White matter and cerebellar involvement in alternating hemiplegia of childhood.

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.

Potential role of a subcutaneously anchored securement device in preventing dislodgment of tunneled-cuffed central venous devices in pediatric patients.

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.

Complications of Minimally Invasive Surgery in Children: A Prospective Morbidity and Mortality Analysis Using the Clavien-Dindo Classification.

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.

A Provisional Experience with Robot-Assisted Soave Procedure for Older Children with Hirschsprung Disease: Back to the Future?

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.

Video-Assisted Needle Core Biopsy in Children Affected by Neuroblastoma: A Novel Combined Technique.

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.

Prenatal Echocardiographic Assessment of Foramen Ovale Appearance in Fetuses with D-Transposition of the Great Arteries and Impact on Neonatal Outcome.

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.

Brachiocephalic vein for percutaneous ultrasound-guided central line positioning in children: A 20-month preliminary experience with 109 procedures.

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.

Congenital Lung Malformations: Shifting from Open to Thoracoscopic Surgery.

BACKGROUND: Over the years the need for surgical treatment, timing of intervention, and the type of surgical approach have been discussed, but the treatment of congenital lung malformations remains controversial. The aim of this study was to compare the thoracotomy approach with the thoracoscopic technique by evaluating different surgical outcomes (duration of surgery, postoperative hospital stay, and complications). METHODS: All patients operated from January 2011 to March 2015 for suspected congenital cystic lung were included in the study. Patients treated for congenital lobar emphysema and tracheobronchial neoplasms were excluded from the study. RESULTS: In the analyzed period, 31 asymptomatic patients were treated: 18 lung resections were performed with thoracotomy (Group A) and 13 with the thoracoscopic approach (Group B). No significant differences were observed between the age and weight at surgery, length of the procedures, complications, and the need for postoperative intensive care between the two groups. The postoperative hospital stay in Group A was twice that for Group 2 (p = 0.0009). CONCLUSION: Comparing thoracoscopic surgery with the traditional open approach, we confirmed the superiority of minimally invasive treatment in terms of postoperative hospital stay. Common technical recommendations can help pediatric centers to develop the thoracoscopic approach for the treatment of congenital pulmonary malformations.

Long-term outcome and need of re-operation in gastro-esophageal reflux surgery in children.

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.

Minimally invasive surgery for paediatric inflammatory bowel disease: Personal experience and literature review.

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.

Transperitoneal Laparoscopic Approach to the Perinephric Area in Children: Technical Report and Lessons Learned.

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.

Cone-like resection, fistulectomy and mucosal rectal sleeve partial endorectal pull-through in paediatric Crohn's disease with perianal complex fistula.

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.

A novel balanced isotonic sodium solution vs normal saline during major surgery in children up to 36 months: a multicenter RCT.

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.

Beneficial effect of fenoldopam mesylate in preventing peak blood lactate level during cardiopulmonary bypass for paediatric cardiac surgery.

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 <2.0 mM/l.

Laparoscopic resection of adrenal neuroblastoma without image-defined risk factors: a prospective study on 21 consecutive pediatric patients.

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.

Sirolimus as maintenance treatment in an infant with life-threatening multiresistant pure red cell anemia/autoimmune hemolytic anemia.

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.

Magnetic resonance urography and laparoscopy in paediatric urology: a case series.

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.

Early diverting colostomy for perianal sepsis in children with acute leukemia.

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.

Provisional unicentric experience with an electronic incident reporting form in pediatric anesthesia.

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.