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In the pediatric population, thoracic surgery is performed to treat a great variety of pathologies. The wide age range and clinical conditions of the pediatric patients require particular anesthetic considerations and management strategies for each case.

Hirschsprung disease is a congenital disease characterized by intestinal aganglionosis of various extents. Most patients are younger than 1 year of age. Though, a minority of cases can be older or even adult. Older the patient the more difficult and prolonged is the endorectal dissection required for the pull-through procedure. Longer surgery leads to longer anal dilatation and trauma with subsequent higher likelihood of continence impairment. The article aims at describing the first case series of robot-assisted Soave procedure, which was adopted as an alternative minimally invasive approach to older patients with Hirschsprung disease. The technical principles are represented by intraoperative seromuscolar leveling biopsies, intracorporeal endorectal cranial dissection, and endorectal pull-through with colo-anal anastomosis. The authors report three procedures that were carried on without complication with a limited requirement for anal dilatation and trauma, given the reduced need for endorectal caudal dissection. The results demonstrate the feasibility of the proposed approach. The robotic approach should be considered as an alternative minimally invasive approach for older children or adults with Hirschsprung disease.

Introduction: Neonates with D-transposition of the great arteries (dTGA) may die at birth because of the inadequate intracardiac mixing due to a misdiagnosed restrictive foramen ovale. We reviewed our experience in echocardiographic assessment and perinatal management of fetuses with dTGA searching for new features that may predict the need for urgent balloon atrial septostomy (BAS) immediately after birth. Patients and Methods: We included fetuses diagnosed with dTGA between January 2000 and December 2014. We assessed pre- and postnatal appearance of the foramen ovale, ductus arteriosus and pulmonary veins. Both the diagnostic findings at the time of last prenatal echocardiogram and those findings deriving from a retrospective reevaluation of stored videos were considered. BAS was defined as urgent if performed in neonates with restrictive foramen ovale and severe hypoxemia. Results: We reviewed 40 fetuses with dTGA. 20/40 fetuses received urgent BAS at birth. Not only the restrictive but also the hypermobile and the redundant appearance of the foramen ovale was significantly associated with urgent BAS (p < 0.0001, p = 0.002 and p = 0.0001, respectively). Conclusions: Prenatal evaluation of the foramen ovale appearance in fetuses with dTGA is still challenging. Based on our experience, also the redundant foramen ovale appearance may need urgent BAS at birth.

Background Ultrasound-guided (USG) cannulation of the brachiocephalic vein (BCV) is gaining worldwide consensus for central venous access in children. This study reports a 20-month experience with this approach in children. Methods All patients who underwent percutaneous USG central venous catheter (CVC) positioning in the BCV between August 2013 and March 2015 have been included. Devices inserted during this period were open-ended, either single or double-lumen tunneled CVC. Our series was divided into three consecutive study periods in order to determine the relative incidence of repositioning and complications. Results During the study period, a total of 95 patients underwent 109 CVC insertions in the BCV. The median length of CVC duration was 230 days for a total of 23,212 catheter days. No major intraoperative complications occurred. Overall rate of CVC-related postoperative complications requiring repositioning or precocious removal was 0.90 per 1,000 catheter days and involved 21 CVC (19%, 95% confidence interval 13-28). These included 18 dislodgments, two infections, and one malfunction. Double-lumen CVCs represented the only significant risk factor for complications (52% complications-three per 1,000 catheter days). Conclusion USG supraclavicular cannulation of the BCV represents a safe approach for central line placement in children. It proved to be versatile, as it can be used in premature infants as well as in adolescents. Provided it is adopted by operators experienced in USG cannulation, we strongly suggest to resort to this approach as a first-line choice in children undergoing tunnelled central line placement for long-lasting therapy.

Anal fistula is a common acquired anorectal disorder in children. Treatment methods that have been used are associated with inconsistent results and possible serious complications. In 2011 a minimally invasive approach, video-assisted anal fistula treatment (VAAFT) was described for adult patients. The aim of the present study was to assess the first series of pediatric patients treated with VAAFT.All patients who underwent VAAFT between August 2013 and May 2015 were included. Demographics, clinical features, preoperative imaging, surgical details, outcome, and medium-term data were prospectively collected for each patient.Thirteen procedures were performed in nine patients. The male to female ratio was 8:1, and the median age was 9.6 years. Five fistulas were idiopathic, three iatrogenic, and one associated with Crohn's disease. Eight complete VAAFT procedures were performed. The remaining five procedures were either fistuloscopy and cutting seton placement or fistuloscopy and electrocoagulation, both without mucosal sleeve. The median length of surgery was 41 min. The median hospital stay was 24 h, and the median length of follow-up was 10 months. Resolution of the fistula was observed in all patients who underwent a complete VAAFT. In four out of five patients who underwent an incomplete procedure (without mucosal sleeve), the fistula recurred. No incontinence or soiling was reported in the medium term.VAAFT proved to be feasible and safe in children. It also proved to be versatile as it could be applied to fistulas of different etiologies. The key to success seems to be an adequate mucosal sleeve. Older children and adolescents benefit most from VAAFT which is a valid alternative to available surgical procedures.

