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Children with severe constipation and a neurodiverse disorder (Autism and/or developmental delay) represent a challenging bowel management group. Treatment outcomes with laxative or enema therapy remain limited and are often complicated by patient/caregiver compliance. We hypothesized that children with neurodiverse disorders and severe constipation would benefit from a bowel management program (BMP) that includes early use of antegrade enemas.Children requiring BMP in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry with diagnosis of constipation and/or constipation with pseudo-incontinence were reviewed. Those with Hirschsprung disease and/or Anorectal Malformation were excluded. BMP needs in patients with a neurodiverse diagnosis were compared to those without to evaluate differences in BMP's.372 patients requiring a BMP were identified. 95 patients (58 autism spectrum, 54 developmental delay) were neurodiverse, and 277 patients were not. Neurodiverse patients had a higher prevalence of enema therapy 62.1% (59/95) vs. 54.9% (152/273) and use of antegrade enema therapy 33.7% (32/95) vs. 21.2% (58/273), p = 0.126. Neurodiverse patients were older 37.9% (36/95)12 years vs. 23.1% (63/273), p = 0.001 and 47.6% (10/21) were changed from laxative to enema therapy over time. 80% (8/10) of those changed from laxatives to enemas used antegrade therapy. 67.3% (35/52) of neurodiverse patients followed over time were on enema therapy at the most recent visit with 80% (28/35) requiring antegrade therapy.A large portion of patients with a neurodiverse disorder who fail laxative therapy use antegrade enemas to achieve effective bowel management. Early consideration of an antegrade conduit may simplify treatment in this group of children.III.

Background Pediatric inflammatory bowel disease (IBD) carries significant morbidity and requires extensive medical and often surgical intervention. The aim of this study was to evaluate the impact of a dedicated Multidisciplinary clinic on the outcomes of children with IBD. Methods A retrospective review of a prospective database, established to track quality and outcomes of children undergoing an abdominal operation for IBD, was performed. Children who were managed before (09/2017–03/2019) and after (04/2019–06/2020) establishment of the multidisciplinary clinic were examined. The clinic instituted several care process protocols including early recovery (ERAS) and garnered additional resources for patients (wound ostomy, nutrition, social work, etc.) Primary outcomes were unanticipated return to the operating room, length of stay, ER visits within 30 days of surgery and hospital readmissions within 30 days of surgery. Results We identified 41 children who underwent a total of 80 major abdominal operations; 46.3% of procedures occurred before and 53.7% occurred after instituting our clinic. There were no notable changes in disease distribution (e.g., ulcerative colitis vs. Crohn's), disease severity, medication exposure, or case urgency (elective vs. emergent). ER visits within 30 days of surgery decreased (4 (9.3%) vs. 10 (27%), p = 0.04) as did readmissions within 30 days of surgery (1 (2.3%) vs. 9 (24.3%), p = 0.005). Conclusions Implementation of a dedicated multidisciplinary clinic for IBD and its attendant focus on protocols and appropriate use of adjunctive resources was associated with decreased emergency department visits and hospital readmissions in the post-operative setting. Level of evidence : III

Background An appendicostomy (ACE) is a surgical option for antegrade enemas in children with severe constipation and/or fecal incontinence who have failed medical management. In 2019, we initiated an expedited post-operative protocol and sought to examine our short-term outcomes compared with our historical cohort. Methods A retrospective review was performed of all children undergoing ACE between 2017 and 2020. Children were excluded if they underwent an associated procedure (e.g. colon resection). Patients were divided into two cohorts: historical cohort (2017–2018, Group A) and the expedited protocol (2019 to present, Group B). The primary outcome was length of stay. Results 30 patients met inclusion (Group A = 16, Group B = 14). The most common indications for ACE were constipation (50%) and constipation or fecal incontinence associated with anorectal malformation (43%). Group B experienced a decreased length of stay (1 vs 3 days, P = 0.001) without differences in 30-day surgical site infection (7.1% vs 18.8%, p = 0.61) or unplanned visit (15.4% vs 18.8%, p = 1.0). Group B had a higher prevalence of MiniACE® button placed through the appendix vs. Malone (42.8% vs 12.5%, p = 0.10). Conclusions Our expedited post-op protocol decreased length of stay without other significant adverse clinical sequelae. Level of evidence Retrospective Comparative Study, Level III.

