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Severe congenital neutropenia (SCN) and Clostridium septicum myonecrosis is an uncommon and life-threatening association requiring urgent combined aggressive medical and surgical management. We report 2 cases of SCN (1 with known Kostmann's syndrome and 1 not known at presentation to have a congenital neutropenic disorder but subsequently received a diagnosis of cyclic neutropenia) who presented with spontaneous C septicum myonecrosis. The cases highlight the importance of response to recombinant human granulocyte colony-stimulating factor in obtaining a satisfactory outcome for these patients. Early, empirical use of recombinant human granulocyte colony-stimulating factor in patients who are suspected of having a congenital neutropenia and who present with life-threatening sepsis is recommended. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate local failure (LF) and toxicity after intraoperative radiation therapy (IORT) in pediatric solid tumors (ST)., Methods: A single-institution retrospective study of 96 pediatric patients (108 applications) with ST treated from 1995 to 2022 with IORT. LF was calculated via cumulative incidence function and overall survival (OS) by Kaplan-Meier method, both from the day of surgery., Results: Median age at time of IORT was 8 years (range: 0.8-20.9 years). Median follow-up for all patients and surviving patients was 16 months and 3 years, respectively. The most common histologies included rhabdomyosarcoma (n = 42), Ewing sarcoma (n = 10), and Wilms tumor (n = 9). Most (95%) received chemotherapy, 37% had prior external beam radiation therapy to the site of IORT, and 46% had a prior surgery for tumor resection. About half (54%) were treated with upfront IORT to the primary tumor due to difficult circumstances such as very young age or challenging anatomy. The median IORT dose was 12 Gy (range: 4-18 Gy), and median area treated was 24 cm 2 (range: 2-198 cm 2 ). The cumulative incidence of LF was 17% at 2 years and 23% at 5 years. Toxicity from IORT was reasonable, with postoperative complications likely related to IORT seen in 15 (16%) patients., Conclusion: Our study represents the largest and most recent analysis of efficacy and safety of IORT in pediatric patients with ST. Less than one quarter of all patients failed locally with acceptable toxicities. Overall, IORT is an effective and safe technique to achieve local control in patients with challenging circumstances., (© 2024 Wiley Periodicals LLC.)

Purpose: To evaluate outcomes after intraoperative radiation therapy (IORT) in high-risk neuroblastoma (NB), including local control, overall survival, and toxicity., Methods and Materials: This was a single institution retrospective study of 92 pediatric patients with NB treated with IORT from 1995 to 2022. Each IORT application was considered a separate event for a total of 110 sites treated. Local failure was calculated using the cumulative incidence function and survival by Kaplan-Meier method from the day of surgery., Results: All patients had high-risk relapsed or treatment refractory disease. Median age was 6 years (range, 2-34 years). Median follow-up for all patients and surviving patients was 16 months and 4 years, respectively. All patients previously received chemotherapy, 93% had prior external beam radiation therapy to the site of IORT (median dose, 21.6 Gy; range, 10-36 Gy), and 94% had a prior surgery for tumor resection. The median IORT dose was 12 Gy (range, 8-18 Gy) and median area treated was 18 cm 2 (range, 2.5-60 cm 2 ). The cumulative incidence of local failure was 23% at 2 years and 29% at 5 years. The overall survival (OS) was 44% at 2 years and 29% at 5 years. Local failure after IORT was associated with worse OS (hazard ratio, 1.74; 95% CI, 1.07-2.84; P = .0267). Toxicity from IORT was rare, with postoperative complications likely related to IORT seen in 7 (8%) patients., Conclusions: Our study represents the largest, most recent analysis of the efficacy and safety of IORT in patients with relapsed or refractory NB. Less than one-third of patients failed locally at 5 years, and achieving local control affected overall survival. Minimal toxicities directly related to IORT were observed. Overall, IORT is an effective and safe technique to achieve local control in high-risk relapsed or refractory neuroblastoma., Competing Interests: Disclosures Dana Casey reports participating in the EMD Serono advisory board rhabdomyosarcoma meeting. Shakeel Modak reports earnings from YMABs and Innervate, however not research funds. He participates on the Data Safety Monitoring Board or Advisory Board for Kymera, EUSA, and US WorldMeds. Kathryn Tringale reports receiving the Hopper-Belmont Foundation Inspiration Award, RSNA Resident Research Grant (unrelated), NCCN 2023 Fellow Award (unrelated), and the ASH Abstract Achievement Award (unrelated)., (Copyright © 2024 American Society for Radiation Oncology. Published by Elsevier Inc. All rights reserved.)

Background: Neuroblastomas rarely occur as primary tumors in the cervical region. Therefore, very little has been reported regarding treatment strategies, complications, and outcomes of these cervical neuroblastomas. The goal of this study is to review the presentation, management, and outcomes of all primary cervical pediatric neuroblastoma cases at a single tertiary care center., Methods: A retrospective cohort review of all neuroblastoma patients treated at a single center were performed. All patients with primary cervical neuroblastoma were reviewed for demographic information, tumor characteristics, treatment, and outcomes., Results: Thirty (1.8%) patients were found to have undergone treatment for cervical neuroblastoma tumors diagnosed on average at 2.1 years old. Most presented with a swollen neck/palpable mass ± Horner's syndrome. Based on features including tumor staging, N-myc proto-oncogene protein (MYCN) amplification status, histology, most were deemed intermediate or high risk. Treatment strategies centered around chemotherapeutic regimens with surgery when possible as well as various adjuvant treatments including radiation therapy, immunotherapy, bone marrow transplant, and a neuroblastoma vaccine. Ten (33.3%) of patients experienced treatment-related complications and four (13.3%) died as a result of their disease progression. All four patients were high-risk patients, two of which had MYCN amplification., Conclusion: Cervical neuroblastomas generally have favorable outcomes. These tumors can be treated effectively with chemotherapy and surgical intervention with various adjuvant therapies. MYCN amplification and higher stage disease presentation contribute to worse outcomes., (© 2023 Wiley Periodicals LLC.)

Patients with stage 4N neuroblastoma (distant metastases limited to lymph nodes) stand out as virtually the only survivors of high-risk neuroblastoma (HR-NB) before myeloablative therapy (MAT) and immunotherapy with anti-G D2 monoclonal antibodies (mAbs) became standard. Because no report presents more recent results with 4N, we analyzed our large 4N experience. All 51 pediatric 4N patients (<18 years old) diagnosed 1985 to 2021 were reviewed. HR-NB included MYCN-nonamplified 4N diagnosed at age ≥18 months and MYCN-amplified 4N. Among 34 MYCN-nonamplified high-risk patients, 20 are relapse-free 1.5+ to 37.5+ (median 12.5+) years post-diagnosis, including 13 without prior MAT and 5 treated with little (1 cycle; n = 2) or no mAb (n = 3), while 14 patients (7 post-MAT, 8 post-mAbs) relapsed (all soft tissue). Of 15 MYCN-amplified 4N patients, 7 are relapse-free 2.1+ to 26.4+ (median 11.6+) years from the start of chemotherapy (all received mAbs; 3 underwent MAT) and 4 are in second remission 4.2+ to 21.8+ years postrelapse (all soft tissue). Statistical analyses showed no significant association of survival with either MAT or mAbs for MYCN-nonamplified HR-NB; small numbers prevented these analyses for MYCN-amplified patients. The two patients with intermediate-risk 4N (14-months-old) are relapse-free 7+ years postresection of primary tumors; distant disease spontaneously regressed. The natural history of 4N is marked by NB confined to soft tissue without early relapse in bones or bone marrow, where mAbs have proven efficacy. These findings plus curability without MAT, as seen elsewhere and at our center, support consideration of treatment reduction for MYCN-nonamplified 4N., (© 2023 UICC.)