Background:Postoperative apnea is a complication in young infants. Awake regional anesthesia (RA) may reduce the risk; however, the evidence is weak. The General Anesthesia compared to Spinal anesthesia study is a randomized, controlled trial designed to assess the influence of general anesthesia (GA) on neurodevelopment. A secondary aim is to compare rates of apnea after anesthesia.Methods:Infants aged 60 weeks or younger, postmenstrual age scheduled for inguinal herniorrhaphy, were randomized to RA or GA. Exclusion criteria included risk factors for adverse neurodevelopmental outcome and infants born less than 26 weeks gestation. The primary outcome of this analysis was any observed apnea up to 12 h postoperatively. Apnea assessment was unblinded.Results:Three hundred sixty-three patients were assigned to RA and 359 to GA. Overall, the incidence of apnea (0 to 12 h) was similar between arms (3% in RA and 4% in GA arms; odds ratio [OR], 0.63; 95% CI, 0.31 to 1.30, P = 0.2133); however, the incidence of early apnea (0 to 30 min) was lower in the RA arm (1 vs. 3%; OR, 0.20; 95% CI, 0.05 to 0.91; P = 0.0367). The incidence of late apnea (30 min to 12 h) was 2% in both RA and GA arms (OR, 1.17; 95% CI, 0.41 to 3.33; P = 0.7688). The strongest predictor of apnea was prematurity (OR, 21.87; 95% CI, 4.38 to 109.24), and 96% of infants with apnea were premature.Conclusions:RA in infants undergoing inguinal herniorrhaphy reduces apnea in the early postoperative period. Cardiorespiratory monitoring should be used for all ex-premature infants.

ObjectiveTo report cross-sectional clinical findings in a large cohort of patients affected by type 1 spinal muscular atrophy.MethodsWe included 122 patients, of age ranging between 3 months and 22 years, 1 month. More than 70% (85/122) were older than 2 years and 25% (31/122) older than 10 years. Patients were classified according to the severity of phenotype and to the number of SMN2 copies.ResultsPatients with the more common and the most severe phenotype older than 2 years were, with few exceptions, on noninvasive ventilation and, with increasing age, more often had tracheostomy or >16-hour ventilation and a gastrostomy inserted. In contrast, 25 of the 28 patients with the mildest phenotype older than 2 years had no need for tracheostomy or other ventilatory or nutritional support. In patients older than 2 years, the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders scores were generally lower compared to those found in younger patients and showed distinct levels of functional abilities according to the severity of the phenotype. Similar findings were also observed on the Hammersmith Infant Neurological Examination.ConclusionsOur findings confirm that, after the age of 2 years, patients with type 1 spinal muscular atrophy generally survive only if they have gastrostomy and tracheostomy or noninvasive ventilation >16 hours and have low scores on the functional scales. More variability, however, can be expected in those with the mildest phenotype, who achieve head control. These data provide important baseline information at the time treatments are becoming available.

There is a lack of information about evaluation of pediatric minimal access surgery complications; the Clavien-Dindo classification was never used for a large series of laparoscopic and thoracoscopic pediatric procedures. With a prospective Morbidity and Mortality database, all the minimal access surgical procedures carried out between 2012 and 2016 were included in this study. Statistical analyses were used to valuate modification of surgical techniques in to 2 periods (period 1: January 2012 to February 2014; period 2: February 2014 to February 2016). A total of 1374 minimal access procedures were performed on 1371 patients. The overall complication rate was 2.9%. No differences, in terms of complications, were observed between elective and emergency procedures (P=0.3). There was a significant difference between the complication rate of thoracoscopic surgery (P=0.027). These results provide the relevance of adequate recording system and standardized classification for analyses and reduction of complications for pediatric minimal access procedures.

Summary Background The use of isotonic electrolytic solutions for the intraoperative fluid management in children is largely recognized, but the exact composition still needs to be defined. Objectives The primary objective of this randomized controlled open trial was to compare the changes in chloride plasma concentration using two intraoperative isotonic fluid regimens (Sterofundin vs normal saline, both added with 1% of glucose) in children undergoing major surgery. Secondary objectives were to compare changes in other electrolytes, renal function, and the occurrence of hypoglycemia. Methods Children aged between 1 and 36 months, scheduled for major surgery, were randomized to receive Sterofundin or saline during the intraoperative time. Children with preoperative electrolyte abnormalities, hemodynamic instability, and severe renal or hepatic dysfunction were excluded. The primary outcome was the Δ of Cl− (Δ = change in plasma concentration between post- and pre-infusion), and secondary outcomes included Δ of other electrolytes and intraoperative hypoglycemia. Results A total of 240 patients were included in the two study sites and randomized to receive Sterofundin plus 1% glucose or normal saline plus 1% glucose, in a open fashion (229 were finally analyzed). Δ of Cl− and Mg++ was statistically less relevant in patients who received intraoperative Sterofundin, and Δ of the other electrolytes was comparable between the two study groups. Relative risk of hyperchloremia was significantly higher when large volumes were infused (over than 46.7 ml·kg−1), regardless of type of crystalloid infused. Hypoglycemia occurred in two of 229 patients. Conclusions Sterofundin is safer than normal saline in protecting young children undergoing major surgery against the risk of increasing plasma chlorides and the subsequent metabolic acidosis.

A 9-month-old boy with life-threatening multiresistant pure red cell anemia/autoimmune hemolytic anemia within the frame of a possible, undiagnosed immune-mediated disease was initially treated with prednisone. Further-line therapies of the following 7 relapses included immunoglobulins, rituximab, cyclophosphamide, and alentuzumab followed by other maintenance treatments as cyclosporine, methotrexate, and mycophenolate. After all the administered therapies failed, the patient was successfully treated by splenectomy followed by fludarabine and then sirolimus as maintenance treatment. Relapses might have been caused by the lack of a complete debulking of triggering cells and/or ineffective maintenance therapy. Splenectomy and sirolimus may have played a complementary role in the management of both situations.

