in 2019 for more than 60% of overall surgeries. In 151 patients of group A initial diagnosis was of monolateral hernia; in 25.8% contralateral side was corrected at the same time because of intraoperative finding of open internal inguinal ring. Surgery duration in group B is shorter than group A;difference loses significance in bilateral corrections. Complications : 2.9% short term: prematurity related as well as to duration in group B. 1.7% relapses, regardless of technique. 5.2% metachronous hernias, related with age and open surgery. 0.6% secondary criptorchidism, unrelated to technique., Conclusions: There is not an evident superiority of laparoscopy over open repair. Laparoscopy should be preferred in case of doubt about bilaterality and in case of emergency surgery.

Background: Homozygous mutations in the transcription factor RFX6 are the cause of the Mitchell-Riley syndrome (MRS) associating neonatal diabetes, congenital digestive system, such as biliary atresia, pancreatic hypoplasia, duodenal and/or jejunal atresia, intestinal malrotation, gallbladder aplasia, cholestasis. A constitutive inactivation of RFX6 leads also to gastric heterotopia. Application of RNA-seq in human diseases may help to better understand pathogenic mechanism of diseases and to predict the risk of developing chronic disorders and personalizing their prevention and treatment. We evaluated oncogenic patterns and cancer predisposition using the transcriptomic profile in a case of MRS with neonatal diabetes, duodenal atresia, and extensive intestinal tract gastric heterotopia., Results: We signalled the interactors of RFX6 with other up and downregulated genes, that may be interested in severity of diabetic condition, in multi-organs impairment and cancer predisposition. Furthermore, several dysregulated genes are involved in biological processes that can lead to promote cancer including "Evading apoptosis" (BAD, BBC3, EGF, FGFR2, FLT3LG, HMOX1, HRAS, IFNAR2, IGF1R, IL12RB1, IL13RA1, IL15, IL2RB, IL2RG, IL6R, KEAP1, MGST1, PDGFA, PDGFRB, PIK3R3, RALB, RALGDS, RASSF1, SOS1, TGFA, TXNRD3), "Proliferation" (APC, BRAF, CCND2, CCND3, CCNE2, FGFR2, FLT3LG, FZD1, FZD6, HMOX1, HRAS, IGF1R, KEAP1, LRP6, MAPK3, MGST1, PDGFA, PDGFB, PDGFRB, RB1, SOS1, TGFA, TXNRD3, WNT10B), "Sustained angiogenesis" (BRAF, FGFR2, FLT3LG, HRAS, IGF1R, JAG1, MAPK3, NOTCH2, PDGFA, PDGFB, PDGFRB, SOS1, TGFA, TGFB1), "Genomic instability" (BAD, BBC3) and "Insensitivity to anti-growth signals" (SMAD2, TGFB1). We also inspected the signalings and their related genes in cancer, such as "PI3K signaling", "ERK signaling", "JAK-STAT signaling", "Calcium signaling", "Other RAS signaling", "WNT signaling"., Conclusions: In our MRS patient, we signaled the interactors of RFX6 with other up- and downregulated genes that may be related to severe diabetic condition, multi-organ impairment, and cancer predisposition. Notably, many dysregulated genes may lead to triggering carcinogenesis. The possibility of the patient developing cancer degeneration in heterotopic gastric mucosa and/or additional long-term tumoral sequelae is not excluded. Personalized prevention and treatment strategies should be proposed., (© 2021. The Author(s).)

Pediatric obesity is a multifaceted disease that can impact physical and mental health. It is a complex condition that interweaves biological, developmental, environmental, behavioral, and genetic factors. In most cases lifestyle and behavioral modification as well as medical treatment led to poor short-term weight reduction and long-term failure. Thus, bariatric surgery should be considered in adolescents with moderate to severe obesity who have previously participated in lifestyle interventions with unsuccessful outcomes. In particular, laparoscopic sleeve gastrectomy is considered the most commonly performed bariatric surgery worldwide. The procedure is safe and feasible. The efficacy of this weight loss surgical procedure has been demonstrated in pediatric age. Nevertheless, there are barriers at the patient, provider, and health system levels, to be removed. First and foremost, more efforts must be made to prevent decline in nutritional status that is frequent after bariatric surgery, and to avoid inadequate weight loss and weight regain, ensuring successful long-term treatment and allowing healthy growth. In this narrative review, we considered the rationale behind surgical treatment options, outcomes, and clinical indications in adolescents with severe obesity, focusing on LSG, nutritional management, and resolution of metabolic comorbidities.

