A Novel Standard for Systematic Reporting of Neuroblastoma Surgery: The International Neuroblastoma Surgical Report Form (INSRF): A Joint Initiative by the Pediatric Oncological Cooperative Groups SIOPEN∗, COG∗∗, and GPOH∗∗∗.

OBJECTIVE: To create the first structured surgical report form for NBL with international consensus, to permit standardized documentation of all NBL-related surgical procedures and their outcomes. SUMMARY OF BACKGROUND DATA: NBL, the most common extracranial solid malignant tumor in children, covers a wide spectrum of tumors with significant differences in anatomical localization, organ or vessel involvement, and tumor biology. Complete surgical resection of the primary tumor is an important part of NBL treatment, but maybe hazardous, prone to complications and its role in high-risk disease remains debated. Various surgical guidelines exist within the protocols of the different cooperative groups, although there is no standardized operative report form to document the surgical treatment of NBL. METHODS: After analyzing the treatment protocols of the SIOP Europe International Neuroblastoma Study Group, Children's Oncology Group, and Gesellschaft fuer Paediatrische Onkologie und Haematologie - German Association of Pediatric Oncology and Haematology pediatric cooperative groups, important variables were defined to completely describe surgical biopsy and resection of NBL and their outcomes. All variables were discussed within the Surgical Committees of SIOP Europe International Neuroblastoma Study Group, Children's Oncology Group, and Gesellschaft fuer Paediatrische Onkologie und Haematologie - German Association of Pediatric Oncology and Haematology. Thereafter, joint meetings were organized to obtain intercontinental consensus. RESULTS: The "International Neuroblastoma Surgical Report Form" provides a structured reporting tool for all NBL surgery, in every anatomical region, documenting all Image Defined Risk Factors and structures involved, with obligatory reporting of intraoperative and 30 day-postoperative complications. CONCLUSION: The International Neuroblastoma Surgical Report Form is the first universal form for the structured and uniform reporting of NBL-related surgical procedures and their outcomes, aiming to facilitate the postoperative communication, treatment planning and analysis of surgical treatment of NBL.

Time Is Money in Case of a Button Battery Ingestion.

Button battery (BB) ingestion is a preventable pediatric health hazard with important morbidity and mortality due to complications. We present 3 pediatric patients with a complicated course after BB ingestion and discuss current guidelines. Urgent endoscopic removal is necessary for every BB impacted in the esophagus. A new strategy before endoscopic removal is the administration of honey or sucralfate. During endoscopy, rinsing the esophageal mucosae with acetic acid can neutralize the alkalic environment and prevent late complications. Prevention of ingestion needs to be pursued by increasing awareness and changing legislation of packaging of BB.

Influence of Surgical Excision on the Survival of Patients With Stage 4 High-Risk Neuroblastoma: A Report From the HR-NBL1/SIOPEN Study.

PURPOSE: To evaluate the impact of surgeon-assessed extent of primary tumor resection on local progression and survival in patients in the International Society of Pediatric Oncology Europe Neuroblastoma Group High-Risk Neuroblastoma 1 trial. PATIENTS AND METHODS: Patients recruited between 2002 and 2015 with stage 4 disease > 1 year or stage 4/4S with MYCN amplification < 1 year who had completed induction without progression, achieved response criteria for high-dose therapy (HDT), and had no resection before induction were included. Data were collected on the extent of primary tumor excision, severe operative complications, and outcome. RESULTS: A total of 1,531 patients were included (median observation time, 6.1 years). Surgeon-assessed extent of resection included complete macroscopic excision (CME) in 1,172 patients (77%) and incomplete macroscopic resection (IME) in 359 (23%). Surgical mortality was 7 (0.46%) of 1,531. Severe operative complications occurred in 142 patients (9.7%), and nephrectomy was performed in 124 (8.8%). Five-year event-free survival (EFS) ± SE (0.40 ± 0.01) and overall survival (OS; 0.45 ± 0.02) were significantly higher with CME compared with IME (5-year EFS, 0.33 ± 0.03; 5-year OS, 0.37 ± 0.03; P < .001 and P = .004). The cumulative incidence of local progression (CILP) was significantly lower after CME (0.17 ± 0.01) compared with IME (0.30 ± 0.02; P < .001). With immunotherapy, outcomes were still superior with CME versus IME (5-year EFS, 0.47 ± 0.02 v 0.39 ± 0.04; P = .038); CILP was 0.14 ± 0.01 after CME and 0.27 ± 0.03 after IME (P < .002). A hazard ratio of 1.3 for EFS associated with IME compared with CME was observed before and after the introduction of immunotherapy (P = .030 and P = .038). CONCLUSION: In patients with stage 4 high-risk neuroblastoma who have responded to induction therapy, CME of the primary tumor is associated with improved survival and local control after HDT, local radiotherapy (21 Gy), and immunotherapy.