Video-assisted surgery was born at the beginning of the century and more widely diffused from the 1960s, principally to respond to the need to perform surgeries applying less invasive methodologies. In the following years, thanks to increasingly technologically advanced equipment, there has been an expansion of clinical indications, until you get to its daily use in paediatrics, in children as infants.

Background Little is known about the incidence of severe critical events in children undergoing general anaesthesia in Europe. We aimed to identify the incidence, nature, and outcome of severe critical events in children undergoing anaesthesia, and the associated potential risk factors. Methods The APRICOT study was a prospective observational multicentre cohort study of children from birth to 15 years of age undergoing elective or urgent anaesthesia for diagnostic or surgical procedures. Children were eligible for inclusion during a 2-week period determined prospectively by each centre. There were 261 participating centres across 33 European countries. The primary endpoint was the occurence of perioperative severe critical events requiring immediate intervention. A severe critical event was defined as the occurrence of respiratory, cardiac, allergic, or neurological complications requiring immediate intervention and that led (or could have led) to major disability or death. This study is registered with ClinicalTrials.gov, number NCT01878760. Findings Between April 1, 2014, and Jan 31, 2015, 31 127 anaesthetic procedures in 30 874 children with a mean age of 6·35 years (SD 4·50) were included. The incidence of perioperative severe critical events was 5·2% (95% CI 5·0–5·5) with an incidence of respiratory critical events of 3·1% (2·9–3·3). Cardiovascular instability occurred in 1·9% (1·7–2·1), with an immediate poor outcome in 5·4% (3·7–7·5) of these cases. The all-cause 30-day in-hospital mortality rate was 10 in 10 000. This was independent of type of anaesthesia. Age (relative risk 0·88, 95% CI 0·86–0·90; p

Background: Esophageal achalasia is not a frequent disorder in children, and different treatments have been proposed during the past decades. This study reviews the results of the laparoscopic Heller-Dor procedure performed in pediatric patients. Methods: We included the patients operated in the period 1994–2009. A single longitudinal anterior esophageal myotomy (Heller) and a 180° anterior gastropexy (Dor) were laparoscopically performed. The patients were checked to detect intra- or postoperative complications and recurrence. Results: 20 children were operated on. Conclusions: As complication and recurrence rates are very low, we consider modified Heller myotomy and Dor gastropexy through a laparoscopic approach our first choice to treat esophageal achalasia in the pediatric population.

Introduction The potential drawbacks of tunneled-cuffed catheters are complications such as local or systemic infection, dislodgment, rupture, malfunction, and deep venous thrombosis. Aim of this study is to describe the incidence of complications, focusing on dislodgment and on the role of new securement devices in reducing this annoying issue. Methods We enrolled all pediatric patients with tunneled-cuffed central venous catheters (CVCs) inserted at the Giannina Gaslini Institute during a 16-month period. Demographic data, technical details, intraoperative and postoperative complications were recorded and stored in a digital database according to Data Protection Act. Results During the study period, we collected 173 tunneled-cuffed CVCs. All but three insertions were successful. There were 50 complications involving 47 CVCs. Complications included 13 infections, 27 dislodgments, 4 thromboses, 3 obstructions, and 3 malfunctions/breaking. In 51 of 173 CVCs, we used subcutaneously anchored securement device (SAS). Conclusions The use of SAS proved to significantly reduce the incidence of complications in pediatric patients, particularly during the first 30 postoperative days. Basing on our results we suggest to routinely adopt this new securement device for high-risk CVC.

Aim This study aims to evaluate the experience gained with video-assisted needle-core biopsy in patients affected by neuroblastoma (NB). Patient and Methods We retrospectively reviewed all the patients presenting at our center with a thoracic, abdominal, and/or pelvic NB who underwent biopsy between 2007 and 2014. Data on demographics, localization, and size of the tumor, image-defined risk factors involved in each case, technical details about biopsies performed, qualitative and quantitative adequacy of tumor sampling and histological diagnosis, postoperative details, and complications were recorded and analyzed. Results During the 7 years of our study 51 patients affected by NB underwent 55 biopsies. Our results focus on the 29 patients undergoing 32 video-assisted needle-core biopsies. The median age was 4 years with a median weight of 13.5 kg. Out of 29, 28 tumors were localized in the abdomen/pelvis compartment, whereas 1 patient presented with a thoracic mass. The median size of the tumors was 57 mm. A total of 28 patients had an adequate tissue sampling for complete tumor characterization. Biopsies were repeated twice in a patient. Three complications occurred in three patients. Conclusions The video-assisted needle-core biopsy combines minimally invasive surgery several advantages with the possibility to obtain multiple samples in different regions with minimal tumor exposition and low complication rate.

Neonates with D-transposition of the great arteries (dTGA) may die at birth because of the inadequate intracardiac mixing due to a misdiagnosed restrictive foramen ovale. We reviewed our experience in echocardiographic assessment and perinatal management of fetuses with dTGA searching for new features that may predict the need for urgent balloon atrial septostomy (BAS) immediately after birth.We included fetuses diagnosed with dTGA between January 2000 and December 2014. We assessed pre- and postnatal appearance of the foramen ovale, ductus arteriosus and pulmonary veins. Both the diagnostic findings at the time of last prenatal echocardiogram and those findings deriving from a retrospective reevaluation of stored videos were considered. BAS was defined as urgent if performed in neonates with restrictive foramen ovale and severe hypoxemia.We reviewed 40 fetuses with dTGA. 20/40 fetuses received urgent BAS at birth. Not only the restrictive but also the hypermobile and the redundant appearance of the foramen ovale was significantly associated with urgent BAS (p0.0001, p = 0.002 and p = 0.0001, respectively).Prenatal evaluation of the foramen ovale appearance in fetuses with dTGA is still challenging. Based on our experience, also the redundant foramen ovale appearance may need urgent BAS at birth.

