Contemporary preclinical mouse models for pediatric rhabdomyosarcoma: from bedside to bench to bedside.

Background: Rhabdomyosarcoma (RMS) is the most common pediatric soft-tissue malignancy, characterized by high clinicalopathological and molecular heterogeneity. Preclinical in vivo models are essential for advancing our understanding of RMS oncobiology and developing novel treatment strategies. However, the diversity of scholarly data on preclinical RMS studies may challenge scientists and clinicians. Hence, we performed a systematic literature survey of contemporary RMS mouse models to characterize their phenotypes and assess their translational relevance. Methods: We identified papers published between 01/07/2018 and 01/07/2023 by searching PubMed and Web of Science databases. Results: Out of 713 records screened, 118 studies (26.9%) were included in the qualitative synthesis. Cell line-derived xenografts (CDX) were the most commonly utilized (n = 75, 63.6%), followed by patient-derived xenografts (PDX) and syngeneic models, each accounting for 11.9% (n = 14), and genetically engineered mouse models (GEMM) (n = 7, 5.9%). Combinations of different model categories were reported in 5.9% (n = 7) of studies. One study employed a virus-induced RMS model. Overall, 40.0% (n = 30) of the studies utilizing CDX models established alveolar RMS (aRMS), while 38.7% (n = 29) were embryonal phenotypes (eRMS). There were 20.0% (n = 15) of studies that involved a combination of both aRMS and eRMS subtypes. In one study (1.3%), the RMS phenotype was spindle cell/sclerosing. Subcutaneous xenografts (n = 66, 55.9%) were more frequently used compared to orthotopic models (n = 29, 24.6%). Notably, none of the employed cell lines were derived from primary untreated tumors. Only a minority of studies investigated disseminated RMS phenotypes (n = 16, 13.6%). The utilization areas of RMS models included testing drugs (n = 64, 54.2%), studying tumorigenesis (n = 56, 47.5%), tumor modeling (n = 19, 16.1%), imaging (n = 9, 7.6%), radiotherapy (n = 6, 5.1%), long-term effects related to radiotherapy (n = 3, 2.5%), and investigating biomarkers (n = 1, 0.8%). Notably, no preclinical studies focused on surgery. Conclusions: This up-to-date review highlights the need for mouse models with dissemination phenotypes and cell lines from primary untreated tumors. Furthermore, efforts should be directed towards underexplored areas such as surgery, radiotherapy, and biomarkers.

Implementation of Enhanced Recovery Protocols After Minimally Invasive Surgery in Children: A Multinational Survey Study Among International Pediatric Endosurgery Group and European Paediatric Surgeons' Association Members.

Aim: The aim of this multinational survey was to provide insights into individual surgeon's experience with implementation of enhanced recovery after surgery (ERAS) programs centered on minimally invasive surgery (misERAS) and to identify perceived barriers to utilization of these protocols. Methods: An online survey was conducted between July 2021 and March 2022 on behalf of the International Pediatric Endosurgery Group (IPEG) and European Paediatric Surgeons' Association (EUPSA) Research Committees. All IPEG and EUPSA members were contacted by e-mail and asked to complete an anonymous questionnaire that included 20 items. Results: Of an estimated 890 IPEG and 800 EUPSA members, 248 completed the survey (14.7%). A minority of respondents (n = 45, 18.1%) stated that misERAS protocols were followed as "utilizing specific ERAS guidelines," whereas 67.3% (n = 167) replied that they were trying to adhere to the basic ERAS principles in their practice. Almost half (n = 117, 47.2%) of the respondents stated that there was an increase in implementation of specific misERAS guidelines over the last 5 years. A lack of education and standardized protocols, lack of support and collaboration, and the existing culture leading to moderate to extreme barriers to misERAS implementation were reported by 25%-40% of respondents. Conclusions: This survey demonstrates that pediatric surgeons from the IPEG and EUPSA communities try to adhere to basic ERAS principles when performing MIS. The major barrier to misERAS implementation is profound lack of education. The fact that only 18% of respondents utilize specific institutional misERAS protocols suggests that at present, ERAS has not found its way into MIS in the broad landscape.

Pectus excavatum in motion: dynamic evaluation using real-time MRI.