A pilot study of the effectiveness of a serious game CliniPup® on perioperative anxiety and pain in children.

INTRODUCTION/AIM: Children experience important anxiety before surgery. Anxiety and pain are positively correlated. Serious gaming is a non-pharmacological intervention to prepare children and parents for an operation. We aimed to evaluate the effectiveness of the serious game CliniPup® on anxiety and pain in children undergoing ambulatory surgery. METHOD: A prospective randomized controlled pilot trial in 72 children aged 5 to 11 years old scheduled for day-care surgery (general surgery, dentistry, otorhinolaryngology, urology) was performed. Participants were randomly assigned into 3 groups: A (CliniPup®), B ("Empty game" without educational information), or C (no game, oral information at the outpatient clinic, current standard of care). Anxiety, pain, and behaviour were evaluated by validated instruments at six time-points: T0: baseline, T1: 1 week preoperatively, T2: at hospital admission, T3: before discharge, T4: 1 week postoperatively, T5: 1 month postoperatively. RESULTS: After playing the game (T1), the estimated mean anxiety score (VASa) was lower in Group A (1.9 units) versus Group B (2.7 units). The estimated mean VASa at T1 for Group A was 2.6 units lower compared to Group C (p = 0.003). For Group B, VASa levels were 1.8 units lower than in Group C (p = 0.045). After correction for "surgery type", Group A continued to show a significantly lower VASa compared to Group C (p = 0.044). On the other time points, no difference in anxiety and pain were observed, nor in post-hospitalization behaviour. CONCLUSION: Children that played the CliniPup® game one week before surgery had a significant reduction in preoperative anxiety after playing the game, but not on the other time points. No differences on peri-operative pain were observed during the different time points. TYPE OF STUDY: Randomized Trial. LEVEL OF EVIDENCE: Level II.

Multicentre survey on the current surgical management of oesophageal atresia in Belgium and Luxembourg.

INTRODUCTION: The surgical management of oesophageal atresia (OA) differs between pediatric surgical teams without consensus. We aimed to describe the current practice of OA treatment in Belgium and Luxembourg and compare this to the literature. MATERIALS AND METHODS: A questionnaire was created and sent to all 18 hospitals (14 pediatric surgical units) performing OA surgery in Belgium and Luxembourg. The results were compared to the literature. RESULTS: Most units treat an average of 2-5 OA+TOF (71%) and ≤1 pure OA (pOA) per year (86%). The preferred surgical approach for OA+TOF is thoracotomy (86%), mostly extra-pleural (75%). Thoracoscopic OA repair is performed in 21%. All centers perform an end-to-end anastomosis (interrupted sutures), and all leave a transanastomotic tube. A chest drain is routinely used in 8units (57%). In pOA the preferred surgical approach is gastrostomy formation with delayed primary anastomosis (77%). The timing for delayed anastomosis is 2 to 24months. Intra-operative lengthening is mostly attempted with Foker technique (46%). If oesophageal replacement is needed, gastric interposition is mostly used (75%). A postoperative contrast study is routinely performed in 86% for OA+TOF and in 100% for pOA. Anti-reflux medication is routinely prescribed by all units but one. CONCLUSION: There are still many differences and controversies in the perioperative management of OA. Part of this is based on habits and is difficult to change without scientific evidence. There is a need for prospective (inter)national registries to further identify the existing differences, leading to a more widely accepted consensus. LEVEL OF EVIDENCE: Level III.

Laparoscopic Treatment of Intestinal Malrotation in Children.

Purpose Intestinal malrotation is a congenital intestinal rotation anomaly, which can be treated by either laparotomy or laparoscopy. Our hypothesis is that laparoscopic treatment leads to less small bowel obstruction because of the fewer adhesions in comparison to laparotomy, without increasing the risk of recurrent volvulus. We analyzed the outcome of patients who had a correction for intestinal malrotation after the introduction of laparoscopy. Methods All patients between 0 and 18 years who underwent a surgical procedure for malrotation in the Radboudumc Amalia Children's Hospital, Nijmegen, the Netherlands, between January 2004 and December 2011 were retrospectively reviewed for duration of operation, perioperative complications, length of hospital stay, and rate of redo surgery for intestinal volvulus or obstruction. Results A total of 83 patients were included of which 33 had a laparoscopic procedure and 50 had a laparotomy for suspected malrotation. Operating time was 63 minutes for the laparoscopic procedure versus 76 minutes for laparotomy (p = 0.588). Significantly more complications were found in the laparotomy group (11 vs. 35%, p = 0.047). However, one patient (aged 4 months) in the laparoscopy group developed an early (< 24 hours) recurrent volvulus. Length of hospital stay was significantly longer after a laparotomy (9 vs. 16 days, p = 0.002). Three (17%) patients in the laparoscopy group needed redo surgery compared with six (9%) in the laparotomy group (p = 0.400). No late volvulus occurred in both groups. After laparotomy, redo surgery because of the small bowel obstruction was more frequent (5 vs. 0%), although this was not statistically significant. Conclusion In both the laparoscopy and laparotomy group, no cases of long-term recurrent volvulus were seen. After laparotomy, more patients developed a late small bowel obstruction because of the adhesions for which redo surgery was needed. In the laparotomy group, the number of complications was significantly higher and the length of hospital stay was significantly longer. Comparing laparoscopy and laparotomy for the treatment of malrotation, no difference exists for the long-term risk of recurrent volvulus. In children aged 6 months or older with suspicion of intestinal malrotation but not presenting with an acute abdomen or hemodynamically instability, laparoscopy should be considered as a first approach to diagnose and subsequently treat intestinal malrotation.