Summary Objective: To present and compare with literature our experience with an electronic anesthesia-related incident reporting form as a quality control measure at Gaslini Children’s Hospital over a 19-month period. Methods: All events that occurred between March 2009 and September 2010 were recorded. We adopted an electronic reporting form included in the online recording process of every anesthetic procedure. Events were divided into near misses and adverse events. Adverse events were further divided into incidents, minor events, and major events. Patients were divided into three age-groups: 3 years. Results: A total of 12 850 anesthetics were performed. Eight (0.06%) near misses and 108 (0.8%) adverse events were reported. Adverse events occurred more frequently in infants. Of 108 events, 35 (32.4%), 61 (56.5%), and 12 (11.1%) were classified as incidents, minor, and major events, respectively. Of all the adverse events, 66 (61%) were respiratory, 27 (25%) organizational, six (5%) drug-related, four (4%) cardiocirculatory, and five (5%) miscellaneous. Conclusions: Infants were at the highest risk to experience adverse events. Although experimental electronic incident reporting proved to be feasible, there is reason to suspect that there was underreporting of near misses. Overreporting of near miss events may be enhanced by easier and more straightforward reporting forms as well as by better education for anesthetic providers about the importance of recognizing and reporting near misses.

The aim of this study was to evaluate the frequency of surgical and organizational events that occurred in the whole Department of Paediatric Surgery at Gaslini Children’s Hospital through an incident-reporting system in order to identify the vulnerabilities of this system and improve it. This is a 6-month prospective observational study (1st January–1st July 2010) of all events (including surgical and organizational events, and near misses) that occurred in our department of surgery (pediatric surgery, orthopedics and neurosurgery units). Over a 6-month study period, 3,635 children were admitted: 1,904 out of 3,635 (52.4%) children underwent a surgical procedure. A total number of 111 adverse events and 4 near misses were recorded in 100 patients. A total of 108 (97.3%) adverse events occurred following a surgical procedure. Of 111 adverse events, 34 (30.6%) required re-intervention. Eighteen of 100 patients (18%) required a re-admission, and 18 of 111 adverse events (16.2%) were classified as organizational. Infection represented the most common event. An electronic physician-reported event tracking system should be incorporated into all surgery departments to report more accurately adverse events and near misses. In this system, all definitions must be standardized and near misses should be considered as important as the other events, being a rich source of learning.

Summary Background: Clonidine has the potential to significantly prolong the duration of caudal epidural anesthesia. We investigated the effect of the addition of clonidine to the MLAC of levobupivacaine in a randomized controlled dose–response trial. Methods: A group of 120 children aged

Neurologically impaired children (NIC) have a high risk of recurrence of gastroesophageal reflux (GER) following fundoplication. A postpyloric feeding tube may be useful when gastric emptying disorders occur; however, dislocation and difficulty in feeding management often require more aggressive procedures. Total esophagogastric dissociation (Bianchi's TEGD) is an alternative to the classic fundoplication procedure, whereas laparoscopic gastric bypass is a frequently performed procedure in morbid obesity, improving gastric outlet.The aim of this paper is to present a preliminary experience on the laparoscopic Roux-en-Y gastrojejunal bypass, associated with Nissen fundoplication and gastrostomy, to treat and prevent GER in NIC with gastric emptying disorders.Eight neurologically impaired children underwent surgical treatment because of feeding problems and pulmonary complications. The procedure included: 1) hiatoplasty, 2) Nissen fundoplication, 3) 20-cm Roux-en-Y gastrojejunal anastomosis and jejuno-jejunal anastomosis, and 4) gastrostomy.All cases were fed on postoperative day 3 without any intraoperative complications. One case developed an obstruction of the distal anastomosis due to adhesion and needed reoperation. Outcome was clinically evaluated with serial upper gastrointestinal contrast studies and endoscopies.Laparoscopic proximal Roux-en-Y gastrojejunal diversion, without gastric resection, is a safe, feasible procedure that improves gastric emptying and reduces the risk of GER recurrence. Yet, long-term results still have to be evaluated.

To present the experience documented over 1 year of analysis and quality control on surgical complications and organizational accidents.All children admitted during the study period at our Institution were included in the analysis, which consisted of four phases: (1) definition and standardization of perioperative diagnostic and therapeutic tracks; (2) staff education; (3) documentation and data implementation, and (4) "Morbidity and Mortality" audit.Over a 1-year study period, 3,116 children were admitted to our Institution: 2,222 out of 3,116 (71.3%) children underwent a surgical procedure. A total number of 184 complications were recorded in 149 patients. One hundred and seventy-one (92.9%) complications occurred following a surgical procedure. Fifty-six out of 149 complicated patients (37.6%) required a re-operation. Thirty-five out of 184 (19%) complications were classified as organizational. Infection represented the most common complication. All cases of anastomotic dehiscence and perforation, bowel obstruction, and stoma malfunction required reintervention. None of the postoperative bleedings required a second surgical procedure.Although a proper statistical comparison with literature complication rates is not feasible, our experience confirms the importance of quality-control audit in health care systems. Prolonged observation, long-term follow up, and comparison with previous results will represent our future goal.

