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Introduction To investigate the current experience of the ARM-Net Consortium in the management of epididymo-orchitis (EO) in patients with anorectal malformations (ARMs), and to identify specific risk factors and the need for urological care involvement. Materials and Methods We retrospectively collected data of EO in patients with ARM between 2015 and 2019. Data on urological aspects, ARM type, surgical approach, associated anomalies, diagnosis, and treatment of EO were collected and analyzed. Results Twenty-nine patients were reported by 12 centers. Twenty-six patients with EO (90%) had ARM with a rectourinary fistula. Median age at first EO was 2 years (range: 15 days–27 years). Twenty patients (69%) experienced multiple EO, and 60% of recurrences were ipsilateral. Associated urological anomalies included vesicoureteral reflux (48%), urethral anomalies (41%), neurogenic bladder (41%), and ectopic vas (10%). A positive urine culture during EO was present in 69%. EO was treated with antibiotics (90%), limiting surgical exploration to 14%. Prevention of recurrences included surgery (bulking agents 15%, vasectomy 15%, and orchiectomy 5%) and antibiotic prophylaxis (20%). Conclusion Urologists may encounter patients with EO in ARM patients, frequently with positive urine culture. An appropriate urologic work-up for most ARM patients is necessary to identify and treat underlying risk factors. A practical scheme for the work-up is suggested for a close collaboration between pediatric surgeons and urologists.

Background Esophageal achalasia (EA) is a rare primary motility disorder in any age group, and particularly rare in the pediatric population, with a reported incidence of 0.18 per 100,000 children a year. EA in pediatric age is currently treated in the same way as in adults, but this approach is based on only a few studies on small case series. The aim of this retrospective study was to assess the long-term outcome of the laparoscopic Heller–Dor (LHD) procedure when performed in pediatric patients with EA at our university hospital. Materials and Methods We considered children and adolescents younger than 16 years old diagnosed with EA and treated with LHD between 1996 and 2022. Clinical data were prospectively collected in an ongoing database. Symptoms were recorded and their severity was calculated using the Eckardt score. Barium swallow, esophageal manometry (conventional or high-resolution), and endoscopy were performed before and after the surgical procedure. Results During the study period, 40 children with a median age of 14 years (interquartile range [IQR]: 11–15) underwent LHD. At a median follow-up of 10.5 years (IQR: 4.5–13.9), a good outcome was achieved in 36/40 patients (90%). Two of the four patients whose surgical procedure failed underwent complementary pneumatic dilations successfully, thus increasing the overall success rate to 95%. A previous endoscopic treatment (in five patients) did not affect the final outcome (p = 0.49). An intraoperative mucosal lesion was detected in only one patient (2.5%) and was repaired at the time without further consequences. During the follow-up, 22 patients underwent endoscopy, and 17 had pH monitoring as well: only 2 of these patients showed reflux esophagitis at endoscopy (one of them with abnormal findings on pH monitoring), amounting to a 9.1% rate of instrumentally confirmed postoperative reflux. Conclusion LHD is a safe and persistently effective treatment for EA in pediatric age, with a success rate comparable with what is usually obtained in adults, and better than what has been reported to date in the pediatric literature. Adding a fundoplication certainly helps ensure an optimal long-term control of any gastroesophageal reflux induced by the myotomy.

Introduction Vascular complications are severe complications of pediatric kidney transplantation (KT). We aimed to investigate whether a complex bench surgery (BS) affects the outcomes. Methods All pediatric KT performed at the University Hospital of Padua from 2015 to 2019 were analyzed, comparing those in which a standard BS was possible to those that necessitated a complex BS. The rates of vascular complications, patients' outcome, and graft survival were compared in the two groups. Results Eighty KTs were performed in 78 patients with a median age of 11 years (interquartile range [IQR] 4.3–14) and a median body weight of 24 kg (IQR 13–37). Thirty-nine donor kidneys (49%) needed a complex BS due to anomalies of renal veins in 12 (31%) and renal arteries in 16 (41%). The remaining 11 grafts (28%) underwent an elongation of the vein. There was no difference in the rate of primary graft non function (p = 0.97), delayed graft function (p = 0.72), and overall survival (p = 0.27). The rates of vascular complications, bleedings, and venous graft thrombosis were similar (p = 0.51, p = 0.59, p = 0.78, respectively). No arterial thrombosis or stenosis was reported. Conclusion Complex BS did not compromise survival of the graft and did not put the allograft at risk of vascular complications, such as bleedings or thrombosis.