OBJECTIVES: The breathing phase for the determination of thoracic indices in patients with pectus excavatum is not standardized. The aim of this study was to identify the best period for reliable assessments of morphologic indices by dynamic observations of the chest wall using real-time MRI. METHODS: In this prospective study, patients with pectus excavatum underwent morphologic evaluation by real-time MRI at 3 T between January 2020 and June 2021. The Haller index (HI), correction index (CI), modified asymmetry index (AI), and modified eccentricity index (EI) were determined during free, quiet, and forced breathing respectively. Breathing-related differences in the thoracic indices were analyzed with the Wilcoxon signed-rank test. Motion of the anterior chest wall was analyzed as well. RESULTS: A total of 56 patients (11 females and 45 males, median age 15.4 years, interquartile range 14.3-16.9) were included. In quiet expiration, the median HI in the cohort equaled 5.7 (4.5-7.2). The median absolute differences (Δ) in the thoracic indices between peak inspiration and peak expiration were ΔHI = 1.1 (0.7-1.6, p < .001), ΔCI = 4.8% (1.3-7.5%, p < .001), ΔAI = 3.0% (1.0-5.0%, p < .001), and ΔEI = 8.0% (3.0-14.0%, p < .05). The indices varied significantly during different inspiratory phases, but not during expiration (p > .05 each). Furthermore, the dynamic evaluation revealed three distinctive movement patterns of the funnel chest. CONCLUSIONS: Real-time MRI reveals patterns of chest wall motion and indicate that thoracic indices of pectus excavatum should be assessed in the end-expiratory phase of quiet expiration. KEY POINTS: • The thoracic indices in patients with pectus excavatum depend on the breathing phase. • Quiet expiration represents the best breathing phase for determining thoracic indices. • Real-time MRI can identify different chest wall motion patterns in pectus excavatum.

Pediatric Minimally Invasive Surgery-A Bibliometric Study on 30 Years of Research Activity.

Background: Pediatric minimally invasive surgery (MIS) is a standard technique worldwide. We aimed to analyze the research activity in this field. Methods: Articles on pediatric MIS (1991−2020) were analyzed from the Web of Science™ for the total number of publications, citations, journals, and impact factors (IF). Of these, the 50 most cited publications were evaluated in detail and classified according to the level of evidence (i.e., study design) and topic (i.e., surgical procedure). Results: In total, 4464 publications and 53,111 citations from 684 journals on pediatric MIS were identified. The 50 most cited papers were published from 32 institutions in the USA/Canada (n = 28), Europe (n = 19), and Asia (n = 3) in 12 journals. Four authors (USA/Europe) contributed to 26% of the 50 most cited papers as first/senior author. Hot topics were laparoscopic pyeloplasty (n = 9), inguinal hernia repair (n = 7), appendectomy, and pyloromyotomy (n = 4 each). The majority of publications were retrospective studies (n = 33) and case reports (n = 6) (IF 5.2 ± 3.2; impact index 16.5 ± 6.4; citations 125 ± 39.4). They were cited as often as articles with high evidence levels (meta-analyses, n = 2; randomized controlled trials, n = 7; prospective studies, n = 2) (IF 12.9 ± 22.5; impact index 14.0 ± 6.5; citations 125 ± 34.7; p > 0.05). Conclusions: Publications on laparoscopic pyeloplasty, inguinal hernia repair, appendectomy, and pyloromyotomy are cited most often in pediatric MIS. However, the relevant number of studies with strong evidence for the advantages of MIS in pediatric surgery is missing.

Global Development of Research on Anorectal Malformations over the Last Five Decades: A Bibliometric Analysis.