Magnetic resonance-guided high intensity focused ultrasound (MRgHIFU) is an established method for producing localized hyperthermia. Given the real-time imaging and acoustic energy modulation, this modality enables precise temperature control within a defined area. Many thermal applications are being explored with this noninvasive, nonionizing technology, such as hyperthermia generation, to release drugs from thermosensitive liposomal carriers. These drugs can include chemotherapies such as doxorubicin, for which targeted release is desired due to the dose-limiting systemic side effects, namely cardiotoxicity. Doxorubicin is a mainstay for treating a variety of malignant tumors and is commonly used in relapsed or recurrent rhabdomyosarcoma (RMS). RMS is the most common solid soft tissue extracranial tumor in children and young adults. Despite aggressive, multimodal therapy, RMS survival rates have remained the same for the past 30 years. To explore a solution for addressing this unmet need, an experimental protocol was developed to evaluate the release of thermosensitive liposomal doxorubicin (TLD) in an immunocompetent, syngeneic RMS mouse model using MRgHIFU as the source of hyperthermia for drug release.

Background: Survival of Wilms tumor (WT) is > 90% in high-resource settings but < 30% in low-resource settings. Adapting a standardized surgical approach to WT is challenging in low-resource settings, but a local control strategy is crucial to improving outcomes., Objective: Provide resource-sensitive recommendations for the surgical management of WT., Methods: We performed a systematic review of PubMed and EMBASE through July 7, 2020, and used the GRADE approach to assess evidence and recommendations., Recommendations: Initiation of treatment should be expedited, and surgery should be done in a high-volume setting. Cross-sectional imaging should be done to optimize preoperative planning. For patients with typical clinical features of WT, biopsy should not be done before chemotherapy, and neoadjuvant chemotherapy should precede surgical resection. Also, resection should include a large transperitoneal laparotomy, adequate lymph node sampling, and documentation of staging findings. For WT with tumor thrombus in the inferior vena cava, neoadjuvant chemotherapy should be given before en bloc resection of the tumor and thrombus and evaluation for viable tumor thrombus. For those with bilateral WT, neoadjuvant chemotherapy should be given for 6-12 weeks. Neither routine use of complex hilar control techniques during nephron-sparing surgery nor nephron-sparing resection for unilateral WT with a normal contralateral kidney is recommended. When indicated, postoperative radiotherapy should be administered within 14 days of surgery. Post-chemotherapy pulmonary oligometastasis should be resected when feasible, if local protocols allow omission of whole-lung irradiation in patients with nonanaplastic histology stage IV WT with pulmonary metastasis without evidence of extrapulmonary metastasis., Conclusion: We provide evidence-based recommendations for the surgical management of WT, considering the benefits/risks associated with limited-resource settings., (© 2022 Wiley Periodicals LLC.)

Background: Barriers in access to pediatric surgical care are common in low- and middle-income countries (LMICs), but also exist in high-income countries, particularly in urban and rural areas., Methods: This article describes "Disparities in Access to Care"-held within the Social Injustice Symposium at the 2020 American Pediatric Surgical Association (APSA) Annual Meeting., Results: This symposium outlined disparities in access to care, illustrated by examples from pediatric trauma and neonatal surgery in U.S. urban, U.S. rural, and non-U.S. global locations (LMICs). Geographic and financial challenges were common to families from the rural U.S. and LMICs. In contrast, families in U.S. urban settings generally do not face geographic barriers, but are often economically and racially diverse and many face complex societal factors leading to poor outcomes. Systemic processes must be changed to improve pediatric surgical health outcomes., Conclusion: A comprehensive health system with an equal emphasis on supportive care and surgery is required in all settings. Global collaboration and partnerships can provide an avenue for advocacy and strategic innovation to improve quality of care., Level of Evidence: Ⅴ., Competing Interests: Declaration of Competing Interest The authors have no competing interests to declare., (Copyright © 2022 Elsevier Inc. All rights reserved.)

In the United States, more than 10 000 cancers occur annually in children aged 0-14 years, and more than 5000 in adolescents aged 15-19. In the last 50 years, significant advances have been made in imaging, molecular pathology, stage and risk assessment, surgical approach, multidisciplinary treatment, and survival for pediatric solid tumors (particularly neuroblastoma, Wilms tumor, rhabdomyosarcoma, and hepatoblastoma). Moreover, the molecular driver for fibrolamellar hepatocellular carcinoma, which occurs in adolescence and young adulthood, has been identified., (© 2022 Wiley Periodicals LLC.)

Most children with tumors will require one or more surgical interventions as part of the care and treatment, including making a diagnosis, obtaining adequate venous access, performing a surgical resection for solid tumors (with staging and reconstruction), performing procedures for cancer prevention and its late effects, and managing complications of treatment; all with the goal of improving survival and quality of life. It is important for surgeons to adhere to sound pediatric surgical oncology principles, as they are closely associated with improved local control and survival. Unfortunately, there is a significant disparity in survival rates in low and middle income countries, when compared to those from high income countries. The International Society of Paediatric Surgical Oncology (IPSO) is the leading organization that deals with pediatric surgical oncology worldwide. This organization allows experts in the field from around the globe to gather and address the surgical needs of children with cancer. IPSO has been invited to contribute surgical guidance as part of the World Health Organization Initiative for Childhood Cancer. One of our goals is to provide surgical guidance for different scenarios, including those experienced in High- (HICs) and Low- and Middle-Income Countries (LMICs). With this in mind, the following guidelines have been developed by authors from both HICs and LMICs. These have been further validated by experts with the aim of providing evidence-based information for surgeons who care for children with cancer. We hope that this initiative will benefit children worldwide in the best way possible. Simone Abib, IPSO President Justin T Gerstle, IPSO Education Committee Chair Chan Hon Chui, IPSO Secretary., (© the authors; licensee ecancermedicalscience.)

Abstract: Congenital diaphragmatic hernia (CDH) is associated with high early mortality. However, advances in newborn care have led to improved survival into adolescence and early adulthood. We report a 17-year-old adolescent boy with recurrent small bowel obstruction after CDH repair at 2 days of age. Emergency physicians should be aware of late complications after neonatal CDH repair., Competing Interests: Disclosure: The authors declare no conflicts of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Desmoplastic small round cell tumor (DSRCT) is characterized by the EWSR1-WT1 t(11;22) (p13:q12) translocation. Few additional putative drivers have been identified, and research has suffered from a lack of model systems. Next-generation sequencing (NGS) data from 68 matched tumor-normal samples, whole-genome sequencing data from 10 samples, transcriptomic and affymetrix array data, and a bank of DSRCT patient-derived xenograft (PDX) are presented. EWSR1-WT1 fusions were noted to be simple, balanced events. Recurrent mutations were uncommon, but were noted in TERT (3%), ARID1A (6%), HRAS (5%), and TP53 (3%), and recurrent loss of heterozygosity (LOH) at 11p, 11q, and 16q was identified in 18%, 22%, and 34% of samples, respectively. Comparison of tumor-normal matched versus unmatched analysis suggests overcalling of somatic mutations in prior publications of DSRCT NGS data. Alterations in fibroblast growth factor receptor 4 ( FGFR4 ) were identified in 5 of 68 (7%) of tumor samples, whereas differential overexpression of FGFR4 was confirmed orthogonally using 2 platforms. PDX models harbored the pathognomic EWSR1-WT1 fusion and were highly representative of corresponding tumors. Our analyses confirm DSRCT as a genomically quiet cancer defined by the balanced translocation, t(11;22)(p13:q12), characterized by a paucity of secondary mutations but a significant number of copy number alterations. Against this genomically quiet background, recurrent activating alterations of FGFR4 stood out, and suggest that this receptor tyrosine kinase, also noted to be highly expressed in DSRCT, should be further investigated. Future studies of DSRCT biology and preclinical therapeutic strategies should benefit from the PDX models characterized in this study. IMPLICATIONS: These data describe the general quiescence of the desmoplastic small round cell tumor (DSRCT) genome, present the first available bank of DSRCT model systems, and nominate FGFR4 as a key receptor tyrosine kinase in DSRCT, based on high expression, recurrent amplification, and recurrent activating mutations., (©2021 American Association for Cancer Research.)

