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Objective: To determine whether brain volumetric and white matter microstructural changes are present and correlate with neurological impairment in subjects with alternating hemiplegia of childhood (AHC). Methods: In this prospective single-center study, 12 AHC subjects (mean age 22.9 years) and 24 controls were studied with 3DT1-weighted MR imaging and high angular resolution diffusion imaging at 3T. Data obtained with voxel-based morphometry and tract-based spatial statistics were correlated with motor impairment using the International Cooperative Ataxia Rating Scale (ICARS) and Movement and Disability sub-scales of Burke-Fahn-Marsden Dystonia Rating Scale (BFMMS and BFMDS). Results: Compared to healthy controls, AHC subjects showed lower total brain volume (P < 0.001) and white matter volume (P = 0.002), with reduced clusters of white matter in frontal and parietal regions (P < 0.001). No significant regional differences were found in cortical or subcortical grey matter volumes. Lower cerebellar subvolumes correlated with worse ataxic symptoms and global motor impairment in AHC group (P < 0.001). Increased mean and radial diffusivity values were found in the corpus callosum, corticospinal tracts, superior and inferior longitudinal fasciculi, subcortical frontotemporal white matter, internal and external capsules, and optic radiations (P < 0.001). These diffusion scalar changes correlated with higher ICARS and BFMDS scores (P < 0.001). Interpretation: AHC subjects showed prevalent white matter involvement, with reduced volume in several cerebral and cerebellar regions associated with widespread microstructural changes reflecting secondary myelin injury rather than axonal loss. Conversely, no specific pattern of grey matter atrophy emerged. Lower cerebellar volumes, correlating with severity of neurological manifestations, seems related to disrupted developmental rather than neurodegenerative processes. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: 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.Aims: To evaluate our long-term outcomes of antireflux surgery in children and to assess the results of redo surgery.Methods: 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.Results: 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 %.Conclusions: 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. [ABSTRACT FROM AUTHOR]

Purpose: 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.Patients and Methods: 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.Results: 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.Conclusions: 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: 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). Methods: 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. Results: 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. Conclusions: 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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: < 1, between 1 and 3, and >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. [ABSTRACT FROM AUTHOR]

Purpose: 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. Materials and methods: 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). Results: 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. Conclusions: 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. [ABSTRACT FROM AUTHOR]

Background: 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.Materials and Methods: 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.Results: 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%.Conclusions: 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. [ABSTRACT FROM AUTHOR]

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 aged <6 years of age received caudal anesthesia with levobupivacaine and 1, 2, or 3 μg·kg 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 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 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 of clonidine provides the optimum balance between improved analgesia and minimal side effects. [ABSTRACT FROM AUTHOR]

Background: 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. Aim: 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. Materials and Methods: 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. Results: 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. Conclusions: 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. [ABSTRACT FROM AUTHOR]

Purpose: To present the experience documented over 1 year of analysis and quality control on surgical complications and organizational accidents.Methods: 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.Results: 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.Conclusion: 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. [ABSTRACT FROM AUTHOR]

Background: 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. Materials and Methods: 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. Results: 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; P < 0.01), with better results in terms of cosmetics. Quality of life is strictly related to underlying disease, as well as long-term survival. Conclusions: 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. [ABSTRACT FROM AUTHOR]

Introduction: 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 and Methods: 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. Results: 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 ( P < 0.05), 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 ( P < 0.05). Patients' perspective of overall outcome was excellent in more than 90% of patients from both groups. Conclusions: 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. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented regarding the utilization of Arndt pediatric bronchial blocker (BB) as a medical protocol for one lung ventilation (OLV) thoracoscopic surgery in children.

Introduction: The aim of this study is to present the “fast-track” experience in children who underwent colon resection. Materials and Methods: Forty-six children who underwent laparoscopic colon resection were prospectively included in the study. Anomalies of colon innervation and inflammatory bowel disease represented the main surgical indications. Results: Left colon/sigmoid resection was performed in 37, total colon resection was done in 5, and right colon resection in 4 children. Total colon resection was always associated to ileostomy. Anastomosis was performed in 41 cases. Patients were postoperatively monitored for pain, return to normal activity, feeding, bowel movements, and complications. Stool passage and oral feeding were started on postoperative day 1, and all patients were discharged before postoperative day 4. One child was readmitted the day after discharge because of an anastomotic leak. No other major complications were recorded. Discussion: Minimally invasive surgery is safe and effective in pediatric colonic surgery and allows a fast recovery time (fast-track). [ABSTRACT FROM AUTHOR]