Background: Registries are important in rare disease research. The Anorectal Malformation Network (ARM-Net) registry is a well-established European patient registry collecting demographic, clinical, and functional outcome data. We assessed the quality of this registry through review of the structure, data elements, collected data, and user experience. Material and methods: Design and data elements were assessed for completeness, consistency, usefulness, accuracy, validity, and comparability. An intra- and inter-user variability study was conducted through monitoring and re-registration of patients. User experience was assessed via a questionnaire on registration, design of registry, and satisfaction. Results: We evaluated 119 data elements, of which 107 were utilized and comprised 42 string and 65 numeric elements. A minority (37.0%) of the 2278 included records had complete data, though this improved to 83.5% when follow-up elements were excluded. Intra-observer variability demonstrated 11.7% incongruence, while inter-observer variability was 14.7%. Users were predominantly pediatric surgeons and typically registered patients within 11–30 min. Users did not experience any significant difficulties with data entry and were generally satisfied with the registry, but preferred more longitudinal data and patient-reported outcomes. Conclusions: The ARM-Net registry presents one of the largest ARM cohorts. Although its collected data are valuable, they are susceptible to error and user variability. Continuous evaluations are required to maintain relevant and high-quality data and to achieve long-term sustainability. With the recommendations resulting from this study, we call for rare disease patient registries to take example and aim to continuously improve their data quality to enhance the small, but impactful, field of rare disease research. Level of Evidence: V.

The aim was to assess the success of a three-drug regimen, consisting of cefazoline, metronidazole and gentamicine, for the antimicrobial treatment of complicated appendicitis and to investigate predictors of failure.This retrospective study included patients who had undergone appendectomy for complicated appendicitis from 2013 to 2018. The shift to second-line antibiotics was considered a failure. The choice was based upon clinical deterioration. Patients were grouped into 2 groups: localized complicated appendicitis (LCA) and extensively complicated appendicitis (ECA) for the study purpose. Univariate and multivariate analysis were performed to identify predictors of failure.Ninety patients (65.2%) with LCA and 48 patients (35%) with ECA were included. Three-drug regimen failed in 50 patients (36%) with a higher rate in the ECA group (50%, p=0.017). In a multivariate analysis, this failure was found to be associated with ECA (adjusted OR 3.00 [1.2-7.4], p=0.041). Children with ECA experienced a longer hospital stay (median length 8 days, plt; 0.001) and antimicrobial therapy (median length 8 days, plt; 0.001). However, no difference in the rate of surgical site infections was found (p=0.514).The institutional antibiotic stewardship program highlighted a high failure rate for the old threedrug regimen. A new protocol should be recommended, especially for the patients affected by ECA.

Background: Congenital pouch colon (CPC) is a rare variant of anorectal malformations (ARM) with its highest reported incidence in India. We aimed to describe five patients affected by CPC, in which the tissue from the terminal dilated colon has been successfully used and to discuss our results on the light of an extended revision of the literature. Materials and Methods: The clinical details of five cases treated for CPC in two Italian Centers were retrospectively reviewed assessing the fate of the terminal dilated colon. Results: In all cases, the tissue from dilated colon has been used. The double vascular system of the dilated pouch allowed increasing bladder capacity (case 4), reconstruction of the vagina (case 3, 5), and lengthening of the colon (case 1, 2, 5). In our series, 3/5 have a good bowel control with daily bowel management after ARM correction. In literature, there are not differences in terms of dependence from bowel management in patients with pouch resected and in patients with pouch saved (P = 0.16). Conclusions: We acknowledge that the analysis of the available literature is limited by the absence of studies with high level of evidence and the removal or the preservation of the abnormal colon tissue seems to follow the surgeon preferences.

End stage renal disease (ESRD) requiring kidney transplant (KT) remains an important cause of morbidity in anorectal malformations (ARM) patients. Current literature is scarce on defining the risk factors for ESRD and the outcomes of KT in ARM patients.This study aimed to identify predisposing factors to ESRD in ARM patients and verify if the long term outcome of KT in these patients differs from pure urological anomalies (UA).Databases of ARM and KT patients treated at our center between 2000 and 2016 were used for comparing characteristics of ARM cases which developed ESRD and those who did not, and the outcome features of MAR-KT vs UA-KT.Out of 117 ARM patients, 9 developed ESRD. All of them had a complex ARM. Association with UA were significantly higher in ARM-KT compared to other ARM patients (100% vs. 52%, p = 0.001). The most common UA associated to KT in ARM patients was renal dysplasia. During the same period 23 patients underwent KT as a sequelae of pure urological anomalies (UA-KT group). The most represented UA were primary vesico-ureteral reflux (65.2%) and posterior urethral valves (14%) in UA-KT (table 2). ARM-KT patients required more often hemodialysis before KT (50% vs. 8.7%, p = 0.05) and an aorto-caval anastomosis at the transplant (75% vs. 30%, p = 0.04) compared to UA-KT. Moreover ARM-KT patients experienced more often graft failure and and the need for a second KT (50% vs. 8.6%, p = 0.02).To our knowledge, this is the first study describing the differences in terms of risk factors and outcomes of KT in ARM patients. We observed a need for KT in 7% of ARM, which is at the higher end of the range reported in the literature. Bilateral dysplasia and cloaca malformation seem the leading cause to ESRD for ARM patients compared to vesico-ureteral reflux and posterior urethral valve in UA-KT. Our experience showed that KT has worst outcome in ARM vs pure UA patients.Patients with complex ARM are more frequently associated to renal dysplasia which lead to KT. Graft in ARM patients seems to be the more vulnerable and prone to failure.