Introduction: The American Pediatric Surgical Association (APSA) travel fellowship was established in 2013 to allow pediatric surgeons from low- and middle-income countries to attend the APSA annual meeting. Travel fellows also participated in various clinical and didactic learning experiences during their stay in North America., Methods: Previous travel fellows completed a survey regarding their motivations for participation in the program, its impact on their practice in their home countries, and suggestions for improvement of the fellowship., Results: Eleven surgeons participated in the travel fellowship and attended the annual APSA meetings in 2013-2018. The response rate for survey completion was 100%. Fellows originated from 9 countries and 3 continents and most fellows worked in government practice (n=8, 73%). Nine fellows (82%) spent >3 weeks participating in additional learning activities such as courses and clinical observerships. The most common reasons for participation were networking (n=11, 100%), learning different ways of providing care (n=10, 90.9%), new procedural techniques (n=9, 81.8%), exposure to a different medical culture (n=10, 90.9%), and engaging in research (n=8, 72.7%). Most of the fellows participated in a structured course: colorectal (n= 6, 55%), laparoscopy (n=2, 18%), oncology (n=2, 18%), leadership skills (n=1, 9%), and safety and quality initiatives (n=1, 9%). Many fellows participated in focused clinical mentorships: general pediatric surgery (n=9, 82%), oncology (n=5, 45%), colorectal (n=3, 27%), neonatal care (n=2, 18%) and laparoscopy (n=2, 18%). Upon return to their countries, fellows reported that they were able to improve a system within their hospital (n=7, 63%), expand their research efforts (n=6, 54%), or implement a quality improvement initiative (n=6, 54%)., Conclusions: The APSA travel fellowship is a valuable resource for pediatric surgeons in low- and middle-income countries. After completion of these travel fellowships, the majority of these fellows have implemented important changes in their hospital's health systems, including research and quality initiatives, to improve pediatric surgical care in their home countries., Level of Evidence: This is not a clinical study. Therefore, the table that lists levels of evidence for "treatment study", "prognosis study", "study of diagnostic test" and "cost effectiveness study" does not apply to this paper., (Copyright © 2020 Elsevier Inc. All rights reserved.)

The spectrum of germline predisposition in pediatric cancer continues to be realized. Here we report 751 solid tumor patients who underwent prospective matched tumor-normal DNA sequencing and downstream clinical use (clinicaltrials.gov NCT01775072). Germline pathogenic and likely pathogenic (P/LP) variants were reported. One or more P/LP variants were found in 18% (138/751) of individuals when including variants in low, moderate, and high penetrance dominant or recessive genes, or 13% (99/751) in moderate and high penetrance dominant genes. 34% of high or moderate penetrance variants were unexpected based on the patient's diagnosis and previous history. 76% of patients with positive results completed a clinical genetics visit, and 21% had at least one relative undergo cascade testing as a result of this testing. Clinical actionability additionally included screening, risk reduction in relatives, reproductive use, and use of targeted therapies. Germline testing should be considered for all children with cancer.

Background: Constitutional or somatic mosaic epimutations are increasingly recognized as a mechanism of gene dysregulation resulting in cancer susceptibility. Beckwith-Wiedemann syndrome is the cancer predisposition syndrome most commonly associated with epimutation and is extremely variable in its phenotypic presentation, which can include isolated tumors. Because to the authors' knowledge large-scale germline DNA sequencing studies have not included methylation analysis, the percentage of pediatric cancer predisposition that is due to epimutations is unknown., Methods: Germline methylation testing at the 11p15.5 locus was performed in blood for 24 consecutive patients presenting with hepatoblastoma (3 patients) or Wilms tumor (21 patients)., Results: Six individuals with Wilms tumor and 1 patient with hepatoblastoma were found to have low-level gain of methylation at imprinting control 1, and a child with hepatoblastoma was found to have loss of methylation at imprinting control 2. The loss of methylation at imprinting control 2 was found to be maternally inherited, despite not being associated with any detectable genomic alteration., Conclusions: Overall, 33% of patients (8 of 24 patients) with Wilms tumor or hepatoblastoma were found to have an epigenetic susceptibility that was detectable in the blood. It is interesting to note that low-level gain of methylation at imprinting control 1 predominantly was detected in females with bilateral Wilms tumors. Further studies in larger cohorts are needed to determine the efficacy of testing all patients with Wilms tumor or hepatoblastoma for 11p15.5 epimutations in the blood as part of DNA analysis because this hallmark of predisposition will not be detected by sequencing-based approaches and detecting a cancer predisposition may modify treatment., (© 2020 American Cancer Society.)

Magnetic resonance-guided high-intensity focused ultrasound (MRgHIFU) is a novel treatment for neuroblastoma using ultrasound-induced thermal ablation with real-time MR thermometry. It is unclear which patients would be amenable to MRgHIFU given the retroperitoneal location of many neuroblastomas within the smaller pediatric abdomen. In addition, planning relies on MR scans, which are not routine in the standard pediatric neuroblastoma workup. This study sought to demonstrate that neuroblastomas are targetable with MRgHIFU and available computed tomographic imaging could be utilized for MRgHIFU virtual treatment. Cross-sectional images of 88 pediatric abdominal neuroblastoma patients were retrospectively processed with custom software to be made compatible with the Sonalleve MRgHIFU platform. Targetability measured percent treatment to lesion volume, within adequate safety margins from critical structures. All images were successfully converted into treatment planning files. Median lesion size was 191±195 cm and depth was 29±17 mm. Up to 78 (85%) patients had targetable lesions with a median targetable volume of 15% and ranging up to 79%. Targetability was highest in superficial, right upper quadrant lesions >200 cm, but limited by proximity to bowel and ribs. This study demonstrates the capacity for MRgHIFU to potentially treat the majority of abdominal neuroblastomas and the feasibility of using computed tomographic images for MRgHIFU virtual treatment planning.