Considerable reorganization of the regional network for pediatric burn treatment during the pandemic was required to cope with severe burn injuries in small children. In support of the emergency network for burns during the COVID-19 pandemic, we referred to regional indications for centralization in our hospital for all children aged less than 5 years who presented with severe burns, >15% of total body surface area (TBSA), or who necessitated admittance to the pediatric intensive care unit (PICU). A new service with a dedicated management protocol was set up to treat pediatric burns in our SARS-CoV-2 pediatric hospital during the lockdown period. A multidisciplinary burn treatment team was set up to offer compassionate and comprehensive burn care. Patient's clinical data, burn features, treatment and follow up were recorded. A higher number of admissions was recorded from February to December 2020 compared with the same period in 2019 (52 vs. 32 admissions). Eighteen patients were admitted to the COVID-19 Service (10 M/8 F; 3.10 ± 2.6 yrs); ten children (55.5%) were hospitalized in the ward and eight in the ICU (44.5%). Fifty percent of the cases presented with lesions extending over >15% TBSA; in one case, TBSA was 35%. All patients suffered 2nd-degree burns; while five patients also had 3rd degree lesions covering more than 15% TBSA. All of the injuries occurred at home. No major secondary infections were recorded. Successful treatment was achieved in 94.4% of cases. The average length of stay was 15.2 ± 12.6 days. A proactive, carefully planned service, involving a multidisciplinary team, was created to ensure appropriate care in a pediatric hospital during the COVID-19 period, despite the effective pandemic associated challenges. Better health promotion in pediatric burn cases should also include dedicated TBSA assessment and a database of children's burn characteristics.

Varicocele is the primary cause of male infertility and the interests of research about varicocele have changed in the last years. The aim of the study was to analyse how topics of interests about varicocele have changed in the last two decades. Literature about paediatric varicocele between 2000 and 2018 was reviewed, and the subjects of the articles were analysed, selecting the main and secondary topics of each article. A study of their prevalence over the years was performed. In the research, 625 articles were found but only 169 provided data in the paediatric age. The total percentage of exclusively paediatric works was 27.04%, being above average in Europe, North and South America. The main subject both for Europe and North America was surgical technique, while for Asia it was videolaparoscopy. The main issue in South America was fertility; Africa and Oceania do not have enough publications to make statistics. Paediatric articles account for less than one-third of overall articles. The first interest of research is surgical technique. In Europe, the topics seem to exclude fertility potential., (© 2020 Wiley-VCH GmbH.)

Background: Cryptorchidism is associated with alteration of fertility potential. The aim of this study is to evaluate mid/long-term morphology and volume of the operated testis for undescended testes by using elastosonography., Materials and Methods: The medical records of consecutive patients who had received orchiopexy at the Authors' Institution between January 2014 and January 2017 were retrospectively considered. The patients enrolled in the study were divided into different groups depending on their age at the time of the procedure and on the time elapsed from it. The radiological and surgical examinations examined position, volume and trophism of both testes., Results: During the study period 270 patients received surgery; only 34 patients complied with the inclusion criteria: 19 with right orchiopexy and 15 with left orchiopexy (P > 0.05) (P = 0.57). The mean testicular volume of the operated testis was 0.59 ± 0.32 ml, while the mean testicular volume of non-operated testis was 0.88 ± 0.34 ml (P < 0.05). The elastosonographic comparison between operated and non-operated testis showed that the operated testis had a higher elastosonographic result (grade 2-3) (P < 0.05). Higher grades at elastosonography corresponded to smaller testicular volume., Conclusion: This study demonstrates that the patient's age at surgery is correlated with significative differences in terms of volume and elasticity (testicular quality). The study also shows that there is not a progressive improvement of elasticity at follow-up., Competing Interests: None.

Background: Female vaginoplasty reconstruction, by choice, is usually performed with adjacent tissue. However in some clinical conditions such as high urogenital confluence sinus, cloacal malformation with extreme vaginal hypoplasia, local tissue may not be available. When vaginal replacement is performed in pediatric patients intestinal segments is preferred to non-operative procedures that require continuative dilations. However mucus production, malignant transformation risk and diversion colitis are important side effects., Technique: We present a nouvel technique for vaginoplasty in a female child presenting with an isolated urogenital sinus malformation without virilization. The patient at 20 months underwent vaginoplasty using tubularized bladder mucosal graft., Results: Surgical procedure was devoid of complications. Pubertal development occurred at age of 15. She underwent regular follow up until 18 years of age. At this age we performed clinical evaluation: absence of vaginal introitus stenosis and good cosmetic results were observed. Then she underwent vaginoscopy with multiple biopsies. Pathology examination of the bladder mucosal graft evidenced a normal structure of the mucosa, with a stratified squamous epithelium., Discussion: Different techniques are taken into account for vaginal reconstruction according to the severity and to the type of malformation. We describe the use of bladder mucosal graft with favorable results after long term follow-up., (Copyright © 2018 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

Objective: Multipotential cells are mobilized into peripheral blood in response to trauma, in particular in severe burns. These cells migrate to the site of injury in response to chemotactic signals to modulate inflammation, repair damaged tissue and facilitate tissue regeneration. We evaluated the possibility of isolating and in vitro expand mesenchymal stromal cells (MSCs) from granulation tissue (GT) during debridement of a burn wound, as a persective strategy to improve skin regeneration. Methods: GT obtained from a 12-month-old burn patient was in vitro cultured. Expanded MCSs were characterized for morphology, immunophenotype, differentiation capacity and proliferative growth. Antifibrotic features were also evaluated. Results: It was possible to isolate and in vitro expand cells from GT with the morphology, phenotype, proliferative and differentiation capacity typical of MSC, these cells were defined as GT-MSC. GT-MSCs exhibited antifibrotic features by releasing soluble factors, this activity was superior to that observed in BM-MSC. Conclusions: Successful isolation and expansion of MSCs from GT is reported. Considering their functional characteristics, GT-MSCs could be considered a good candidate adjuvant therapy to improve burn wound healing, particularly in pediatrics.