Pediatric abdominal surgery is constantly evolving, alongside the advent of new surgical technologies. A combined use of new tools and traditional surgical approaches can be useful in the management of complex cases, allowing less invasive procedures and sometimes even avoiding multiple interventions. This combination of techniques has implications even from the anesthetic point of view, especially in post-operative pain control. Thereby, tertiary level centres, including highly-specialized professionals and advanced equipment, can maximize the effectiveness of treatments to improve the final outcomes. Our paper aims to present some possible combinations of techniques recently used at our institution to provide a one-session, minimally invasive treatment within different areas of abdominal surgery.

Introduction and importance: Accidental ingestion of foreign bodies (FBs) is common among infants. In case of sharp FBs, the risk of accidental organ damage with potential life-threatening complications constitutes an absolute indication for removal. We present the case of a child, who, following the ingestion of an open safety pin, was successfully treated exclusively with minimally invasive techniques. Case presentation A 9-month-old male patient was admitted for hematemesis. An anteroposterior and lateral X-ray of the thorax and abdomen revealed the presence of an open safety pin in the epi-mesogastric region, without a precise localization. Upper and lower gastrointestinal endoscopy, fluoroscopy, and laparoscopy were combined in the same intervention to localize and safely remove the foreign body. The patient was dismissed on a postoperative day 1. Clinical discussion and conclusion The two main pitfalls of this scenario were the initially uncertain location of the foreign body and the young age of the patient. A combination of different techniques was used to safely locate and remove the foreign body, reducing hospitalization and avoiding repeated radiological exposure. An experienced team in a tertiary paediatric surgical and endoscopic centre increases the chances of success and minimizes invasiveness and the risk of complications., Highlights • We report on a completely minimally invasive extraction of a sharp foreign body • Hints to ensure optimal combination of laparoscopy and endoscopy are provided • Importance of referral to a tertiary centre with expertise in paediatric endoscopy and minimally invasive surgery

BACKGROUND Allograft venous thrombosis is a severe complication after kidney transplantation (KT). Early diagnosis and prompt treatment are crucial in preserving the survival of the allograft. In this study, we aimed to describe an emergent strategy for the management of acute allograft venous thrombosis. CASE PRESENTATION A 4-year-old girl, weighing 13.5 kg, was diagnosed with bilateral congenital renal hypodysplasia, urogenital sinus and anorectal malformation. The patient was referred to our department for living-donor KT. Her mother was eligible as a donor, presenting a body weight ratio of 1:4.5. Thrombosis of the inferior vena cava (ICV) was also identified, without any predisposing factor for thrombophilia. KT was performed by an extraperitoneal approach without complications. Venous anastomosis required a human vascular graft sutured to the ICV, and renal artery was anastomosed to the aorta. On postoperative day (POD) 8, acute abdominal pain and hematuria led to the diagnosis of an allograft venous thrombosis. An emergent laparotomy was required to explant the allograft, followed by bench surgery. The allograft was irrigated with thrombolytic agents and lactated Ringer's solution and then after removing the venous vascular graft, it was reimplanted through vascular anastomosis with the ICV and aorta. The recovery of perfusion and function was good with diuresis since day 4 after re-surgery. At 2-year follow-up, the child presented normal allograft function with an estimated GFR of 65 ml/min/1.73 m2 . CONCLUSION According to our experience, explantation of the kidney allograft, followed by irrigation with thrombolytics in bench surgery, and reimplantation resulted in unexpected optimal outcomes in the case of allograft venous thrombosis.

Introduction: Cholecysto-choledocolithiasis is a rare entity in children and its management is still challenging and controversial. The laparoendoscopic rendezvous (LERV) procedure, consisting of laparoscopic cholecystectomy and simultaneous endoscopic retrograde cholangiopancreatography for the management of symptomatic choledocholithiasis is well described in adult patients. However, in the literature, few reports about its application in the pediatric population have been recorded. Aim of the Study: The aim of the present study is to report our first successful cases of symptomatic cholecysto-choledocholithiasis LERV treatment. Methods: Two girls suffering of hemolytic disease presented to our third referral center with acute abdominal pain due to cholecysto-choledocholithiasis. Preoperative, perioperative, and postoperative data were retrospectively reviewed. Results: Surgery was performed without complications. The girls were dismissed once re-alimentation and re-canalization were achieved and had no other episodes of biliary cholic. Reported advantages of LERV include: a shorter in-hospital stay, a reduction in the number of procedures and anesthesia, and a reduced overall risk of complications. Conclusions: The promising result with our 2 cases suggest that, when performed in highly specialized centers, LERV is a safe procedure, which leads to considerable benefits, despite logistic and organizational difficulties.