Purpose: The impact of the extent of surgical resection including nephrectomy for high-risk neuroblastoma patients is controversial. In this study, we compared the renal late effects and long-term survival for patients who underwent kidney-sparing surgery (KSS) versus concurrent ipsilateral nephrectomy (CIN) for high-risk, intraabdominal neuroblastoma (HRIN)., Methods: A retrospective analysis of patients diagnosed with HRIN between Jan 1998 and Dec 2008 in a tertiary referral center was performed. Demographics, preoperative features, surgical resection extent and outcomes were analyzed., Results: Of 58 patients who underwent surgical management of HRIN, 6 underwent CIN and 52 underwent KSS. Renal image-defined risk factors (IDRFs) were more common in patients who underwent CIN. Operating time was longer and EBL higher in CIN patients. There was no difference in recurrence or overall survival between the groups. Estimated GFR (eGFR) was comparable between the groups preoperatively, but was reduced postoperatively and at long-term follow-up in patients who underwent CIN., Conclusion: Compared to KSS, CIN is not associated with an increase in local recurrence or inferior survival but does lead to reduced kidney function (eGFR of 90 ml/min/1.73 m 2 for CIN versus 127 ml/min/1.73 m 2 for KSS, p = 0.03) but without significant impact on clinical outcome., Levels of Evidence: III (Retrospective comparative study)., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Introduction: Ventriculoperitoneal shunts (VPSs) are the mainstay of treatment of hydrocephalus but have frequent complications including shunt failure and infection. There has been no comparison of laparoscopic versus open primary VPS insertion in children. We hypothesized that laparoscopic VP shunt insertion may improve patient outcomes., Methods: A prospectively-maintained, externally-validated database of pediatric patients who underwent VPS insertion at a single center between 2012 and 2016 was reviewed. Outcomes including subsequent revisions, shunt infections, operative time, and hospital stay between open and laparoscopic groups were compared., Results: 210 patients underwent VPS insertion - 41 laparoscopically and 169 open. Operative time was longer for laparoscopic insertions. There was no difference in shunt infections, complications or length of stay. There was no difference between overall revisions or in confirmed peritoneal obstructions in the laparoscopic (12%) versus open VPS insertions (5%), p = 0.13., Conclusions: This first cohort analysis of laparoscopic versus open VPS insertion in pediatric patients indicates no difference in confirmed peritoneal obstructions. With increasing use of laparoscopic placement in some centers, it remains important to elucidate if there is a subset of pediatric patients who might benefit from this technique; possible candidates may be those who are overweight/obese or have had previous intra-abdominal surgery., Level of Evidence: III - Retrospective cohort study., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Bone conduction headphones (Fig. 1) offer the unique ability to provide auditory information to the user without obstructing external sounds. We apply this technology to robotic surgery to provide the surgeon with force feedback information with minimal distraction. The device is evaluated by pairing it with a force sensor that is attached to a suture pad. Four participants were tasked to complete 25 sutures on the suture pad while either receiving no feedback or audio, visual, or combined feedback that represents the magnitude of their applied force. Trials performed with bone conducting headphones had noticeable improvements compared to previous trials without feedback, while the most noticeable improvements were observed for cases with both visual and auditory feedback. Auditory feedback may have an important role in a robotic surgery setting and bone conduction headphones may enable this form of feedback with minimal distraction.

Background: The global burden of pediatric surgical conditions continues to remain inadequately addressed, particularly in low- and middle-income countries. Among the many factors contributing to this gap are a lack of access to care secondary to resource shortages and inequitable distribution, underfinancing of healthcare systems, poor quality of care, and contextual challenges such as natural disasters and conflict. The relative contribution of these and other factors varies widely by region and even with countries of a region., Methods: This review seeks to discuss the heterogeneity of global pediatric surgery and offer recommendations for addressing the barriers to high-quality pediatric surgical care throughout the world., Results: There is significant heterogeneity in pediatric surgical challenges, both between regions and among countries in the same region, although data are limited. This heterogeneity can reflect differences in demographics, epidemiology, geography, income level, health spending, historical health policies, and cultural practices, among others., Conclusion: Country-level research and stakeholder engagement are needed to better understand the heterogeneity of local needs and drive policy changes that contribute to sustainable reforms. Key to these efforts will be improved financing, access to and quality of pediatric surgical care.

Purpose: Multifocal hepatoblastoma (HB) is often treated with total hepatectomy and transplantation owing to concerns of surgical resectability, local recurrence, and/or metachronous tumor in the remnant liver. We aimed to review HB patients to determine the risk of local recurrence in multifocal disease., Methods: We undertook retrospective cohort analysis of all HB patients at a single tertiary referral center between 2001 and 2015. Demographics, diagnostic features, operative details, and outcomes were analyzed., Results: Sixty patients underwent surgical management of HB. 39 had unifocal, and 21 had multifocal disease. Of multifocal patients, 9 underwent liver transplantation, 10 anatomic resections, and 2 nonanatomic resections. Overall, two patients had recurrence in the remnant liver - both from the unifocal group. There were equivalent distant (lung) recurrences between the groups (8% for unifocal versus 14% for multifocal), p = 0.89. At a mean of 75 months of follow-up, overall survival was 97% for unifocal patients and 86% for multifocal patients, p = 0.12., Conclusion: Multifocal HB was not associated with increased local recurrence in the setting of R0 resection and chemotherapy. These data do not support the contention that all patients with multifocal HB require a total hepatectomy and transplantation to reduce the incidence of local recurrence and/or metachronous tumor development., Level of Evidence: Level III - Limited cohort analysis., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objective: Magnetic resonance-guided high-intensity focused ultrasound (MRgHIFU) is a potential noninvasive therapy for fetal conditions. In utero MRgHIFU delivery and proton resonance frequency shift (PRFS) thermometry monitoring will control accuracy of HIFU ablation and confirm in situ tissue heating in a rabbit model., Methods: High-resolution 3T MR images were acquired in late-gestation rabbits (approximately 30 days, n = 5). HIFU sonications, using magnetic resonance (MR) thermometry as a guide, were delivered to achieve necrosis in relevant fetal targets. Thermometry, posttreatment magnetic resonance imaging (MRI), and follow-up histology confirmed ablation., Results: Placentas (n = 14) were treated with 127 ± 34 Wac; thermometry-indicated temperatures reached 67°C. Lungs (n = 8) were treated with 85 ± 15 Wac and reached 73°C, livers (n = 6) with 80 ± 15 Wac and reached 74°C, and kidneys (n = 5) with 100 Wac and reached 66°C. Histological changes showed focal areas of necrosis with circumferential hemorrhage and/or vasodilation, which transitioned abruptly to healthy tissue., Conclusion: MRgHIFU therapy can effectively target and thermally treat specific in utero organs in this acute fetal rabbit model. PRFS gives in situ temperature control of therapy on tissues. Conceivably, MRgHIFU therapy may be applicable to specific fetal organ anomalies clinically and has the potential to improve the overall fetal outcome over traditional invasive surgical procedures., (© 2019 John Wiley & Sons, Ltd.)

Background: Traditional stratification of expertise in laparoscopic simulation assigns participants to novice, intermediate, or expert groups based on case numbers. We hypothesized that expert video assessment might refine this discrimination of psychomotor expertise, especially in light of new measurable parameters., Materials and Methods: One hundred five participants performed a defined intracorporeal suturing task in the pediatric laparoscopic surgery simulator armed with force-sensing capabilities. Participants were stratified into novice, intermediate, and expert groups via three classification schemes: (1) number of complex laparoscopic cases, (2) self-declared level of expertise, and (3) average expert rating of participants' videos. Precision, time to task completion, and force analysis parameters (FAP = total, maximum and mean forces in three axes) were compared using one-way analysis of variance tests. P < .05 was considered significant., Results: Participants stratified on the basis of case numbers and on the basis of self-declared level of expertise had statistically significant differences in time to task completion, but no significant difference in FAP. When participants were restratified according to expert assessment of their video performance, time to task completion as well as total and mean forces in X, Y, and Z axes allowed discrimination between novices, intermediates, and experts, thus establishing construct validity for the latter. Precision did not allow discrimination in any stratification scheme., Conclusion: Compared with traditional stratification, video assessment allows refined discrimination of psychomotor expertise within a simulator. Assessment of FAP may become a relevant tool for teaching and assessing laparoscopic skills.

Treatment of Hodgkin lymphoma (HL) has advanced over time, rendering a fatal disease now largely curable. Multiagent chemotherapy regimens, hematopoietic stem cell transplantation, and radiotherapy are the mainstays of care. Surgical intervention is rarely indicated other than for biopsy at diagnosis. However, for patients with recurrent relapsed HL isolated to one anatomical location, refractory to all other therapy, there may be a beneficial role for surgical excision. Herein, we report the surgical management of three relapsed patients with stage IVB HL who were refractory to multiple other therapeutic approaches, who all achieved good event-free survival after operative management., (© 2018 Wiley Periodicals, Inc.)