Background: The preoperative assessment involves the process of evaluating the patient’s clinical condition, which is intended to define the physical status classification, eligibility for anesthesia, and the risks associated with it, thus providing elements to select the most appropriate and individualized anesthetic plan. We obtained evidence concerning pediatric preoperative evaluation on specific conditions and clinical problems: upper respiratory infections, asthma and bronchial hyperresponsiveness, bronchopulmonary dysplasia, allergies, heart murmurs, and vaccination. We produce a set of recommendations, with emphasis on the level of evidence, clinical relevance, and the risk/benefit ratio.

Fundoplication is considered a mainstay in the treatment of gastro-esophageal reflux. However, the literature reports significant recurrences and limited data on long-term outcome. To evaluate our long-term outcomes of antireflux surgery in children and to assess the results of redo surgery. We retrospectively analyzed all patients who underwent Nissen fundoplication in 8 consecutive years. Reiterative surgery was indicated only in case of symptoms and anatomical alterations. A follow-up study was carried out to analyzed outcome and patients’ Visick score assessed parents’ perspective. Overall 162 children were included for 179 procedures in total. Median age at first intervention was 43 months. Comorbidities were 119 (73 %), particularly neurological impairments (73 %). Redo surgery is equal to 14 % (25/179). Comorbidities were risk factors to Nissen failure (p = 0.04), especially children suffering neurological impairment with seizures (p = 0.034). Follow-up datasets were obtained for 111/162 = 69 % (median time: 51 months). Parents’ perspectives were excellent or good in 85 %. A significant positive impact of redo Nissen intervention on the patient’s outcome was highlighted; antireflux surgery is useful and advantageous in children and their caregivers. Children with neurological impairment affected by seizures represent significant risk factors.

This study presents a technical report of a standardized approach to the perinephric area in a series of pediatric patients, demonstrating that whatever renal or suprarenal surgery is planned, this can be approached and accomplished laparoscopically with an identical or very similar port triangulation, thus facilitating the learning curve within the same surgical team.All patients undergoing renal and adrenal gland surgery with a minimally invasive approach in the period from October 2008 to November 2013 were retrospectively reviewed and included in the study. Technical details and clinical outcomes are described.In total, 68 patients matched the inclusion criteria and were therefore retrospectively examined. No major intraoperative complication occurred. Two patients developed recurrent pelvic-ureteric junction obstruction and were managed with double J stent positioning and laparoscopic dismembered pyeloplasty, respectively. Two patients presenting with adrenal mass experienced a moderate bleeding that could be treated conservatively.Thanks to the reproducibility of the procedure and on the grounds of the presented experience, we support the proposal that the transperitoneal laparoscopic approach to the kidney and the adrenal gland could represent a relatively easy and safe way to quickly achieve confidence and skill in the management of related diseases.

Background Perianal abscesses and fistulae have been reported in approximately 15% of patients with paediatric Crohn's disease and they are associated with poor quality of life. Several surgical techniques were proposed for the treatment of perianal Crohn's disease, characterized by an elevated incidence of failure, incontinence, and relapse. Aim of our study was to present the technical details and results of our surgical technique in case of recurrent, persistent, complex perianal ano-rectal destroying Crohn's disease not responding to medical treatment. Methods Data of patients who underwent surgical treatment (cone-like resection, fistulectomy, sphincter reconstruction, endorectal advancement sleeve flaps like in Soave endorectal pull-through) for complicated high-level trans, inter or suprasphincteric fistulae between January 2009 and June 2014 were retrospectively reviewed. Results 20 surgical procedures were performed in 11 patients (males 72.7%) with transsphincteric (n = 5), intersphincteric (n = 4) and suprasphincteric (n = 2) fistulae. Three patients needed a second treatment. Two patients needed more than 2 surgeries and one temporary colostomy. No patient presented anal incontinence at 15 months’ median follow-up. Conclusions Although several procedures may be required to obtain a complete remission of perianal lesions, in our series the proposed surgical technique seemed effective and safe, preserving anal continence in all treated cases and reducing the need of faecal diversion.

The incidence of paediatric inflammatory bowel disease (PIBD) has dramatically increased in the last 20 years. Although first reported in mid 1970s’, diagnostic laparoscopy has started to be routinely adopted in paediatric surgical practice since late 1990s’. Minimally invasive surgery was first limited to diagnostic purposes. After 2002 it was also applied to the radical treatment of PIBD, either Crohn’s disease (CD) or Ulcerative colitis. During the last decade minimally invasive approaches to PIBD have gained popularity and have recently became the “gold standard” for the treatment of such invalidating and troublesome chronic diseases. The authors describe and track the historical evolution of minimally invasive surgery for PIBD and address all available opportunities, including most recent advancements such as robotic surgery, single port approaches and minimally invasive treatment of perianal fistulising CD. A systematic review of all series of PIBD treated with minimally invasive approaches published so far is provided in order to determine the incidence and type of patients’ complications reported up to present days. The authors also describe their experience with minimally invasive surgery for PIBD and will report the results of 104 laparoscopic procedures performed in a series of 61 patients between January 2006 and December 2014.