Antenatal factors play a role in NEC pathogenesis. This study aimed to investigate the predictive value of fetal ductus venosus doppler (DV) for NEC in fetal growth restriction fetuses (FGRF) and to assess the predictive accuracy of IG21 and Fenton curves in NEC development. Data from FGRF, postnatal findings, and Doppler characteristics were collected between 2010 and 2020 at a single center. Patients were then divided into two groups (i.e., with and without NEC). Bivariate and multivariate analyses were performed. We identified 24 cases and 30 controls. Absent or reversed end-diastolic flow (AREDF) and increased resistance in the DV were more impaired in cases (p < 0.05). Although the median birthweight was not different, the Fenton z-score was lower in NEC (p < 0.05). Fetal cardiopulmonary resuscitation, synchronized intermittent mandatory ventilation, neonatal respiratory distress, persistent patent ductus arteriosus (PDA), and inotropic support were more frequent in the NEC group. Furthermore, NEC patients had lower white blood cells (WBC) (p < 0.05). The predictive model for NEC (model 4), including Fenton z-score, WBC, PDA, and DV had an AUC of 84%. Fetal Doppler findings proved effective in predicting NEC in FGR. The Fenton z-score was the most predictive factor considering the fetal growth assessment showing high sensitivity.

Introduction: Splenopexy has been proposed as the treatment of choice in case of wandering spleen (WS). We report our experience and review the current literature focusing on surgical management an...

Extraperitoneal approach is sometimes recommended for kidney transplantation (KT) in children weighting15 kg. We hypothesized that this approach might be as successful as in patients with normal weight. Data of all consecutive KTs performed between 2013 and 2019 were retrospectively reviewed. Early outcomes and surgical complications were compared between children weighing ≤15 kg (low-weight (LW) group) and those weighing15 kg (Normal-weight (NW) group). All the 108 KTs were performed through an extraperitoneal approach. The LW group included 31 patients (mean age 3.5 ± 1.4 years), whose mean weight was 11.1 ± 2.0 kg. In the LW group,-a primary graft nonfunction (PNGF) occurred in one patient (3.2%), surgical complications occurred in nine (29%), with four venous thrombosis. In the NW group, PNGF occurred in one case (1.3%), delayed graft function (DGF) in eight (10%), surgical complications in 11 (14%) with only one case of venous thrombosis. In both groups, no need for patch during wound closure and no wound dehiscence were reported. The extraperitoneal approach can be effectively used in LW children. No differences were observed in the overall complication rate (P = 0.10), except for the occurrence of venous thrombosis (P = 0.02). This might be related to patients' characteristics of the LW group.

Necrotizing enterocolitis (NEC), the first cause of short bowel syndrome (SBS) in the neonate, is a serious neonatal gastrointestinal disease with an incidence of up to 11% in preterm newborns less than 1500 g of birth weight. The rate of severe NEC requiring surgery remains high, and it is estimated between 20–50%. Newborns who develop SBS need prolonged parenteral nutrition (PN), experience nutrient deficiency, failure to thrive and are at risk of neurodevelopmental impairment. Prevention of NEC is therefore mandatory to avoid SBS and its associated morbidities. In this regard, nutritional practices seem to play a key role in early life. Individualized medical and surgical therapies, as well as intestinal rehabilitation programs, are fundamental in the achievement of enteral autonomy in infants with acquired SBS. In this descriptive review, we describe the most recent evidence on nutritional practices to prevent NEC, the available tools to early detect it, the surgical management to limit bowel resection and the best nutrition to sustain growth and intestinal function.