Introduction: Ventriculoperitoneal shunts (VPSs) are the mainstay of treatment of hydrocephalus but frequently need revision. We sought to directly compare the impact of laparoscopic versus open peritoneal shunt revision on the need for subsequent VPS revisions in pediatric patients., Materials and Methods: A prospectively maintained, externally validated database of pediatric patients who underwent a first peritoneal VPS revision at a single center between 2008 and 2016 was reviewed. Outcomes, including subsequent revisions, shunt infections, operative time, and hospital stay between open and laparoscopic groups, were compared., Results: A total of 148 patients underwent a first peritoneal VPS revision during the time period-40 laparoscopically and 108 open-with no significant difference in age or gender between the groups. Operative time, length of stay after shunt revision, and shunt infection rates did not vary between laparoscopic versus open revisions. There was no significant difference between need for subsequent overall (peritoneal or ventricular) shunt revisions in the laparoscopic (20%) versus the open group (34%), P = .07. However, there were significantly fewer frequent peritoneal revisions in the laparoscopic group (3% versus 15%, P = .04)., Conclusions: This first cohort analysis of laparoscopic versus open VPS revision in pediatric patients suggests that laparoscopic peritoneal VPS revision may reduce the rate of subsequent peritoneal revisions without increasing shunt infections or operative time in pediatric patients.

Background: Simulation is becoming more important in the teaching and assessment of technical skills. The purpose of this study was to refine the use of motion analysis parameters (MAPs) to assess performance of a defined task in low-cost pediatric laparoscopic simulators of differing size., Methods: 105 participants performed a defined intracorporeal suturing task in large and small pediatric laparoscopic simulators. Outcomes included MAPs - path length, extreme velocity events, and extreme acceleration events in all available degrees of freedom for novices, intermediates, and experts. ANOVA p <0.05 was judged significant., Results: In the smaller simulator, all MAPs discriminated between expertise groups in all degrees of freedom. In the larger simulator, all but one MAP discriminated between expertise groups. Experts demonstrated the greatest variability in performance between the larger and smaller simulators., Conclusion: Analysis of motion in the performance of a defined intracorporeal suturing task allowed discrimination between novices, intermediates, and experts in large and small low-cost pediatric laparoscopic simulators. Further refinement in MAPs will determine their role in surgical education., Level of Evidence: Not applicable., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: In pediatric minimal access surgery, the operative domain may vary from that of an adult to that of a neonate. This study aimed to quantify the impact of decreased operative domain on forces generated in the performance of a defined intracorporeal suturing task. Methods: One hundred five participants performed a defined intracorporeal suturing task in small and large simulators. Time to task completion and force analysis parameters (FAPs = total, maximum, and mean forces in X , Y, and Z axes) were measured. Expertise level was assigned based on the number of laparoscopic cases. Outcomes were analyzed using paired sample t -tests, P value of <.05. Results: Time to task completion varied significantly for experts between adult and pediatric simulators but not for intermediates or novices. Total, maximum, and mean forces in the X ("side to side") axis were significantly greater in the larger laparoscopic simulator for all levels of expertise. In the Y axis ("in and out" movement) and Z axis ("up and down" movement), total and mean forces were higher in the adult simulator regardless of the level of expertise. Differences in maximum force between the adult and pediatric simulators in the Z axis ("up and down" movement) varied significantly for novices and intermediates but not for experts. Conclusion: Forces were greater, particularly in the side-to-side plane, in the larger simulator for participants of all levels in the performance of this defined intracorporeal suturing task. Further analysis will determine the reasons for and implications of the increased force parameters in the simulator of larger domain.

Background: Young children are at risk for poorly managed pain after surgery, with significant negative consequence to their quality of life and health outcomes. Mobile applications offer a highly accessible, engaging, and interactive medium to improve pain assessment and management; however, they generally lack scientific foundation or support., Aims: The aims of this study were to describe a successful parent-science partnership in the development and testing of Achy Penguin, a parent-developed iOS app to help assess and manage acute pain in young children, and to evaluate and refine the usability of Achy Penguin in young children with acute postoperative pain., Methods: Three cycles of iterative usability testing were conducted with 20 4- to 7-year-old children ( M  = 5.8 years) in hospital who had recently undergone surgery ( n  = 6-7 children/cycle). Semistructured qualitative interviews were analyzed using simple content analysis., Results: Feedback from children and further integration of evidence-based pediatric pain knowledge led to refinements in app pain assessment and management content, as well as app flow and functionality. Changes improved children's ease of use and understanding and satisfaction by simplifying language in app instructions and content, adding audio and pictorial instructions, and increasing the engagement, interactiveness, immersiveness, and general appeal of pain management strategies., Conclusions: This article showcases the value of collaborative partnerships between various stakeholders (parents, app developers, and researcher/health care providers) to address gaps in pediatric pain care. The Achy Penguin app shows promise for improving pain assessment and management in young children, although further evaluation of app effectiveness and implementation is warranted., Competing Interests: Lesley Baker in the founder of For Jack and Jill, LLC, and developer of the Achy Penguin© app based in Seattle, Washington. She received no funding and no compensation for refinements to the app throughout this study. The Achy Penguin app is publicly available for download and use, inclusive of all features, at no cost through the Apple iTunes store (https://itunes.apple.com/us/app/achy-penguin/id919797264?mt=8). All other authors have no conflicts of interest to declare., (© 2018 The Author(s). Published with license by Taylor & Francis Group, LLC.)

Objective: Laparoscopic simulators help improve surgical skills in an ex vivo setting. New simulators incorporate force and motion assessment, but often at high financial cost. Our goal is to establish global access to a laparoscopic simulator, which offers both traditional summative assessment (time to task completion and precision) as well as advanced formative assessment (force and motion sensing capabilities) so that educators anywhere may be able to create simulators with increased educational value., Design: A low-cost laparoscopic simulator incorporating an off-the-shelf optical sensor, inertial measurement unit, holders, and a housing unit for a microcontroller was integrated into a plastic box with a high-definition digital camera and a three-dimensional mouse. Open source software was developed to offer real-time feedback in force and motion. The system was calibrated for accuracy and consistency., Results: The simulator was assembled from off-the-shelf components and open-source software. Total estimated cost was $350 United States Dollars. The mouse was calibrated by applying known forces in known directions. Linear forces measured in all axes showed linear output trends with r 2 -values of between 0.988 and 0.999. Accuracy in motion evaluation was evaluated and this demonstrated low average errors in the motion sensors of 5.4% to 6.8%., Conclusions: This low-cost, off-the-shelf, open-access laparoscopic simulator provides accurate and consistent measures of force and motion. We believe that collaborative efforts between surgeons and engineers can allow the creation of these surgical teaching devices at a reasonable cost such that they can be used in resource-rich and resource-limited settings.

Rhabdomyosarcoma (RMS) represents the most common soft tissue sarcoma in the pediatric age group. While RMS has been traditionally classified on the basis of its histological appearance (with embryonal and alveolar being most common), it is now clear that the PAX-FOXO1 fusion product drives prognosis. We report here a case of pelvic embryonal RMS in a 3-month-old male who was subsequently found to have developed brain metastases during the course of chemotherapy. Cytogenetic analysis of the brain metastases at the time of autopsy as well as next-generation sequencing analysis revealed a reciprocal translocation involving the SH3 domain containing ring finger 3 gene (SH3RF3, on chromosome 2q13) and the Lipase C gene (LIPC, on chromosome 15q21.3). Due to the poor quality of the pretreatment and postresection samples, cytogenetics and NGS analysis looking for the presence of this balanced translocation in these specimens could not be performed. To the authors' knowledge, this translocation has never been described in RMS. Further studies are needed to determine the biological and clinical implications of this novel translocation.