OBJECTIVES: To evaluate the efficacy of fenoldopam mesylate (dose 0.2 µg/kg/min) in reducing the occurrence of hyperlactataemia (i.e. peak level of blood lactate >2.0 mM/l) during cardiopulmonary bypass (CPB) in paediatric cardiac surgery. Hyperlactataemia occurring during CPB for paediatric cardiac surgery is considered an early biomarker of an increased risk of poor outcome. METHODS: This was a dose/effectiveness clinical study applying Simon’s two-stage optimal design with 5% type I error rate and 90% statistical power. Following parents’ written informed consent, 53 children undergoing elective cardiac surgery with CPB between March 2009 and February 2012 were enrolled. Inclusion criteria were children weighing 3–15 kg scheduled for elective cardiac surgery and with expected CPB time of 60–180 min. Patients requiring surgery with total circulatory arrest were excluded. All patients received fenoldopam infusion at a dose of 0.2 µg/kg/min from the beginning of surgery until the end of CPB. RESULTS: The primary end-point was the evaluation of response to fenoldopam, i.e., blood lactate levels ≤2.0 mM/l. A total of 53 children, median age 5.7 months (range 11 days to 48 months) were enrolled. In the first stage, 18 of 19 (95%) children achieved normalization of lactate values. Then the study was continued to stage II by enrolling an additional 34 patients. At study conclusion, 96.2% of patients showed normalized lactate values. Fenoldopam infusion was well tolerated in all patients. No adverse events were observed. CONCLUSIONS: In this study, fenoldopam at a dose of 0.2 µg/kg/min was well tolerated in paediatric patients undergoing elective cardiac surgery with CPB. In 96.2% of patients, infusion of fenoldopam was associated with intraoperative blood lactate

Over the last 20 years MIS has progressively gained popularity in children with cancer. We therefore aimed at evaluating the safety of Minimally Invasive Surgery (MIS) resection in a series of children affected by adrenal neuroblastoma (NB) presenting without Image-Defined Risk Factors (IDRFs). An Institutional protocol for MIS resection of adrenal NB in pediatric patients without IDRFs has been applied since 2008. Absence of IDRFs represented the main indication for MIS in NB, regardless of tumor size. All pediatric patients who underwent MIS for NB between January 2008 and May 2013 were included. Specific technical considerations, demographic data, and outcome have been recorded. Twenty-one patients underwent MIS resection for IDRFs-negative adrenal NB. Nine of these patients experienced preoperative downgrading of IDRFs after chemotherapy. Radiological median diameter of the mass was 30 mm (range 10–83 mm). Median operative time was 90 min. Median hospital stay was 4 days. All patients were treated successfully, without serious intraoperative complications. One mild intraoperative hemorrhage occurred and was treated without the need for conversion to open surgery nor blood transfusion was required. No postoperative complications, including port-site or peritoneal metastases were experienced. This study demonstrated the safety and effectiveness of MIS for the resection of adrenal NB without IDRFs in children. Pediatric surgeons dedicated to oncology should be aware of this alternative approach to open resection.

Aim of this study was to present a series of neonates and ex-preterm babies who underwent inguinal hernia repair focusing on complications and possible indication to perform routine contralateral groin exploration.This is a retrospective study of a series of consecutive patients weighing less than 5 kg who underwent inguinal hernia repair between January 2007 and December 2012. Only the affected side was treated. Patients have been routinely followed up postoperatively. We resorted to available outpatients' charts and admission notes to record demographic data, surgical details, complications and the occurrence of metachronous hernias. A questionnaire was administered to all patients' relatives to confirm the long-term outcome.One hundred fifty-four patients were operated for a total of 184 herniotomies (88 right sided, 36 left sided and 30 bilateral). Median length of follow-up was 42 months (range 6 months-7.5 years). Thirteen patients (13/124 = 10.5 %) developed metachronous hernia that proved to be significantly more frequent in patients weighing less than 1,500 g at birth (p 0.05). We observed 10 % of complications, including 2.7 % testicular atrophy and 4.5 % recurrence. Atrophy proved to occur more frequently in patients who experienced preoperative incarceration (p 0.05). No other risk factors were identified.The results of our series demonstrated that, though technically demanding, herniotomy in the neonate and ex-preterm is associated with a relatively low incidence of complications. Based on our results and in accordance with literature data, we do not advocate routine contralateral exploration in case of unilateral hernia but surgery to be performed only on the symptomatic side, as soon as possible after initial diagnosis. Very low birth weight patients should be followed with care in the early postoperative period due to the higher likelihood of developing a metachronous hernia.

Paediatric urology often presents challenging scenarios. Magnetic resonance urography (MRU) and laparoscopy are increasingly used. We retrospectively studied children affected by a disease of the upper urinary tract who after MRU were elected for laparoscopic treatment. This pictorial essay draws on our experience; it illustrates some specific MRU findings and highlights the usefulness of MRU for the diagnosis of upper urinary tract pathology in children. It also offers some examples of the potential additional diagnostic information provided by laparoscopy as well as its therapeutic role.

Thoracoscopy is increasingly being used for thoracic surgery in both adults and children. Improvements in technology and surgical skills are the main reasons for the dramatic increase in patients being referred for thoracoscopic surgery. As the age and weight of the patients being referred for surgery are declining, newborns and infants are frequently scheduled for thoracoscopic surgery. Deflation of the lung at the surgical site is extremely useful for adequate surgical exposure, especially in the case of pulmonary resection [1].