Children with ulcerative colitis (UC) may undergo a staged approach for restorative proctocolectomy and ileal pouch anal anastomosis (IPAA). Previous studies in adults suggest a decreased morbidity with delayed pouch creation, but pediatric studies are limited. We compared outcomes for delayed versus early pouch construction in children.Patients with UC undergoing IPAA were selected from the National Surgical Quality Improvement Program Pediatric database from 2012 to 2018. Patients were categorized as early (2-stage) or delayed (3-stage) pouch construction based on Current Procedural Terminology codes. Our primary outcome was any adverse event. We used a multivariable logistic regression model to assess the relationship between timing of pouch creation and adverse events.We identified 371 children who underwent IPAA: 157 (42.3%) had early pouch creation and 214 (57.6%) had a delayed pouch. Those with an early pouch creation were more likely to have exposure to immunosuppressants (11% vs. 5%, p = 0.017) and steroids (30% vs. 10%, p 0.001) at the time of surgery. After controlling for patient characteristics, there were no significant differences in adverse events between the two groups.Children undergoing early pouch creation have increased exposure to steroids and immune suppressants; nevertheless, no differences in adverse events were identified.II.

Objective To determine the impact of tumor characteristics and treatment approach on (1) local recurrence, (2) scoliosis development and (3) patient-reported quality of life in children with sarcoma of the chest wall. Summary background data Children with chest wall sarcoma require multimodal therapy including chemotherapy, surgery and/or radiation. Despite aggressive therapy which places them at risk for functional impairment and scoliosis, these patients are also at significant risk for local recurrence. Methods A multi-institutional review of 175 children (median age 13 years) with chest wall sarcoma treated at seventeen Pediatric Surgical Oncology Research Collaborative institutions between 2008-2017 was performed. Patient-reported quality of life was assessed prospectively using PROMIS surveys. Results The most common diagnoses were Ewing sarcoma (67%) and osteosarcoma (9%). Surgical resection was performed in 85% and radiation in 55%. A median of 2 ribs were resected (IQR = 1-3), and number of ribs resected did not correlate with margin status (p = 0.36). Local recurrence occurred in 23% and margin status was the only predictive factor (HR 2.24, p = 0.039). With a median follow-up of 5 years, 13% developed scoliosis (median Cobb angle 26) and 5% required corrective spine surgery. Scoliosis was associated with posterior rib resection (HR 8.43; p = 0.003) and increased number of ribs resected (HR 1.78; p = 0.02). Overall, patient-reported quality of life is not impaired following chest wall tumor resection. Conclusions Local recurrence occurs in one-quarter of children with chest wall sarcoma and is independent of tumor type. Scoliosis occurs in 13% of patients, but patient-reported quality of life is excellent.

Introduction Enhanced recovery protocols include multimodal perioperative pain control and frequently include use of NSAIDs. There is conflicting evidence that ketorolac use in inflammatory bowel disease (IBD) may precipitate disease flares and postoperative complications. The outcomes of children who receive ketorolac in this setting are not well known. We sought to evaluate ketorolac utilization in children following colectomy for ulcerative colitis. Methods All patients undergoing colectomy for ulcerative colitis between 2007 and 2017 at a tertiary children's hospital were reviewed. We collected patient age, duration of symptoms, operative details, medication utilization, length of stay, and postoperative complications. We performed a cohort comparison of these variables across patients who did vs. did not receive postoperative ketorolac. Results Sixty children were identified with median age at diagnosis of 12.6 years (IQR: 9.9–14.5). At colectomy, patients had a median PUCAI score of 60 (45–70), ESR 34 mm/h (15–50), hemoglobin 10.9 g/dL (9.3–12.9), and albumin 3.1 g/dL (2.4–3.8). Postoperatively, 45% (n = 27) received ketorolac. Patients in both cohorts had a similar length of stay, duration of opioid exposure, total morphine equivalents utilized, readmission rate, and unexpected return to the operating room. There were no documented cases of postoperative bleeding, acute kidney injury, or disease related flares among children receiving ketorolac. Conclusions Administration of ketorolac after colectomy in IBD was not associated with an increase in any postoperative complications, though the study was underpowered to detect these differences. However, ketorolac administration did not lead to a decreased utilization of opioid analgesia. Further prospective research is necessary to understand whether ketorolac in this population is safe and offers benefit. Type of study Retrospective study. Level of evidence III

Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm of intermediate malignancy. We describe the largest cohort of IMT patients to date, aiming to further characterize this rare, poorly understood tumor. This is a multi-institutional review of IMT patients ≤39 years, from 2000 to 2018, at 18 hospitals in the Pediatric Surgical Oncology Research Collaborative. One hundred and eighty-two patients were identified with median age of 11 years. Thirty-three percent of tumors were thoracic in origin. Presenting signs/symptoms included pain (29%), respiratory symptoms (25%) and constitutional symptoms (20%). Median tumor size was 3.9 cm. Anaplastic lymphoma kinase (ALK) overexpression was identified in 53% of patients. Seven percent of patients had distant disease at diagnosis. Ninety-one percent of patients underwent resection: 14% received neoadjuvant treatment and 22% adjuvant treatment. Twelve percent of patients received an ALK inhibitor. Sixty-six percent of surgical patients had complete resection, with 20% positive microscopic margins and 14% gross residual disease. Approximately 40% had en bloc resection of involved organs. Median follow-up time was 36 months. Overall 5-year survival was 95% and 5-year event-free survival was 80%. Predictors of recurrence included respiratory symptoms, tumor size and distant disease. Gross or microscopic margins were not associated with recurrence, suggesting that aggressive attempts at resection may not be warranted.

Wilms tumor (WT) is the most common renal malignancy in children. Children with favorable histology WT achieve survival rates of over 90%. Twelve percent of patients present with metastatic disease, most commonly to the lungs. The presence of a pleural effusion at the time of diagnosis of WT may be noted on staging imaging; however, minimal data exist regarding the significance and prognostic importance of this finding. The objectives of our study are to identify the incidence of pleural effusions in patients with WT, and to determine the potential impact on oncologic outcomes. A multi-institutional retrospective review was performed from January 2009 to December 2019, including children with WT and a pleural effusion on diagnostic imaging treated at Pediatric Surgical Oncology Research Collaborative (PSORC) participating institutions. Of 1259 children with a new WT diagnosis, 94 (7.5%) had a pleural effusion. Patients with a pleural effusion were older than those without (median 4.3 vs 3.5 years; P = .004), and advanced stages were more common (local stage III 85.9% vs 51.9%; P .0001). Only 14 patients underwent a thoracentesis for fluid evaluation; 3 had cytopathologic evidence of malignant cells. Event-free and overall survival of all children with WT and pleural effusions was 86.2% and 91.5%, respectively. The rate and significance of malignant cells present in pleural fluid is unknown due to low incidence of cytopathologic analysis in our cohort; therefore, the presence of an effusion does not appear to necessitate a change in therapy. Excellent survival can be expected with current stage-specific treatment regimens.

Hepatocellular carcinoma (HCC) is a rare cancer in children, with various histologic subtypes and a paucity of data to guide clinical management and predict prognosis.A multi-institutional review of children with hepatocellular neoplasms was performed, including demographic, staging, treatment, and outcomes data. Patients were categorized as having conventional HCC (cHCC) with or without underlying liver disease, fibrolamellar carcinoma (FLC), and hepatoblastoma with HCC features (HB-HCC). Univariate and multivariate analyses identified predictors of mortality and relapse.In total, 262 children were identified; and an institutional histologic review revealed 110 cHCCs (42%; 69 normal background liver, 34 inflammatory/cirrhotic, 7 unknown), 119 FLCs (45%), and 33 HB-HCCs (12%). The authors observed notable differences in presentation and behavior among tumor subtypes, including increased lymph node involvement in FLC and higher stage in cHCC. Factors associated with mortality included cHCC (hazard ratio [HR], 1.63; P = .038), elevated α-fetoprotein (HR, 3.1; P = .014), multifocality (HR, 2.4; P.001), and PRETEXT (pretreatment extent of disease) stage IV (HR, 5.76; P.001). Multivariate analysis identified increased mortality in cHCC versus FLC (HR, 2.2; P = .004) and in unresectable tumors (HR, 3.4; P.001). Disease-free status at any point predicted survival.This multi-institutional, detailed data set allowed a comprehensive analysis of outcomes for children with these rare hepatocellular neoplasms. The current data demonstrated that pediatric HCC subtypes are not equivalent entities because FLC and cHCC have distinct anatomic patterns and outcomes in concert with their known molecular differences. This data set will be further used to elucidate the impact of histology on specific treatment responses, with the goal of designing risk-stratified algorithms for children with HCC.This is the largest reported granular data set on children with hepatocellular carcinoma. The study evaluates different subtypes of hepatocellular carcinoma and identifies key differences between subtypes. This information is pivotal in improving understanding of these rare cancers and may be used to improve clinical management and subsequent outcome in children with these rare malignancies.