Posttransplant lymphoproliferative disorder (PTLD) is a severe complication of solid organ transplantation. A common site for PTLD development is the gastrointestinal (GI) tract. The purpose of this study was to evaluate the incidence, clinical features, and overall survival of pediatric patients with GI-PTLD, and to assess whether major surgical interventions increased mortality. Records of pediatric transplant patients who developed GI-PTLD between January 2000 and June 2015 were retrospectively reviewed at our institution. Of 814 patients who received solid organ transplants, 34 (4%) developed GI-PTLD. Lung and multiorgan transplants had the highest incidence of GI-PTLD (both 11%). Patients often had multisite GI involvement (47%). Within the first year of transplantation, 38% of the 34 patients developed GI-PTLD. Of the patients with Epstein-Barr Virus-positive disease, 12/22 (55%) presented in the first 12 months of transplantation, compared with only 1/12 (8%) of the patients with Epstein-Barr Virus-negative disease (P=0.002). Major surgical interventions were required in 41% of patients; overall survival rate for these surgical patients was 71%, compared with 60% for patients not requiring major surgical interventions (P=0.49). Despite multimodal treatments, overall survival remains poor for patients with GI-PTLD; however, major surgical intervention did not significantly impact overall survival in this cohort.

Background: Mastering proper force manipulation in minimally invasive surgery can take many hours of practice and training. Improper force control can lead to necrosis, infection, and scarring. A force-sensing skin (FSS) has been developed, which measures forces at the distal end of minimal access surgeries' (MAS) instruments without altering the instrument's structural integrity or the surgical workflow, and acts as a minimally disruptive add-on to any MAS instrument., Methods: A proof of concept study was conducted using a FSS-equipped 5 mm straight-tip needle holder. Participants (n = 19: 3 novices, 11 fellows, and 5 staff surgeons) performed one intracorporeal suturing knot task (ISKT). Using participant task video footage, each participant's two puncture forces (each wall of the Penrose drain) and three knot tightening forces were measured. Force metrics from the three expertise groups were compared using analysis of variance (ANOVA) and Tukey's honest significance test with statistical significance assessed at P < .05., Results: Preliminary ISKT force metric data showed differences between novices and more experienced fellows and surgeons. Of the five stages of the ISKT evaluated, the first puncture force of the Penrose drain seemed to best reflect the difference in skill among participants. The study demonstrated ISKT knot tightening and puncture force ranges across three expertise levels (novices, surgical fellows, and staff surgeons) of 0.586 to 6.089 newtons (N) and 0.852 to 2.915 N, respectively., Conclusion: The investigation of force metrics is important for the implementation of future force feedback systems as it can provide real-time information to surgeons in training and the operating theater.

Background: Laparoscopic models are increasingly recognized as important tools in surgical training. The purpose of this study was to compare pediatric and adult laparoscopic surgical skills, and gain insight into the upskilling in both groups., Materials and Methods: Adult- and pediatric-sized laparoscopic simulators were fitted with custom-built motion tracking hardware and software. Participants were recruited at the Education Booth of the 2012 combined SAGES/IPEG meeting. They each performed 1 adult and 1 pediatric intracorporeal suturing task. Velocity, acceleration, and range were studied in all degrees of freedom available during laparoscopic surgery (pitch, yaw, roll, and surge). Participants were stratified by expertise based on the traditional metrics of self-reported caseloads., Results: A total of 57 participants (15 novices, 7 intermediates, and 35 experts) were recruited. Experts had significantly higher extreme events in three of the four degrees of freedom when using the pediatric simulator than when using the adult simulator. Few significant differences were seen when comparing novice and intermediate performances on the adult versus pediatric simulator. Linear regression showed no difference between adult and pediatric experts tested on the adult or pediatric simulator., Conclusions: Experts were more challenged with the pediatric than with the adult suturing task. No difference was noted for overall averaged performance metrics comparing adult and pediatric experts suturing in adult versus pediatric simulators. As a participant's level of expertise improves, a model progressing from larger to smaller domains in the performance of defined laparoscopic tasks may, by virtue of its greater challenge, encourage psychomotor development.

Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous condition that predisposes to numerous proliferative lesions, including perivascular epithelioid cell tumors (PEComas), such as lymphangioleiomyomatosis (LAM) and angiomyolipomas, and rare neuroendocrine neoplasms. We describe herein a TSC2-harboring tuberous sclerosis patient manifesting with a synchronous well-differentiated L-cell rectal neuroendocrine tumor and leiomyomatosis-like LAM of the rectum. The background large bowel wall was thickened by confluent nodular areas comprising vessels and spindle-to-epithelioid cells, which are immunoreactive for myoid (smooth muscle actin, muscle specific actin, and desmin) and melanocytic markers (HMB45, Melan-A, microphthalmia transcription factor, and CD117). With the exception of TSC-related pancreatic neuroendocrine tumors, the association between tuberous sclerosis and neuroendocrine neoplasms remains largely unknown in the gastrointestinal tract. Neuroendocrine tumorigenesis in tuberous sclerosis is often linked to inactivating mutations of TSC2 leading to aberrant activation of mammalian target of rapamycin (mTOR) pathway. In this report, we document, for the first time, two foci of L-cell rectal neuroendocrine tumor arising in the setting of tuberous sclerosis, thus broadening the spectrum of TSC-associated endocrine disorders. Moreover, to our knowledge, this is only the second documented case of gastrointestinal leiomyomatosis-like LAM in a patient with tuberous sclerosis. The current case provides further evidence that, similar to pancreatic neuroendocrine tumors, neuroendocrine tumors of the luminal gastrointestinal tract may also be a feature of tuberous sclerosis and can be seen in association with PEComas.

Background: Orthotopic liver transplantation (OLT) is considered the standard for children with hepatoblastoma (HB) in whom complete surgical resection is not possible. However, OLT is not always available or feasible., Objective: To describe the outcome of children with HB who were initially deemed unresectable and underwent complex hepatectomy with planned close margins, and ultimately avoided OLT., Methods: Demographic data, surgical and pathologic details, and survival information were collected from children treated for HB between January 2010 to December 2015., Results: Among six children (median age 12 months (3-41 months)), PRETEXT classification was III (n = 2), III/IV (n = 1), and IV (n = 3). Patients received a median of six cycles (range 4-7) of platinum-based induction chemotherapy; five received doxorubicin. Experienced pediatric surgeons performed extended right and left hepatectomy in five and one patients, respectively, with assistance of an experienced liver transplant surgeon (n = 4). Microscopic margins were positive (n = 2) and negative but close (n = 4; 2-5 mm). Two patients required vascular reconstruction of the vena cava. At median follow-up of 3.3 years (1.7-4.6 years), there was no evidence of local recurrence. One patient had recurrence of pulmonary disease 3 months after surgery., Conclusions: Patients with advanced HB treated with complex surgical resections with positive or close negative margins had good outcomes without OLT. We suggest that planned positive or close microscopic margins in highly selected HB patients may spare the morbidity of OLT and offer an alternative for those ineligible for OLT. Our experience illustrates the importance of a multidisciplinary team specialized in the management of liver tumors., (© 2017 Wiley Periodicals, Inc.)