PURPOSE: Anorectal malformations (ARM) are one of the most challenging congenital malformations in pediatric surgery. We aimed to assess the research activity on ARM over the last five decades. METHODS: Data on original research publications were retrieved from the Web of Science Core Collection (1970-2020), and analyzed for countries, authors, scientific journals, and top-ten papers. Scientific quantity was assessed by the number of publications. Research quality was estimated from the number of citations, average citation rate per item (ACI), and h-index. RESULTS: A total number of 1595 articles with 19,419 citations (ACI = 12.2; h-index = 54) were identified. The annual number of publications and citations significantly increased over time (p < 0.0001). The USA (n = 386; 24.2%), Japan (n = 153; 9.6%), and China (n = 137; 8.6%) were the most productive countries; and the USA (n = 7850; ACI = 20.3; h-index = 44), Japan (n = 1937; ACI = 12.6; h-index = 21), and the Netherlands (n = 1318; ACI = 17.3; h-index = 22) were the top cited countries. Articles were preferentially published in JPS (n = 391; 24.5%), PSI (n = 181; 11.3%), and EJPS (n = 56; 3.5%). Top-ten cited papers focused on classification (n = 1), surgical technique (n = 3), associated syndromes (n = 2), postoperative outcome (n = 3), and basic research (n = 1). CONCLUSION: This bibliometric study provides valuable insights into the global development of ARM research, and shows that clinical studies and international collaborations dominate in this field.

Impact of Perioperative Absolute Neutrophil Count on Central Line-Associated Bloodstream Infection in Children With Acute Lymphoblastic and Myeloid Leukemia.

BACKGROUND: There is lack of evidence concerning safety of placement of tunneled central venous catheters (TCVCs) in neutropenic children with acute leukemias. Here, we evaluate the impact of absolute neutrophil count (ANC) at the time of TCVC placement on development of central line-associated bloodstream infections (CLABSI) in children with lymphoblastic (ALL) or myeloid leukemia (AML). MATERIALS AND METHODS: A retrospective observational study of children undergoing TCVC placement at a tertiary referral hospital between January 2000 and December 2019 was performed. Traditional and competing-risks regression models were used to estimate the effect of perioperative ANC on development of CLABSI. RESULTS: A total of 350 children (median age 6.4 [IQR: 3.1-10.9] years) underwent 498 consecutive TCVC implantations in neutropenic (n = 172, 34.5%) and non-neutropenic conditions (n = 326, 65.5%). The median length of observation per TCVC was 217.1 (IQR: 116.1-260.5) days with a total of 99,681 catheter days (CD). There were no differences in early (within first 30 days after TCVC placement) and overall CLABSI rates between neutropenic and non-neutropenic patients (HR 1.250, p = 0.502; HR 1.633, p = 0.143). We identified female sex (HR 2.640, p = 0.006) and the use of TCVC for treatment of relapsed leukemia (HR 4.347, p < 0.0001) as risk factors for early CLABSI and the use of double-lumen catheters (HR 2.607, p = 0.003) and use of TCVCs during leukemia relapse (HR 2.004, p = 0.005) for overall study period. CONCLUSION: The placement of TCVC in children with neutropenia undergoing anticancer therapy for acute leukemia is safe and not associated with an elevated rate of CLABSI.

Midgut development in rat embryos using microcomputed tomography.

The development of the mammalian gut was first described more than a century ago. Since then, it has been believed that a series of highly orchestrated developmental processes occur before the intestine achieves its final formation. The key steps include the formation of the umbilicus, the so-called "physiological herniation" of the midgut into the umbilical cord, an intestinal "rotation", and the "return of the gut" into the abdominal cavity. However, this sequence of events is predominantly based on histological sections of dissected embryos, a 2D technique with methodological limitations. For a better understanding of spatial relationships in the embryo, we utilized microcomputed tomography (µCT), a nondestructive 3D imaging method. Here, we show the detailed processes and mechanisms of intestinal development in rat embryos, including the development of the umbilicus, the formation of loops inside the umbilical coelom, and the subsequent shift of these loops into the abdominal cavity. Our 3D datasets of developing intestines will substantially advance the understanding of normal mammalian midgut embryology and offer new possibilities to reveal unknown mechanisms in the pathogenesis of congenital disorders.

20 Years of Single-Incision-Pediatric-Endoscopic-Surgery: A Survey on Opinion and Experience Among International Pediatric Endosurgery Group Members.