Pulmonary nodules that are deep within lung parenchyma and/or small in size can be challenging to localize for biopsy. This study describes current trends in performance of image-guided localization techniques for pulmonary nodules in pediatric patients.A retrospective review was performed on patients 21 years of age undergoing localization of pulmonary nodules at 15 institutions. Localization and resection success, time in interventional radiology (IR), operating room (OR) and total anesthesia time, complications, and technical problems were compared between techniques.225 patients were included with an average of 1.3 lesions (range 1-5). Median nodule size and depth were 4 mm (range 0-30) and 5.4 mm (0-61), respectively. The most common localization techniques were: wire + methylene blue dye (MBD) (28%), MBD only (25%), wire only (14%), technetium-99 only (11%), coil + MBD (7%) and coil only (5%). Localization technique was associated with institution (p 0.01); technique and institution were significantly associated with mean IR, OR, and anesthesia time (all p 0.05). Comparing techniques, there was no difference in successful IR localization (range 92-100%, p = 0.75), successful resection (94-100%, p = 0.98), IR technical problems (p = 0.22), or operative complications (p = 0.16).Many IR localization techniques for small pulmonary nodules in children can be successful, but there is wide variability in application by institution and in procedure time.Retrospective review, Level 3.

Structured abstract Purpose Experience with autologous blood patch (ABP) pleurodesis for persistent air leak in the pediatric population is limited. The purpose of this series was to describe the experience with ABP at a single tertiary children's hospital. Methods A retrospective study was performed of all thoracic procedures done by the pediatric surgery service over three years. Results Ten patients underwent a total of 17 ABPs. The median age of patients was 12 years (IQR 6–16). The most common underlying reasons for a thoracic procedure included: blebectomy for spontaneous pneumothorax (2), need for lung biopsy (2), resection of known malignant tumor (2), and empyema (2). The median number of days of persistent air leak before first ABP was 7.5 days (IQR 7–10). A second ABP was performed in 6 cases with a third procedure performed in one case. None of the patients developed respiratory compromise during ABP and no infectious complications were identified following ABP. Conclusions Our cohort demonstrates that ABP for persistent air leak following thoracic surgery is effective with minimal morbidity in children. We believe ABP can be used early and in patients with a broad range of underlying lung pathology.

OBJECTIVE To describe utilization and long-term outcomes of pneumonectomy in children and adolescents with cancer. SUMMARY BACKGROUND DATA Pneumonectomy in adults is associated with significant morbidity and mortality. Little is known about the indications and outcomes of pneumonectomy for pediatric tumors. METHODS The Pediatric Surgical Oncology Research Collaborative (PSORC) identified pediatric patients

Purpose: Use of extrapleural pneumonectomy for malignancy in children is rarely performed with few descriptions in the literature. In this article, we describe a 19-month-old girl who underwent an extrapleural pneumonectomy with enbloc resection of the diaphragm and pericardium for advanced pleuropulmonary blastoma. We achieved an R0 resection and survival without local disease recurrence at 23 months. Conclusion: Extrapleural pneumonectomy is feasible in some circumstances to achieve oncologic control in cases of advanced PPB. This should be kept as part of the surgeons' armamentarium. Keywords: Pleuropulmonary blastoma, Tension physiology, Pneumonectomy, Extrapleural pneumonectomy

The recognition of pulmonary manifestations of inflammatory bowel disease (IBD) is important as these diseases can be confused with infectious etiologies (e.g., tuberculosis or fungal infection) and, as a result, may unnecessarily delay institution of appropriate therapy (e.g., infliximab). Furthermore, they are a source of morbidity that may be overlooked and, like other IBD-related pathologies, are often responsive to treatment with corticosteroids, immunomodulators, or biologic therapies. The purpose of this paper is to describe the cases of six children at a single institution with differing presentations, treatments, and responses to treatment of their IBD-related lung disease to improve recognition of this uncommon process.