Background: Esophageal achalasia is not a frequent disorder in children and different treatments have been proposed during past decades. This study reviews the results of the laparoscopic Heller-Dor procedure performed in pediatric patients in two different surgical units. Methods: We included the patients aged

Perineal sepsis is a life-threatening complication of acute leukemia. Although conservative management (antibiotics, incision, and drainage, alone or in combination) is considered the criterion standard, it provides an outcome that is not fully satisfactory, with an overall mortality of roughly 30%. This report presents a case series of 4 children who underwent early defunctioning colostomy for the treatment of perineal sepsis during leukemia. This management proved to be successful and allowed prompt reestablishment of chemotherapy, thus improving overall results. Routine application of this "aggressive" management in these cases will presumably increase overall survival of children with leukemia.

Minimally invasive surgery is being increasingly applied to inflammatory bowel diseases (IBDs). Few pediatric series from selected research have been described to date. This study describes a unicentric experience of laparoscopic treatment of children with IBDs. All consecutive patients with IBDs between February 2006 and February 2010 who underwent laparoscopic treatment were included. We reviewed notes and recorded demographic data, indications, perioperative management, surgical details, length of surgery, complications, postoperative management, length of hospitalization and functional outcome. We performed 25 procedures on 16 patients (12 ulcerative colitis, 3 Crohn’s disease, and 1 indeterminate colitis). Median age was 12 years. A total of 50% patients underwent elective surgery; 11 underwent staged laparoscopic subtotal colectomy (LSTC) followed by J-pouch ileorectal anastomosis (JPIRA). Three patients underwent straight LSTC + JPIRA. All procedures included protective ileostomy. Length of surgery ranged between 120 and 380 min depending on the procedure (LSTC ± JPIRA). No conversion was required. Length of hospitalization ranged between 3 and 18 days. We observed six complications (24%) mainly represented by adhesions that were effectively treated laparoscopically. Ten patients were restored (ileostomy closure) and were assessed for continence that turned out to be good in 80%. Laparoscopy proved to be feasible, safe and effective for the treatment of IBD in children. Although we observed a relatively low incidence of complications, stoma site adhesions still remain the major issue, which can be effectively dealt with laparoscopically. Functional outcome as well as cosmesis is satisfactory. As results are encouraging, at present we prefer laparoscopy for the surgical treatment of IBD in pediatric patients.

Chronic intestinal pseudo-obstruction (CIPO) can occur as a consequence of neuropathies including diffuse Intestinal Neuronal Dysplasia (IND), a relatively rare enteric nervous system (ENS) abnormality. Although various authors reported of diffuse IND associated either with intestinal malrotation or megacystis, the co-existence of these three entities in the same patient has never been described before. The aim of this paper is to report for the first time in literature a series of patient with such association, focusing on one who carries a de novo duplication of chromosome 12, suggesting a new syndromic association (megacolon, megacystis, malrotation). © 2011 Wiley-Liss, Inc.

Summary Activation of the oxidative burst and failure of CD4+CD25+ cell regulation have been implicated in idiopathic nephrotic syndrome (iNS). The intimate mechanism is, however, unknown and requires specifically focused studies. We investigated reactive oxygen species (ROS) generation [di-chlorofluorescein-diacetate (DCFDA)] fluorescence assay and the regulatory adenosine 5′-triphosphate (ATP) pathways in the blood of 41 children with iNS, utilizing several agonists and antagonists of nucleotide/nucleoside receptors, including the addition of soluble apyrase. The CD4+CD25+CD39+/CD73+ expression was determined in vivo in parallel during disease activity. Overall, we found that the percentage of CD39+CD4+CD25+ was reduced markedly in iNS by 80% (3·43 ± 0·04% versus 13·14 ± 0·07% of total lymphocytes, P

Clonidine has the potential to significantly prolong the duration of caudal epidural anesthesia. We investigated the effect of the addition of clonidine to the MLAC of levobupivacaine in a randomized controlled dose-response trial.A group of 120 children aged6 years of age received caudal anesthesia with levobupivacaine and 1, 2, or 3 μg·kg(-1) of clonidine. The MLAC was determined according to a Dixon-Massey protocol. The primary outcome was effective surgical anesthesia. Secondary outcomes were the duration of postoperative analgesia, postoperative pain scores, clonidine side effects, and time to hospital discharge.The MLAC of caudal levobupivacaine was 0.106%, 0.077%, and 0.035% with 1, 2, and 3 μg·kg(-1) of clonidine, respectively. There were significant dose-dependent increases in median duration of analgesia. The incidence of delayed discharge, somnolence, and PONV was significantly increased in the 3 μg·kg(-1) of clonidine group.Clonidine produces a local anesthetic sparing effect with a dose-dependent decrease in levobupivacaine MLAC for caudal anesthesia. In addition, there is a dose-dependent prolongation of postoperative analgesia following lower abdominal surgery in children. A dose of 2 μg·kg(-1) of clonidine provides the optimum balance between improved analgesia and minimal side effects.

Summary The mechanism responsible for proteinuria in non-genetic idiopathic nephrotic syndrome (iNS) is unknown. Animal models suggest an effect of free radicals on podocytes, and indirect evidence in humans confirm this implication. We determined the oxidative burst by blood CD15+ polymorphonucleates (PMN) utilizing the 5-(and-6)-carboxy-2′,7′-dichlorofluorescin diacetate (DCF-DA) fluorescence assay in 38 children with iNS. Results were compared with PMN from normal subjects and patients with renal pathologies considered traditionally to be models of oxidative stress [six anti-neutrophil cytoplasmic autoantibody (ANCA) vasculitis, seven post-infectious glomerulonephritis]. Radicals of oxygen (ROS) production was finally determined in a patient with immunodeficiency, polyendocrinopathy, enteropathy X-linked (IPEX) and in seven iNS children after treatment with Rituximab. Results demonstrated a 10-fold increase of ROS production by resting PMN in iNS compared to normal PMN. When PMN were separated from other cells, ROS increased significantly in all conditions while a near-normal production was restored by adding autologous cells and/or supernatants in controls, vasculitis and post-infectious glomerulonephritis but not in iNS. Results indicated that the oxidative burst was regulated by soluble factors and that this regulatory circuit was altered in iNS. PMN obtained from a child with IPEX produced 100 times more ROS during exacerbation of clinical symptoms and restored to a near normal-level in remission. Rituximab decreased ROS production by 60%. In conclusion, our study shows that oxidant production is increased in iNS for an imbalance between PMN and other blood cells. Regulatory T cells (Tregs) and CD20 are probably involved in this regulation. Overall, our observations reinforce the concept that oxidants deriving from PMN are implicated in iNS.