Aim/Background: Assessment of current role and future trends of Single-Incision-Pediatric-Endoscopic-Surgery (SIPES) in pediatric surgery among International Pediatric Endosurgery Group (IPEG) members two decades after introduction. Materials and Methods: An online survey was conducted between December 2019 and April 2020 on behalf of the IPEG Research Committee. All IPEG members were contacted by e-mail and asked to complete an anonymous questionnaire that included 39 items on SIPES. Results: One hundred eighty-four practicing pediatric surgeons completed the questionnaire from a pool of 890 IPEG members. The majority (76%) of respondents performed SIPES for more than 6 years with the following caseload per month: 1 case (31%), 2-5 cases (30%), 6-10 cases (24%), and >10 cases (17%). The four most commonly performed procedures were appendectomy (95%), Meckel diverticulectomy (55%), treatment of ovarian pathologies (43%), and U-stitch gastrostomy (40%). Complex reconstructive SIPES procedures were performed rarely. Most surgeons (95%) stated that better cosmesis is the predominant advantage of SIPES procedures. The majority of respondents (70%) felt that there is no convincing scientific evidence that SIPES offers benefits to multi-port minimally invasive procedures. Conclusion: Twenty years after introduction of SIPES, this technique has found its place in pediatric endoscopic surgery. Eighty percent of participating IPEG members of this survey apply SIPES for cases of lower complexity, such as appendectomy mainly for cosmetic reasons. The fact that 70% of respondents state that the scientific evidence for the benefits of SIPES is not convincing suggests that further studies and discussion on this technique are needed.

Vacuum-assisted closure (VAC) prevents wound dehiscence following posterior sagittal anorectoplasty (PSARP): An exploratory case-control study.

BACKGROUND: Wound dehiscence (WD) of the anocutaneous anastomosis or perineal body after posterior sagittal anorectoplasty (PSARP) is common. We aimed to evaluate the efficacy of a perineal vacuum-assisted closure (VAC) for prevention of WD following repair of anorectal malformations (ARM) with rectoperineal and rectovestibular fistula. METHODS: A retrospective dual-center case-control study of children undergoing PSARP without colostomy between 2011 and 2019 was performed. The VAC group received preoperative bowel preparation (PBP), postoperative application of a VAC, loperamide (only Location A), intravenous antibiotics (IA), and total parenteral nutrition (TPN). The non-VAC group underwent PBP, loperamide (Location A), IA, and TPN without VAC. Primary outcome was WD at the anocutaneous anastomosis or reconstructed perineal body within the first 14 days after surgery. RESULTS: The study population included 18 patients (VAC group) and 20 children (non-VAG group) with rectoperineal and rectovestibular fistula. The incidence of WD in the VAC group was 0% compared to 25% in the non-VAC group (0/18 vs. 5/20, p = 0.04). No VAC related complications occurred. CONCLUSION: Postoperative application of a VAC embedded in a perioperative treatment protocol has the potential to prevent wound dehiscence of the neoanus and reconstructed perineal body following PSARP. TYPE OF STUDY: Case-control study. LEVEL OF EVIDENCE: Level III.

Publication Trends and Global Collaborations on Esophageal Atresia Research: A Bibliometric Study.

INTRODUCTION: Research on esophageal atresia (EA) has been heavily published over the past decades. Herein, we aimed to study the quantity and quality as well as key topics in EA research with regards to global collaborations among countries and authors. MATERIALS AND METHODS: Publications on EA from 1945 to 2018 were extracted from the Web of Science core collection database. Productivity (quantity) was assessed by the number of publications. Quality was estimated from the number of citations, citation rate per item and year, h-index, and impact index. Collaborative networks were evaluated using VOSviewer. All measures were analyzed for countries, authors, and journals. The 10 most cited original articles between 1969 and 2018 in 5-year intervals (n = 100) were manually screened to assess the key points of EA research. RESULTS: A total of 2,170 publications from 85 countries published in 388 journals were identified yielding 26,755 citations, both significantly increasing over time (p < 0.001). The most productive countries and authors also accounted for high-quality publications and benefited from an active global network. The most productive journals derived from the field of pediatric surgery but accounted only for one-third of EA papers. The best cited journals were unspecific for pediatric surgery. Long-term outcome remained the most important topic in EA research, followed by surgical techniques, epidemiology, associated anomalies, perioperative complications, and postnatal management. In contrast, basic science was underrepresented. CONCLUSION: Over the past seven decades, EA publications increased tremendously. Productiveness and quality benefited from global networking. Long-term outcome remains the key interest of EA research.