Background/Purpose The anterior anus (AA) is a condition, almost exclusively present in females, in which the anus is located abnormally anterior along the perineal body, well separated from the vulva. Definition and treatment are still controversial. This study aimed to evaluate the medium-long term follow up of patients with AA conservatively managed, considering the gynaecologic aspects in post-menarchal girls. Matherials/Methods This cross-sectional study includes AA patients older than 3 years at time of the study, followed in two referral centres for ARM between January 2000 and May 2017. The API (Anal Position Index) was applied to define AA. A questionnaire regarding the ano-rectal function, occurrence of urinary infection (UTI), familiarity for ARM/AA was administered to parents and patients. Gynecological examination was performed in post-menarchal patients. Results Fifty-three patients (all females) were retrieved. Three were excluded (2 underwent surgery at another center, 1 was lost at follow-up), 7/50 had major malformations (2 oesophageal atresia, 4 cardiovascular malformations and 1 with Fallot, uretheral duplicity and vertebral anomalies), 5/50 had familiarity for ARM/AA. Only 10% were constipated. Fifteen patients underwent gynecological examination. Their mean API was 0.278 +/- 0.013 DS, they had good buttock tropism, normal resident bacteria, and no UTI. Conclusion AA patients in our centers do not undergo any kind of surgery. At least three quarters of them have a perfectly normal bowel habits and adolescents do not present symptoms related to their condition. These results support the conservative management of this condition.

Gastrostomy placement is crucial in neurologically impaired (NI) children to ensure an adequate food intake and a safe route for drugs administration and to reduce the risk of primary aspiration. NI patents are more prone to gastroesophageal reflux. The association with fundoplication is deemed to reduce reflux-related respiratory complications. However, long-term benefits of this approach are not clear. We therefore aimed to compare long-term reflux-related respiratory complications of gastrostomy only (GO) to gastrostomy with fundoplication (GF). We retrospectively reviewed 145 consecutive NI children managed from 2008 to 2018. As long-term outcomes, we analyzed number and length of hospital admissions (Reflux-Related-Hospitalization, RRH) and emergency department accesses (Reflux-Related-Accesses, RRA) due to respiratory problems. Results were analyzed with appropriate statistical method. Median age at referral and at gastrostomy placement were 2.2 and 3.4 years (SD 5.6), respectively. Median follow-up was four years (range 1&ndash, 12). Anti-reflux procedures were performed in 26/145 patients (18%), tracheotomy in 23/145 (16%). RRH following surgery showed lower number of admissions/year (0.32 vs. 1 for GO vs. GF, p &lt, 0.005) and days hospitalization/year (3 vs. 13, p = 0.08) in GO compared to GF, RRA was similar (0.60 vs. 0.65, p = 0.43). Gastrostomy placement alone appeared not to be inferior to gastrostomy plus fundoplication with respect to long-term respiratory-related outcomes for NI children in our center.

Background Hirschsprung’s disease (HSCR) is a serious congenital bowel disorder with a prevalence of 1/5000. Currently, there is a lack of systematically developed guidelines to assist clinical decision-making regarding diagnostics and management. Aims This guideline aims to cover the diagnostics and management of rectosigmoid HSCR up to adulthood. It aims to describe the preferred approach of ERNICA, the European Reference Network for rare inherited and congenital digestive disorders. Methods Recommendations within key topics covering the care pathway for rectosigmoid HSCR were developed by an international workgroup of experts from 8 European countries within ERNICA European Reference Network from the disciplines of surgery, medicine, histopathology, microbiology, genetics, and patient organization representatives. Recommendation statements were based on a comprehensive review of the available literature and expert consensus. AGREE II and GRADE approaches were used during development. Evidence levels and levels of agreement are noted. Results Thirty-three statements within 9 key areas were generated. Most recommendations were based on expert opinion. Conclusion In rare or low-prevalence diseases such as HSCR, there remains limited availability of high-quality clinical evidence. Consensus-based guidelines for care are presented.

This particular case highlights the importance of a multidisciplinary team expertise in the differential diagnostic process of acute abdominal pain. The case involves a 10-year old Chinese boy who presented with intermittent abdominal pain; the clinical and radiologic picture narrowed the differential diagnosis between an acute appendicitis and an intestinal lymphoma. Diagnosis of a high grade - B lymphoma was made by performing a colonoscopy; this procedure was deemed the best option to obtain a quick diagnosis with low invasiveness.

Aim:Male patients with anorectal malformations (ARM) are classified according to presence and level of the recto-urinary fistula. This is traditionally established by a preoperative high-pressure distal colostogram that may be variably interpreted by different surgeons. The aim of this study was to evaluate the inter- and intraobserver variation in the assessment by pediatric surgeons of preoperative colostograms with respect to the level of the recto-urinary fistula. Materials and Methods:Sixteen pediatric surgeons from 14 European centers belonging to the ARM-Net Consortium twice scored 130 images of distal colostograms taken in sagittal projection at a median age of 66 days of life (range: 4-1,106 days). Surgeons were asked to classify the fistula in bulbar, prostatic, bladder-neck, no fistula, and "unclear anatomy" example. Their assessments were compared with the intraoperative findings (kappa) for two scoring rounds with an interval of 6 months (intraobserver variation). Agreement among the surgeons' scores (interobserver variation) was also calculated using Krippendorff's alpha. A kappa over 0.75 is considered excellent, between 0.40 and 0.75 fair to good, and below 0.40 poor. Surgeons were asked to score the images in "poor" and "good" quality and to provide their years of experience in ARM treatment. Results:Agreement between the image-based rating of surgeons and the intraoperative findings ranges from 0.06 to 0.45 (mean 0.31). Interobserver variation is higher (Krippendorff's alpha between 0.40 and 0.45). Years of experience in ARM treatment does not seem to influence the scoring. The mean intraobserver variation between the two rounds is 0.64. Overall, the quality of the images is considered poor. Images categorized as having a good quality result in a statistically significant higher kappa (mean: 0.36 and 0.37 in the first and second round, respectively) than in the group of bad-quality images (mean: 0.25 and 0.23, respectively). Conclusions:There is poor agreement among experienced pediatric colorectal surgeons on preoperative colostograms. Techniques and analyses of images need to be improved in order to generate a homogeneous series of patients and make comparison of outcomes reliable.