Background: Previous investigators have shown that novices are able to assess surgical skills as reliably as expert surgeons. The purpose of this study was to determine how novices and experts arrive at these graded scores when assessing laparoscopic skills and the potential implications this may have for surgical education., Methods: Four novices and four general laparoscopic surgeons evaluated 59 videos of a suturing task using a 5-point scale. Average novice and expert evaluator scores for each video and the average number of times that scores were changed were compared. Intraclass correlation coefficients were used to determine inter-rater and test-retest reliability. Evaluators were asked to define the number of videos they needed to watch before they could confidently grade and to describe how they were able to distinguish between different levels of expertise., Results: There were no significant differences in mean scores assigned by the two evaluator groups. Novices changed their scores more frequently compared to experts, but this did not reach statistical significance. There was excellent inter-rater reliability between the two groups (ICC = 0.91, CI 0.85-0.95) and good test-retest reliability (ICC > 0.83). On average, novices and experts reported that they needed to watch 13.8 ± 2.4 and 8.5 ± 2.5 videos, respectively, before they could confidently grade. Both groups also identified similar qualitative indicators (e.g., instrument control)., Conclusion: Evaluators with varying levels of expertise can reliably grade performance of an intracorporeal suturing task. While novices were less confident in their grading, both groups were able to assign comparable scores and identify similar elements of a suturing skill as being important in terms of assessment.

Background: This study analyzes instrument motion for segments of a defined intra-corporeal suturing task in a laparoscopic simulator. We describe a system providing real-time velocity and acceleration assessment in the performance of this task. Analysis of the deconstructed task segments allows targeted assessment and teaching., Methods: A traditional box trainer was fitted with a custom-built motion-tracking system. Participants were stratified into novice, intermediate and expert groups. They performed a defined intra-corporeal suturing task. Real-time data were collected in four degrees of freedom (DOFs) (Roll, Surge, Pitch, Yaw). The task was then deconstructed into four segments: loading needle/pull-through, double-throw knot, first single-throw knot, and second single-throw knot. Motion analysis parameters (MAPs) were studied for each DOF., Results: Sixty-four participants were tested (14 novices, 19 intermediates, 31 experts). The largest difference in MAPs was seen in the 'double-throw knot' segment. MAPs for the 'loading needle/pull-through' segment revealed differences between novices and experts in Roll and Pitch DOFs only. For the 'first single knot' segment, similar MAP trends were noted across all DOFs, with significant differences between novices versus experts and intermediates versus experts. For the 'second single knot' segment, the difference in MAPs was preserved only for novices versus experts., Conclusions: By analyzing motion for a defined suturing task in a laparoscopic simulator, we can gain insight into the specific hand motions distinguishing experts from non-experts. Such information may allow teaching in a more focused, effective and efficient manner.

Mastering proper force manipulation in minimally invasive surgery can take many years. Improper force control can lead to necrosis, infection, and scarring. This paper describes a novel system to measure, log, and display external forces at the distal end of minimally invasive surgical instruments in real-time. The system, comprising of a Force- Sensing Sleeve, Bluetooth electronics module, and an Android mobile application. A sensorized 5 mm minimally invasive surgical needle holder was evaluated for bending force accuracy, linearity, and repeatability in six directions. The results showed that the system responded linearly to forces at the tool-tip independent of direction with an RMS error of 0.088 N. Repeatability was affected by system noise potentially arising from temperature drift and thermal noise. Future work will include characterization of communication performance for force feedback in surgical training and assessment.

Background: Laparoscopic models are recognized as important training tools. Lower fidelity systems are used mainly for simpler tasks; an advanced suturing task may allow for additional training of experts. The purpose of this study was to explore the educational role of an advanced suturing task using motion analysis and establish the task's construct validity., Methods: The pediatric laparoscopic surgery (PLS) simulator was customized with motion-tracking hardware and software. Participants were stratified by expertise, then performed an advanced task involving intracorporeal suturing in a vertical plane, with the suture passing superiorly to inferiorly. Traditional PLS scores were calculated, and motion was analyzed in the four degrees of freedom available in laparoscopic surgery (Pitch, Yaw, Roll, and Surge). Data were compared to historic results for a standard suturing task., Results: Sixty participants were recruited (8 novices, 13 intermediates, and 39 experts). Analysis of motion in all degrees of freedom allowed discrimination between participants based on expertise level. Compared with the standard task, PLS scores for the advanced task were significantly lower for intermediates and experts, and the number of extreme motion events was significantly higher, indicating that advanced task is more challenging. In addition, only 76.3% of experts, 76.9% of intermediates, and 37.5% of novices were able to successfully complete the advanced task., Conclusions: Performance of an advanced intracorporeal suturing task allowed discrimination of expertise level. The task's increased complexity may help hone laparoscopic technical skills, particularly among advanced performers, and even allow discrimination of psychomotor expertise within the traditional cohort of experts.

We evaluated the construct validity (including responsiveness), reliability, and feasibility of the Pain Squad multidimensional smartphone-based pain assessment application (app) in children and adolescents with cancer, using 2 descriptive studies with repeated measures. Participants (8-18 years) undergoing cancer treatment were drawn from 4 pediatric cancer centers. In study 1, 92 participants self-reported their level of pain twice daily for 2 weeks using the Pain Squad app to assess app construct validity and reliability. In study 2, 14 participants recorded their level of pain twice a day for 1 week before and 2 weeks after cancer-related surgery to determine app responsiveness. Participants in both studies completed multiple measures to determine the construct validity and feasibility of the Pain Squad app. Correlations between average weekly pain ratings on the Pain Squad app and recalled least, average, and worst weekly pain were moderate to high (0.43-0.68). Correlations with health-related quality of life and pain coping (measured with PedsQL Inventory 4.0, PedsQL Cancer Module, and Pain Coping Questionnaire) were -0.46 to 0.29. The app showed excellent internal consistency (α = 0.96). Pain ratings changed because of surgery with large effect sizes between baseline and the first week postsurgery (>0.85) and small effect sizes between baseline and the second week postsurgery (0.13-0.32). These findings provide evidence of the construct validity, reliability, and feasibility of the Pain Squad app in children and adolescents with cancer. Use of real-time data capture approaches should be considered in future studies of childhood cancer pain. A video accompanying this abstract is available online as Supplemental Digital Content at http://links.lww.com/PAIN/A169.

Background: Laparoscopic models for ex vivo up-skilling are becoming increasingly important components of surgical education. This study aims to establish the construct validity and possible educational role of a new laparoscopic box trainer equipped with a motion-tracking device., Methods: A structured questionnaire was used to assign participants into novice, intermediate, or expert categories according to level of experience in minimal access surgery (MAS). Participants carried out a well-defined intracorporeal suturing task. Three specific motion analysis parameters (MAPs)-velocity, acceleration, and range-were measured and analyzed as movements in the four degrees of freedom available in traditional MAS using tracking sensors at the trocar insertion sites., Results: The number of extreme velocity and acceleration events in all four degrees of freedom proved capable of differentiating between participants in the three categories of surgical experience using an ANOVA test (p < 0.001). Post hoc analysis confirmed these differences in the number of extreme velocity and acceleration events between all groups tested except for the velocity of the roll between the intermediates and experts., Conclusion: These findings confirm construct validity for this new laparoscopic box trainer system, which employs a novel analysis based on motion parameters. Motion parameters provide information regarding the overall smoothness of the operator's instrument handling, an important aspect of a surgeon's technique. This preliminary data will be used to design a simulator with real-time motion feedback to enhance its educational value.