An exploratory randomized controlled trial comparing wood-composite and synthetic fibreglass splint systems for the immobilization of paediatric upper limb fractures.

AIMS: This exploratory randomized controlled trial (RCT) aimed to determine the splint-related outcomes when using the novel biodegradable wood-composite splint (Woodcast) compared to standard synthetic fibreglass (Dynacast) for the immobilization of undisplaced upper limb fractures in children. METHODS: An exploratory RCT was performed at a tertiary paediatric referral hospital between 1 June 2018 and 30 September 2019. The intention-to-treat population consisted of 170 patients (mean age 8.42 years (SD 3.42); Woodcast (WCG), n = 84, 57 male (67.9%); Dynacast (DNG), n = 86, 58 male (67.4%)). Patients with undisplaced upper limb fractures were randomly assigned to WCG or DNG treatment groups. Primary outcome was the stress stability of the splint material, defined as absence of any deformations or fractures within the splint during study period. Secondary outcomes included patient satisfaction and medical staff opinion. Additionally, biomechanical and chemical analysis of the splint samples was carried out. RESULTS: Of the initial 170 patients, 168 (98.8%) completed at least one follow-up, and were included for analysis of the primary endpoint. Both treatment groups were well-matched regarding to age, sex, and type and localization of the fracture. Splint breakage occurred in three patients (3.6%; 95% confidence interval (CI), 0.007% to 0.102%) in the WCG and in three children (3.5%, 95% CI 0.007% to 0.09%) in the DNG (p > 0.99). The incidence of splint-related adverse events did not differ between the WCG (n = 21; 25.0%) and DNG (n = 24; 27.9%; p = 0.720). Under experimental conditions, the maximal tensile strength of Dynacast samples was higher than those deriving from Woodcast (mean 15.37 N/mm² (SD 1.37) vs 10.75 N/mm² (SD 1.20); p = 0.002). Chemical analysis revealed detection of polyisocyanate-prepolymer in Dynacast and polyester in Woodcast samples. CONCLUSION: Splint-related adverse events appear similar between WCG and DNG treatment groups during the treatment of undisplaced forearm fractures. Cite this article: Bone Joint J 2020;102-B(10):1405-1411.

Safety of tunneled central venous catheters in pediatric hematopoietic stem cell recipients with severe primary immunodeficiency diseases.

Tunneled central venous catheters (TCVCs) provide prolonged intravenous access for pediatric patients with severe primary immunodeficiency disease (PID) undergoing hematopoietic stem cell transplantation (HSCT). However, little is known about the epidemiology and clinical significance of TCVC-related morbidity in this particular patient group. We conducted the retrospective analysis of patients with severe PID who received percutaneous landmark-guided TCVC implantation prior to HSCT. We analyzed 92 consecutive TCVC implantations in 69 patients (median [interquartile range] age 3.0 [0-11] years) with severe combined immune deficiency (n = 39, 42.4%), chronic granulomatous disease (n = 17, 18.4%), and other rare PID syndromes (n = 36, 39.2%). The median length of TCVC observation was 144.1 (85.5-194.6) days with a total of 14,040 catheter days at risk (cdr). The overall rate of adverse events during catheter insertion was 17.4% (n = 16) and 25.0% during catheter dwell period (n = 23, catheter risk [CR] per 1000 cdr = 1.64). The most common complication was TCVC-related infection with an overall prevalence of 9.8% (n = 9, CR = 0.64), followed by late dislocation (n = 6, 6.5%, CR = 0.43), early dislocation (n = 4, 4.3%) and catheter dysfunction (n = 4, 4.3%, CR = 0.28). TCVCs are safe in children with severe PID undergoing HSCT with relatively low rates of TCVC-related infection.

A scientometric analysis of neuroblastoma research.