Objective To evaluate the efficacy of the screening protocol and the clinical follow-up to detect urological anomalies (UA) in patients with ARM (ARM-P) in our institution. Secondary aim was to define the prevalence of UA and their relationship with severity of ARM. Methods ARM-P were selected from a prospectively maintained database from 2000 to 2016. Exclusion criteria were: incomplete or with less than 3 years of follow-up and absence of surgical correction of the anorectal anomalies. Data from urological screening and follow-up were collected. Patients were divided into complex malformations (Group 1) and less complex malformations (Group 2). Results One hundred seventeen of 149 were included in the study period (62 group 1, 55 group 2). UA were detected in 36/117 at birth (30.7%) with a difference between groups (P = .0005). VUR was detected in 16 (6 with hydronephrosis, 10 with normal ultrasound at birth). A bladder ultrasound after potty training showed 18 lower urinary tract dysfunction (LUTD; 15 with UA detected at birth, 3 with normal ultrasound). 8 LUTD were found during clinical follow-up, confirmed by ultrasound. Spinal MRI detected spinal cord anomalies (SCA) in 52/117 (44.4%). Considering the subgroups with neurogenic bladder and SCA there was a difference between groups (13/33 vs 1/19). Conclusion This study suggests that ARM-P are at increased risk of UA, most of which were detected on neonatal ultrasound. While screening protocol can show 88% of UA, follow-up can detect 12.9 % of total abnormalities without difference between groups. This data has to be considered when planning follow-up for these patients.

Background: Tunneled central venous catheters (CVC) are crucial in the management of children affected by short bowel syndrome (SBS). This work aims to investigate the outcomes of tunneled CVC and to identify factors influencing their survival. Methods: All the children diagnosed with SBS and undergone a procedure of insertion of a tunneled CVC from 2010 to 2019 were included. Demographic data and surgical information about the procedures were collected. Regression models and Kaplan–Meier analysis were performed to estimate the survival. Results: Eighteen patients, eight males (44%), with a median length of residual bowel measuring 72 cm (IQR 50–102 cm), were enrolled. Thirty-nine Broviac CVCs were inserted with a mean number of 2.2 CVCs per patient and 13365 line-days. The overall incidence of complications was 3.2/1000 line-days, and the incidence of central line associated bloodstream infections (CLABSI) was 1.1/1000 line-days. No episode of catheter thrombosis was reported. The median survival was 269 days (IQR 82–1814 days). The survival was negatively influenced by a younger age at insertion ( R2 = 0.29; p Conclusion: CVC-related complications negatively influenced the survival of the line. An elder age at insertion together with a larger CVC diameter increased the survival of the line, while a shorter residual bowel was associated with an anticipated removal due to complications.

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. 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). 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

Ventriculopleural shunt is still considered a third-line option for CSF diversion, when both peritoneal and atrial cavity are contraindicated. Different approaches have been used and in modern surgery, lesser invasive techniques are predominant. The goal of this manuscript is to present a minimally invasive placement of a pleural catheter. We describe a minimally invasive approach to the pleural space using an a-traumatic peel-away introducer under ultrasonographic intraoperative control. Furthermore, consideration about complications, follow-up and advantages of the abovementioned technique will be discussed. Percutaneous US guided placement for pleural catheter is a safer and modern minimally invasive approach to the pleural space. Pleural effusion is the predominant complication, encountered especially in younger children.

Neck swelling is a common condition that brings pediatric patient to the clinician office. However, congenital malformations of the neck, leading to surgical treatment, are rarely diagnosed in the neonatal age. The present chapter lists the most common surgical condition treated by pediatric surgeons and some of their peculiar aspects when encountered early in life. Thyroglossal cyst and branchial remnants are the main topic of this chapter.