Introduction: Nonsurgical closure after primary silo placement, secondary plastic closure (SPC), has been used as an alternative to secondary surgical closure (SSC) in gastroschisis. The benefits described were closure without formal surgical procedure, cosmetic aspect, and minimization of intra-abdominal pressures. This study compared requirements for mechanical ventilation and general anesthesia, nutritional care, and outcomes between SPC and SSC., Patients and Methods: We included patients with primary staged-silo reduction with a 1-year minimum follow-up. SPC was performed at bedside with sedation using a nonadherent dressing. SSC was performed in operating room under general anesthesia using standard surgical techniques., Results: This retrospective study included 64 patients, 23 SPC and 41 SSC. The characteristics of the two groups were comparable. Mechanical ventilation was used for 15 SPC and 41 SSC (p=0.0001) with a comparable median duration (5.5 and 6.0 days, not significant [NS]). General anesthesia was required for 9 SPC and 41 SSC (p<0.0001). Complications included one SPC and six SSC with necrotizing enterocolitis, zero SPC and four SSC with intestinal atresia, two SPC and four SSC with small bowel obstruction, zero SPC and one SSC with abdominal compartment syndrome resulting in a short bowel syndrome (NS). Median duration of parenteral nutrition (30 and 27 days), time to first feeds (14 and 14 days), time at or above minimal enteral feeding (22 and 17 days), time to full feeds (31 and 28 days), length of stay (LOS) in neonatal intensive care unit (24 and 23.5 days) and overall hospital LOS (37 and 36 days) were not statistically different between SPC and SSC patients without complications, respectively. These data were comparable for SPC and SSC patients with complications. Five SPC and six SSC developed an umbilical hernia (NS); two patients in each group required a surgical repair (NS)., Conclusion: Plastic closure of gastroschisis after primary silo reduction is simple, safe, reproducible, and associated with a significant lower incidence of mechanical ventilation. Nutritional management and length of hospital stay were similar to conventional surgical closure for patients. Plastic closure allows nonoperative management without general anesthesia at patient's bedside, in comparison with surgical closure that must be performed under general anesthesia in the operating room. Plastic closure does not appear to be associated with more umbilical hernias in this retrospective study., (Georg Thieme Verlag KG Stuttgart · New York.)

Background/aim: Established indications for surgery in necrotizing enterocolitis (NEC) are pneumoperitoneum and failure to improve or clinical deterioration with medical treatment alone. It has been proposed that infants with intestinal necrosis may benefit from surgery in the absence of one of these indications yet the diagnosis of definitive intestinal necrosis is challenging. Recent data suggest that abdominal ultrasound (US) examination focused on the gastrointestinal tract and the peritoneal cavity may be of utility in this regard. The aim of this study was to evaluate the ability of abdominal US to detect intestinal necrosis in infants with radiographically confirmed NEC., Methods: Twenty-six consecutive infants with Bell stage II or III NEC were prospectively included in the study between September 2013 and July 2014. Infants with a pre-existing indication for surgery were excluded. At least one abdominal US examination was performed in each patient using a standardized previously described method. Surgery was performed at the discretion of the attending surgeon based on clinical and imaging findings. Clinical, radiographic, US, and intra-operative data were recorded to allow comparison between US findings, surgical findings and outcome., Results: US demonstrated signs of intestinal necrosis in 5 of the 26 patients. All of these five had laparotomy. Intestinal necrosis requiring resection was confirmed in four and the other was found to have NEC but no necrosis was identified. In 21 patients US did not suggest intestinal necrosis. Of these, only one had surgery in whom NEC but no necrosis was identified. The remaining 20 responded to medical treatment for NEC and were assumed not to have had intestinal necrosis based on improvement without surgical intervention. The sensitivity, specificity, positive predictive value and negative predictive values of US for the detection of bowel necrosis were calculated as 100, 95.4, 80.0, and 100%, respectively., Conclusion: Our prospective findings suggest that abdominal US can identify those infants with NEC who may need surgery by detecting bowel necrosis (prior to the development of perforation or medical deterioration) with high sensitivity and specificity. Early surgical intervention in the clinical pathway of NEC may lead to improved outcomes.

Objectives: To compare the outcomes of laparoscopic nephrectomy (LN) with open radical nephrectomy (ORN) in the management of consecutive pediatric neoplasms., Patients and Methods: Retrospective cohort study of consecutive children treated for primary renal tumors between 2006 and 2011, segregated based on surgical modality (LN/ORN). Pre-, intra- and postoperative data and outcomes were collected., Results: Demographics from the 45 patients (13 LN, 32 ORN) were similar, and tumors in the LN group were smaller [6.59 ± 1.8 cm vs. 10.99 ± 2.99 cm ORN (p < 0.05)]. Six patients had preoperative chemotherapy (two LN, four ORN). No tumor ruptures occurred with either technique. Wilms tumor (seven LN, 24 ORN) was the most common diagnosis, followed by renal cell carcinoma (four LN, four ORN). Procedure length was similar between groups (282 ± 79 LN, 263 ± 81 min ORN). Mean length of stay was significantly shorter for LN (2.9 vs. 5.9 days; p = 0.002). Postoperative narcotic requirements and use of nasogastric tube were higher in the ORN group. After a median follow-up of 18 (LN) and 33 months (ORN), 1 and 4 recurrences occurred, respectively., Conclusions: LN is an attractive alternative to open surgery in carefully selected cases of pediatric renal tumors. Procedure length and incidence of intra-operative rupture were not increased, while post-operative recovery and hospital stay were shorter for LN. Longer follow-up is mandatory to confirm comparable oncological outcomes to ORN., (Copyright © 2013 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Study Objective: To characterize preoperative risk stratification with aim of identifying the accurate surgical approach of benign and malignant adnexal masses in pediatric patients., Design: A retrospective chart review of all cases of adnexal masses surgically managed between January 2001 and December 2006., Setting: The Hospital for Sick Children, Toronto, Canada., Participants: 129 cases of 126 pediatric and adolescent patients who underwent operative management of their adnexal masses., Main Outcome Measures: Ultrasonographic characteristics (cyst size and character), surgical approach (laparoscopy vs laparotomy) and method of cyst removal (cystectomy vs oophorectomy). Data was assessed with a Fisher Exact test where appropriate (P < .05)., Results: Malignancies were more frequently treated by laparotomy (n = 14, 98.6%, P < .001), and benign cases by laparoscopy (n = 78, 97%, P < .001). On ultrasonography, malignant masses were more often complex (n = 16, 100%, P = .006) and ≥8 cm (n = 16, 100%, P < .001) than benign masses (≥8 cm n = 60, 53%, complex n = 76, 67%). Combining ultrasonographic measurements of ≥8 cm and complexity identified 100% of malignant masses (n = 16) and 36% of benign masses (n = 41, P < .001, PPV = 37.1, NPV = 100%). Additional imaging including CT/MRI was ordered by pediatric surgeons (n = 17, 77%) more often than pediatric gynecologists (n = 44, 41%, P = .002). Furthermore, pediatric surgeons managed adnexal masses by oophorectomy (n = 12, 55%) more often as compared to pediatric gynecologists (n = 19, 18%, P < .001)., Conclusion: Using preoperative characteristics of complexity and ≥8 cm reduces the number of benign masses treated with laparotomy while ensuring malignant masses are managed with an open approach., (Copyright © 2014 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

Background: Construct validity for the pediatric laparoscopic surgery (PLS) simulator has been established through a scoring system based on time and precision. We describe the development and initial validation of motion analysis to teach and assess skills related to pediatric minimal access surgery (MAS)., Methods: Participants were asked to perform a standardized intracorporeal suturing task. They were classified as novices, intermediates, and experts. Motion in the four degrees of freedom available during traditional MAS (PITCH, YAW, ROLL and SURGE) was assessed using range, velocity, and acceleration., Results: Analysis of motion allowed discrimination between the 75 participants according to level of expertise. The most discriminating motion parameter was the acceleration in performing the ROLL (pronation/supination) with values of 30±27 for novices, 15±5 for intermediates, and 3.7±3 for experts (p<0.001)., Conclusions: Tracking and analyzing the motion of instruments within the PLS simulator allow discrimination between novices, intermediates, and experts, thus establishing construct validity. Further development may establish motion analysis as a useful "real time" modality to teach and assess MAS skills., (Copyright © 2014 Elsevier Inc. All rights reserved.)