BACKGROUND: Thousands of research articles on neuroblastoma have been published over the past few decades; however, the heterogeneity and variable quality of scholarly data may challenge scientists or clinicians to survey all of the available information. Hence, holistic measurement and analyzation of neuroblastoma-related literature with the help of sophisticated mathematical tools could provide deep insights into global research performance and the collaborative architectonical structure within the neuroblastoma scientific community. In this scientometric study, we aim to determine the extent of the scientific output related to neuroblastoma research between 1980 and 2018. METHODS: We applied novel scientometric tools, including Bibliometrix R package, biblioshiny, VOSviewer, and CiteSpace IV for comprehensive science mapping analysis of extensive bibliographic metadata, which was retrieved from the Web of ScienceTM Core Collection database. RESULTS: We demonstrate the enormous proliferation of neuroblastoma research during last the 38 years, including 12,435 documents published in 1828 academic journals by 36,908 authors from 86 different countries. These documents received a total of 316,017 citations with an average citation per document of 28.35 ± 7.7. We determine the proportion of highly cited and never cited papers, "occasional" and prolific authors and journals. Further, we show 12 (13.9%) of 86 countries were responsible for 80.4% of neuroblastoma-related research output. CONCLUSIONS: These findings are crucial for researchers, clinicians, journal editors, and others working in neuroblastoma research to understand the strengths and potential gaps in the current literature and to plan future investments in data collection and science policy. This first scientometric study of global neuroblastoma research performance provides valuable insight into the scientific landscape, co-authorship network architecture, international collaboration, and interaction within the neuroblastoma community.

The outcome of Bishop-Koop procedure compared to divided stoma in neonates with meconium ileus, congenital intestinal atresia and necrotizing enterocolitis.

To determine the potential value and suitability of Bishop-Koop procedure (BK) compared to divided stoma (DS) in neonates with meconium ileus (MI), congenital intestinal atresia (CIA), and necrotizing enterocolitis (NEC).A retrospective data collection from 2000 to 2019 on neonates undergoing BK and DS formation and closure for MI, CIA, and NEC was conducted. Ostomy related complications following both procedures were analyzed.One hundred two consecutive patients managed with a BK (n = 57, 55.8%) and DS (n = 45, 44.2%) for MI (n = 38, 37.2%), CIA (n = 31, 30.5%), and NEC (n = 33, 32.3%) were analyzed. Mean operating time for ostomy creation did not differ significantly between BK and DS groups (156 ±â€Š54 vs 135 ±â€Š66.8 min, P = .08). The prevalence of stoma-related complications following BK and DS formation was 8.7% and 31.1%, respectively (P = .005). The complication rate after BK and DS closure was 3.5% and 6.7%, respectively (P = .65). The operating time for ostomy reversal and length of hospital stay after stoma closure were significantly shorter in BK group (82.2 ±â€Š51.4 vs 183 ±â€Š84.5 min and 5.5 ±â€Š2.7 vs 11.3 ±â€Š3.9 days, P < .001).BK procedure is safe, reliable, and suitable technique in neonatal surgery with low complications rate following ostomy creation as well as shorter operating time and length of hospital stay after ostomy closure compared to DS ostomies. Surgeons should keep this technique as an alternative approach in their repertoire.

Duodenal Atresia Repair Using a Miniature Stapler Compared to Laparoscopic Hand-Sewn and Open Technique.

Background: Laparoscopic duodenal atresia (DA) repair is a demanding procedure that requires performing a watertight anastomosis in a small working space. Drawbacks of the approach have been high leakage rates and long operative times. In this article, we evaluate our initial experience with DA repair using a laparoscopic miniature stapler (LA-MS) and compared outcomes with a historic cohort of laparoscopic hand-sewn (LA-HS) and open repairs (ORs). Materials and Methods: A retrospective analysis of all patients who underwent surgery for DA at our two centers between January 2010 and April 2018 was performed. Demographics, comorbidities, intra- and postoperative data, and outcome parameters were evaluated and statistically analyzed. Results: DA repair was performed in 44 patients. Ten patients underwent laparoscopic DA repair using an MS, 21 patients laparoscopic repair with HS anastomosis, and 13 patients underwent OR. Median age and weight at surgery was 13.5 days (range: 2-173) and 3300 g (range: 1630-5600) in the LA-MS group, 4 days (range: 2-269) and 2750 g (range: 1700-4095) in the LA-HS group and 4 days (range: 1-17) and 2222 g (range: 1520-3590) in the OR group, respectively. Mean operative time was significantly shorter in the laparoscopic stapled group compared with LA-HS group (145 ± 37 minutes (range: 97-217) versus 201 ± 47 minutes (range: 119-275), P < .004). Duodenojejunostomy was performed more frequently in the laparoscopic stapled group compared with the open procedure (P = .008). Overall complication rate was similar between groups. Time to initiation of feeds and time to full feeds were significantly shorter in the laparoscopic stapled group compared with the open approach (5 versus 11.9 days, P = .041 and 14.5 versus 24.4 days, P = .020). Conclusion: Laparoscopic DA repair using an MS is a novel, safe, and feasible technique that was associated with significantly shorter operating times than HS laparoscopic DA repair. Owing to its simplicity, this technique has the potential to become the new standard of care.