Neonatal appendicitis carries a high mortality rate. We describe a peculiar case presented after an incarcerated hernia manually reduced in a 4-week-old male neonate with ipsilateral undescended testis. Laparoscopy allowed a prompt recognition of an unexpected intra-abdominal life-threatening condition. Pathogenesis, treatment, and outcome are discussed on the light of a comprehensive literature review.

The use of the Da Vinci Xi system is gaining popularity among all surgical disciplines. A splenectomy is a treatment option for patients with hematological disorders and splenic lesions. The laparoscopic approach is nowadays the standard of care. Despite the initial controversy, recently it has been demonstrated the superiority of robotic splenectomy performed in "difficult" cases. We report, to our knowledge, the first case of robot-assisted splenectomy following embolization of the splenic artery in a 15-year-old patient with chronic immune thrombocytopenia, worsened by a severe cerebral sinus thrombosis, while being treated with eltrombopag and mycophenolate. Due to the need for a rapid rise in platelet counts and failure of several medical treatments, splenectomy was advocated. To raise the platelet count pre-operatively and minimize intraoperative bleeding, the embolization of the spleen artery was performed before the planned splenectomy. The intervention was carried on without any complication and at 1 year follow up the patient is in good clinical condition and has improved his neurological condition. We propose a robotic splenectomy following embolization of the splenic artery as a feasible and safe procedure. The advantages of the Da Vinci Xi system are highlighted especially in complex cases, requiring maximum precision.

Purpose Necrotizing enterocolitis (NEC) is a severe neonatal disease. The present study aimed to identify factors predisposing the development of severe forms of NEC. Methods This retrospective study examined NEC patients in a single centre between 2002 and 2015. Data concerning clinical characteristics, therapeutic management as well as short-term outcomes were collected. We compared the patients receiving successful medical treatment and those requiring surgical intervention. Patients who underwent surgery were distinguished in three subcategories. Bivariate and multivariate analyses were used for the statistical analysis. Results We identified 155 patients in the study period. 102 were treated conservatively and 53 required surgery. 8 received a primary peritoneal drainage, 31 received a drainage and a subsequent laparotomy and 14 received a laparotomy. Multivariate regression analysis identified a lower risk for surgery with a later onset and higher serum pH values, whereas an increased risk with higher C reactive Protein (CRP) levels at the onset. Pneumatosis intestinalis was identified as a protective factor. Overall mortality was 6.4%, with higher percentage in surgical NEC. Conclusion This study suggests that a later onset is a protective sign for the progression to surgery, whereas lower pH values and higher CRP levels are prognostic factors associated with the need for surgery. The line of treatment involving explorative laparotomy in case of perforation seems to be rewarded by low morbidity and mortality rate.

Fecal incontinence (FI) is a major social and economic burden. Adults suffering from post-surgery consequences or trauma and children affected by congenital anorectal malformations are the main categories of patients suffering from FI due to defective sphincter complex. Current therapeutic options for FI are often only partially effective, require lifelong care and are resource consuming. Besides, in both congenital and acquired anal sphincter defects, muscle damage is often irregular, leading to a diffuse derangement not suitable for surgical repair. In this context, regenerative medicine offers an increasing number of theoretically suitable solutions to restore sphincter structures, but only a few preclinical studies have been published regarding the use of cell therapy, biomaterials or tissue engineered structures for FI application. Here we present, for the first time, the generation of an in vitro engineered muscular sphincter using a population of adult human perivascular stem cells (pericytes) and 3D bioprinting. 3D bioprinting is an emerging technology for fabricating artificial tissue and organ constructs, which lends itself perfectly to the design of muscle constructs for anal sphincter repair. In our approach, 3D functional constructs have been created by simultaneously depositing cells and supporting material in a spatially defined, layer-by-layer stacking organization, generating a biomimetic spatial arrangement. Specifically, we developed a 3D myo-structure starting from human muscle derived pericytes, perivascular stem cells able to undergo robust myogenesis while also guaranteeing a rapid vascularization supporting and recruiting new blood vessels. The results presented here represent a pre-clinic study that strongly supports the feasibility of this innovative approach to treat the forms of fecal incontinence that are unresponsive to conservative treatments.