Either "open" and laparoscopic spleen surgery in pediatric age are well known and performed with ease in children. Yet, few data regarding follow-up and outcome are discussed in the international literature.Clinical notes of all patient who underwent spleen surgery in a single center between 2000 and 2007 were reviewed and a specific follow-up questionnaire was administered, aiming to evaluate pre- and postoperative data, especially considering underlying disease, cosmetic results, and quality of life after surgery.Fifty-one patients underwent spleen surgery in our series, 33 of whom returned a complete follow-up questionnaire and were included in the study. Splenectomy was performed in 26 patients, whereas 7 patients underwent a partial splenectomy; 19 cases (57.6%) were approached laparoscopically. A total of 4 complications (12%) occurred in our series, none of them being intraoperative. Hospital stay resulted as significantly shorter in laparoscopic cases (5.5 +/- 2.9 vs. 8.7 +/- 4.8 days; P0.01), with better results in terms of cosmetics. Quality of life is strictly related to underlying disease, as well as long-term survival.Whatever surgical approach is adopted, spleen surgery is safe, effective, and reproducible. When feasible, the laparoscopic approach should be preferred to the traditional open approach, as far as efficacy and safety are similar, in order to reduce hospital stay, abdominal wall traumatism, and consequently, improve postoperative pain control and cosmetic results.

Endorectal pull-through (ERPT) is a widely accepted procedure for the treatment of Hirschsprung's disease (HSCR). This study was aimed at presenting the long-term results of patients with classic HSCR who were operated on with a laparoscopic-assisted Georgeson procedure and to compare them to patients treated with a Soave-Boley procedure.Patients treated for Hirschsprung disease in the period 1997-2006 with a minimum follow-up of 6 months were prospectively included in this study. Demographic details, associated anomalies, surgical technique, length of aganglionosis, and postoperative complications were collected. A questionnaire was submitted to all families to assess general health, bowel adaptation, fecal and urinary continence, cosmetic results, and patients' and parents' perspective of overall outcome.Overall, 162 patients underwent a pull-through procedure: 25 patients treated with Georgeson and 21 with Soave-Boley ERPT were eligible for this study. Conversion was required in 3 of 28 patients approached laparoscopically. Hospitalization was shorter for patients treated laparoscopically (P0.05), whereas length of surgery was comparable. Complication rate was similar for both groups, as well as growth that remained within normal ranges for age. Long-term outcome, in terms of bowel movements, was similar. None of the patients experienced fecal and/or urinary incontinence. Cosmetic results proved to be excellent to good in all patients undergoing the Georgeson and in 67% of patients undergoing the Soave-Boley procedure (P0.05). Patients' perspective of overall outcome was excellent in more than 90% of patients from both groups.Overall results proved to be similar. Likewise, long-term bowel function did not show significant differences. Nonetheless, if we consider hospitalization and cosmetic results, it becomes clear that the minimally invasive approach should be preferred, when possible, to improve patients' comfort, perspective of overall health status, and psychologic acceptance.

Malignant neuroblastic tumors (MNT) commonly affect children in the early years of life and become rare afterwards. The clinical characteristics and outcome of these tumors occurring in adolescents are still unclear. In this review, the main clinical reports available in the literature are summarized together with some original data. In general, MNT occurring in adolescents, if compared to those occurring in children, are more frequently diagnosed in advanced stages, have uncommon sites of metastases, but have less frequently unfavorable biological markers. If treated with standard protocols as those used for children, their survival is poor; in particular, patients with localized disease seem to have a more aggressive course than children.

Background: The role of surgery is debated for children with gastroesophageal reflux disease (GERD), particularly when they show atypical symptoms. This study was designed to evaluate the safety and outcome of laparoscopic Nissen-Rossetti fundoplication performed in a selected population of children with gastroesophageal reflux and atypical supraesophageal symtpoms. Methods: This prospective study included 595 patients younger than 14 years with GERD who reported recurrent respiratory symptoms and had no benefit from standard medical treatment. Surgery was performed for 48 patients with anatomic anomalies, life-threatening events, or respiratory complications after ineffective medical treatment. The subjective and objective outcomes were evaluated. Results: No major intraoperative complications were experienced, and there was no recurrence of gastroesophageal reflux during a postoperative follow-up period of 12 months. The parents’ final subjective evaluation of the outcomes 12 months after surgery was positive in 44 cases and negative in 4 cases. Conclusions: Children with difficult-to-treat chronic respiratory symptoms must be evaluated for GERD, even if the need for surgery is low (8%), because complete eradication of reflux is mandatory. Radical treatment of GERD allows the pulmonologist to perform correct respiratory treatment and to prevent the development chronic and life-threatening complications.