CHL1 and NrCAM are Primarily Expressed in Low Grade Pediatric Neuroblastoma.

BACKGROUND: Neural cell adhesion molecules like close homolog of L1 protein (CHL1) and neuronal glia related cell adhesion molecule (NrCAM) play an important role in development and regeneration of the central nervous system. However, they are also associated with cancerogenesis and progression in adult malignancies, thus gain increasing importance in cancer research. We therefore studied the expression of CHL1 and NrCAM according to the course of disease in children with neuroblastoma. METHODS: CHL1 and NrCAM expression levels were histologically assessed by tissue microarrays from surgically resected neuroblastoma specimens of 56 children. Expression of both markers was correlated to demographics as well as clinical data including metastatic dissemination and survival. RESULTS: CHL1 was expressed in 9% and NrCAM in 51% of neuroblastoma tissue samples. Expression of CHL1 was higher in patients with low Hughes grade 1a/b (p=0.01). NrCAM was more often detected in patients with a low International Staging System (INSS) score 1/2 (p=0.04). CONCLUSION: CHL1 and NrCAM expression was associated with low-grade pediatric neuroblastoma. These adhesion molecules may play a role in early tumor development of neuroblastoma.

Homemade Glove Port for Single-Incision Pediatric Endosurgery (SIPES) Appendectomy-How We Do It.

Introduction Single-incision pediatric endosurgery (SIPES) for the treatment of acute appendicitis in children has recently gained popularity due to its advantages including minimization of postoperative scars or less incisional pain. The principal disadvantages of SIPES include the limited degrees of freedom of movement and high health care costs. To overcome these issues, some surgeons have reported to use noncommercial ports for SIPES appendectomy. Case Report In this report, we present a case of a 10-year-old female patient with acute appendicitis undergoing SIPES appendectomy using own homemade glove port and straight rigid instruments. Conclusion SIPES appendectomy using the glove port is a low-cost alternative to commercially available port systems. It is easy to set up and use.

Outcome of landmark-guided percutaneously inserted tunneled central venous catheters in infants and children under 3 years with cancer.

BACKGROUND: There is a paucity of information on procedural and long-term outcomes of tunneled central venous catheters (TCVC) in infants and children younger than 3 years undergoing anticancer therapy. This study aims to evaluate the success, safety, and complications leading to surgical revision or premature removal of TCVC in this particular patient group. METHODS: The clinical course of pediatric patients with percutaneous inserted TCVC, including Groshong (GC) and Hickman/Broviac (HB) catheters, has been analyzed retrospectively. The data analysis includes patient and device characteristics, adverse events during insertion, and dwell period complications. RESULTS: A consecutive series of 238 children undergoing implantation of 273 TCVC, including 148 (54.2%) GC and 125 (45.8%) HB catheters, with a total of 38,209 catheter days at risk (cdr) were reviewed. The patient cohort consisted of 65 (23.8%) infants, 77 (28.2%) children aged 1-2 years, and 131 (48.0%) aged 2-3 years. The overall rate of adverse events during catheter insertion was 12.8% (n = 35) with no differences between age groups or devices. The overall rate of long-term complication was 28.2% (n = 77, catheter risk [CR] per 1,000 cdr = 1.75), with the highest prevalence in infants (P = 0.01). The most common complication was late dislocation (n = 24, 8.8%, CR = 0.47), followed by early dislocation (n = 20, 7.3%) and infection (n = 18, 7.4%, CR = 0.42). CONCLUSION: Percutaneous landmark-guided insertion of TCVC in neonates and small children with cancer is safe. Patterns of long-term complications are different from those for older children and should be prevented through appropriate management.