Introduction Impossibility to place a gastrostomy and failed gastroesophageal reflux surgery with unsafe swallow are the main indications to Feeding Jejunostomy (FJ) in children. The aim of this study is to quantify the incidence of complications associated with FJ. Materials and Methods A retrospective review of patients who had surgically inserted FJ between January 2009 and August 2013 at our institution was conducted. Data were obtained from medical records, operative notes, and radiology database, focusing on complications. Results A total of 19 patients, average age 39.6 months (3–168 months), were treated during the study period. Indications to FJ were gastroesophageal reflux disease (GERD) associated with unsafe swallow in 12, esophageal atresia in 5, and foregut dysmotility in 2. Seventeen FJ were inserted via laparotomy and 2 were laparoscopically assisted. In all cases, a serosal tunnel on the antimesenteric border was fashioned. No intraoperative complications were recorded. Tube dislodgement/blockage occurred on an average of 0.48 times per month in 18 out of 19 patients. The average radiation dose received for tube reinsertion/manipulation was 3.316 mSv/year/patient (0–10.66). Major postoperative complications occurred in 7 out of 19. After an average follow-up of 21 months, two have abandoned the use of FJ due to poor tolerance and three have fully weaned off. Two patients died due to unrelated causes. Conclusion FJ, as an alternative means for enteral feeding, may require multiple readmissions and exposure to radiological procedures. The high risk of severe complications should be considered when offering this procedure.

Midgut volvulus in infants may lead to extreme short bowel syndrome. Strategies to avoid post-ischemia bowel loss have been proposed, involving thrombolysis prior to a definitive surgical treatment. Haemorrhagic risks in these patients may withhold from this approach. Herein we describe the use of urokinase systemic infusion after an unsatisfactory second look laparotomy, performed in a term baby with midgut volvulus. Continuous infusion of urokinase was given for seventeen hours prior to a third laparotomy. A total of 25 cm of small bowel were finally kept in place. Twelve months after the 1st laparotomy the patient was on oral feeding 125 calories/Kg/day. This case showed the possible rescue role of a fibrinolytic agent against persistent ischemia after the second look laparotomy for neonatal volvulus.

Background Mini-invasive techniques for gastroesophageal reflux disease (GERD), such as endoscopic injections of inert materials, have been introduced in recent years. However, results are still preliminary. Cell injection has emerged as an alternative strategy in both vesicoureteral reflux and incontinence. Here we report, for the first time, the injection of muscle precursor cells (MPCs) in the gastroesophageal junction (GEJ). Materials and methods MPCs were derived from expanded satellite cells isolated from skeletal muscle fibers of green fluorescent protein (GFP) positive mice. Via laparotomy, GFP-negative mice were subjected to cryoinjury of GEJ followed by injection of MPCs (experimental animals), bone marrow derived cells, or saline (controls). Results Immunofluorescence analyses of experimental GEJs demonstrated coexpression of GFP and desmin in grafted cells. GFP+ muscle neofibers were evident at 4 wk after injection. Coexpression of GFP and smooth muscle actin was also observed at 2 wk. Conclusions Satellite cells could be easily harvested, expanded in culture, and used as injectable substance in the GEJ. These results could be the background for the development of a new injection technique for GERD treatment, which might combine bulging and functional actions.

AIM It has been demonstrated that in a neonatal rat model of necrotizing enterocolitis (NEC), amniotic fluid stem (AFS) cells decrease intestinal damage and improve survival via modulation of stromal cells expressing cyclooxygenase 2 in the lamina propria. Herein, we aimed to evaluate the effect of AFS cells on body weight and fluid retention in this NEC model. Methods AFS cells were obtained from green fluorescent protein (GFP) + pregnant rats at E16 and expanded in culture. A total of 185 neonatal rats had NEC induced by gavage feeding of hypertonic formula + hypoxia + oral lipopolysaccharide (4 mg/kg/d) and were randomized to intraperitoneal phosphate buffered saline (PBS, n = 93) or AFS cells (n = 92). A total of 36 breastfed (BF) rats were used as controls. All rats were being killed at 96 hours of life. Groups were compared for body weight and presence of free intraperitoneal fluid using nonparametric and contingency tests. Data are expressed as mean ± standard deviation. RESULTS There were no differences in birth weight among the groups (PBS = 5.6 ± 0. 3 g; AFS cells = 5.6 ± 0. 3 g; BF = 5.6 ± 0. 3 g; p = 1). The body weight at randomization was not different between PBS (5.61 ± 0. 5 g) and AFS cells (5.60 ± 0. 5; p = 1) rats. After the rats were killed, BF rats were significantly heavier (12.5 ± 0.1 g) than PBS (5.12 ± 0.4 g) and AFS cell rats (4.95 ± 0.3; p < 0.0001). From randomization to being killed, PBS rats had 9% of weight loss in comparison with 12% in AFS cell rats (p = 0.08). After the rats were killed, 42 (45%) PBS rats developed ascites with evident abdominal distension in comparison with 19 (21%) AFS cells (p = 0.0005). None of BF animals had ascites. CONCLUSION Gavage feeding and undernutrition severely affect growth in this model of NEC. Administration of AFS cells result in lower incidence of ascites than in PBS rats. This could explain the differences in body weight between the two groups of rats that were killed. Furthermore, studies on liver function and fluid composition are needed to investigate our speculation.