Retroperitoneal liposarcoma: current insights in diagnosis and treatment.

Retroperitoneal liposarcoma (RLS) is a rare, biologically heterogeneous tumor that present considerable challenges due to its size and deep location. As a consequence, the majority of patients with high-grade RLS will develop locally recurrent disease following surgery, and this constitutes the cause of death in most patients. Here, we review current insights and controversies regarding histology, molecular biology, extent of surgery, (neo)adjuvant treatment, and systemic treatment including novel targeted agents in RLS.

A true laparoscopic herniotomy in children: evaluation of long-term outcome.

Thirteen years of experience with a laparoscopic technique mimicking, as closely as possible, the classical open technique are presented in this report. Consecutive laparoscopic herniotomies in 385 children (178 girls and 207 boys; age 0.13 to 16.8 years) with a total of 525 hernia repairs were included into a retrospective review. Overall, 95% of children were treated on a day-care basis; all operations were completed laparoscopically. Mean operation time was 32.1 minutes (26.2 for unilateral hernias and 34.5 for bilateral). In total, 10.1% of children presented with clinically bilateral hernias, whereas 29.2% of clinical unilateral hernias had a patent contralateral processus vaginalis. We discovered femoral hernias in 2.6% and direct inguinal hernias in 0.5% of the cases. In 79 cases, a concomitant of umbilical herniorraphy was performed. There were no cases of testicular malposition or atrophy. We had a 1% hernia recurrence rate. Cosmetic outcome was excellent. Laparoscopy provides the surgeon with a superior diagnostic tool, facilitating the diagnosis of any kind of inguinal hernia or controlateral patent processus vaginalis and its repair in the same session; surgery- and anesthesia-related stress is minimized. This makes it an interesting alternative to the open repair of inguinal hernias in children, providing an excellent view on the cord structures and leaving them, as well as the testis, untouched.

A new minimally invasive technique for the repair of femoral hernia in children: about 13 laparoscopic repairs in 10 patients.

BACKGROUND: Femoral hernias in children are rare and often misdiagnosed. The classic treatment is through an open anterior approach. Since the advent of laparoscopic treatment of inguinal hernia in children, laparoscopy has been proposed to offer an accurate diagnosis and treatment, especially in case of recurrent hernia or bilateral disease. This review was undertaken to report our experience with the primary laparoscopic diagnosis and treatment of pediatric femoral hernias and to investigate its safety and feasibility. METHODS: All cases of pediatric femoral hernia in a consecutive series of children treated laparoscopically for groin hernias in a single institution over a 7-year period (2001-2007) were identified and studied for patient characteristics, presentation, pre- and perioperative findings, details of the operative repair, and postoperative outcome. RESULTS: Out of a prospectively studied series of 462 laparoscopic pediatric inguinal hernia repairs in 389 patients, 13 femoral hernias were treated in 10 patients (6 boys), with a mean age of 71/2 years (range, 1.7-12). The preoperative diagnosis of femoral hernia was accurate in 7 patients. Seven femoral hernias were exclusively right sided; 3 were bilateral. All 13 femoral hernias were successfully treated by a standardized transabdominal laparoscopic approach with the use of three 3.5-mm trocars. All patients were treated in a day care setting. No postoperative complications occurred. No recurrences were seen until the present time, with a mean follow-up of 31/2 years. CONCLUSIONS: Laparoscopy provides a straightforward, accurate diagnosis for the rare and often missed pediatric femoral hernias. The new technique described offers a safe and efficient minimally invasive anatomical repair of the crural orifice in children, even when not suspected preoperatively. The laparoscopic diagnosis of 13 femoral hernias from a cohort of 462 laparoscopic groin hernia repairs (2.8%) may suggest a higher prevalence rate of this unusual type of hernia in children than earlier described in literature.