This chapter provides a brief introduction to the nuances and challenges associated with the surgical evaluation of the pediatric patient.

Background To better characterize short-term and long-term outcomes in children with pancreatic tumors treated with pancreaticoduodenectomy (PD). Methods Patients 21 years of age or younger who underwent PD at Pediatric Surgical Oncology Collaborative (PSORC) hospitals between 1990 and 2017 were identified. Demographic, clinical information, and outcomes (operative complications, long-term pancreatic function, recurrence, and survival) were collected. Results Sixty-five patients from 18 institutions with a median age of 13 years (4 months-22 years) and a median (IQR) follow-up of 2.8 (4.3) years were analyzed. Solid pseudopapillary tumor of the pancreas (SPN) was the most common histology. Postoperative complications included pancreatic leak in 14% (n = 9), delayed gastric emptying in 9% (n = 6), marginal ulcer in one patient, and perioperative (30-day) death due to hepatic failure in one patient. Pancreatic insufficiency was observed in 32% (n = 21) of patients, with 23%, 3%, and 6% with exocrine, or endocrine insufficiencies, or both, respectively. Children with SPN and benign neoplasms all survived. Overall, there were 14 (22%) recurrences and 11 deaths (17%). Univariate analysis revealed non-SPN malignant tumor diagnosis, preoperative vascular involvement, intraoperative transfusion requirement, pathologic vascular invasion, positive margins, and need for neoadjuvant chemotherapy as risk factors for recurrence and poor survival. Multivariate analysis only revealed pathologic vascular invasion as a risk factor for recurrence and poor survival. Conclusion This is the largest series of pediatric PD patients. PD is curative for SPN and benign neoplasms. Pancreatic insufficiency is the most common postoperative complication. Outcome is primarily associated with histology.

Background: Image-guided percutaneous core needle biopsy (PCNB) is increasingly utilized to diagnose solid tumors. The objective of this study is to determine whether PCNB is adequate for modern biologic characterization of neuroblastoma. Procedure: A multi-institutional retrospective study was performed by the Pediatric Surgical Oncology Research Collaborative on children with neuroblastoma at 12 institutions over a 3-year period. Data collected included demographics, clinical details, biopsy technique, complications, and adequacy of biopsies for cytogenetic markers utilized by the Children's Oncology Group for risk stratification. Results: A total of 243 children were identified with a diagnosis of neuroblastoma: 79 (32.5%) tumor excision at diagnosis, 94 (38.7%) open incisional biopsy (IB), and 70 (28.8%) PCNB. Compared to IB, there was no significant difference in ability to accurately obtain a primary diagnosis by PCNB (95.7% vs 98.9%, P =.314) or determine MYCN copy number (92.4% vs 97.8%, P =.111). The yield for loss of heterozygosity and tumor ploidy was lower with PCNB versus IB (56.1% vs 90.9%, P

Hirschsprung disease (HD) is a complex surgical and medical problem that appears to have varied health and social outcomes with the age and neurodevelopmental state of patients. In general, long-term outcomes are thought to be good for the majority of patients despite recognized problems with constipation and/or fecal incontinence. However, there are no universally accepted pathways regarding post-operative bowel management programs nor clearly defined follow-up pathways making the current outcome measures difficult to interpret. Further, other factors that may influence outcome including age at the time of procedure and procedure type continue to lack consensus. Improved support of children in resource limited environments and during periods of transition into the adult medical care environment are needed to improve outcome. Recent proliferation of multidisciplinary care teams and consortia may help to better understand outcomes and address current knowledge gaps. Continuing these collaborations will be imperative to continuing improvements in care which may ultimately impact outcome.

Purpose We sought to examine the short-term outcomes following single-stage repair of rectoperineal and rectovestibular fistulae in infants and identify risk factors for wound complication. Methods Patients with a rectoperineal or rectovestibular fistula treated with a single-stage repair beyond the neonatal period (>30days of age) at a pediatric colorectal center (2011–2016) were reviewed. Results 36 patients with a rectoperineal and 7 patients with a rectovestibular fistula were repaired using the Posterior Sagittal Anorectoplasty (PSARP) approach. Median follow-up was 31months. The median age and weight at the time of repair were 166days and 6.5kg. Four patients (11%) suffered a wound complication (3 rectoperineal, 1 rectovestibular). Two required a diverting colostomy to allow wound healing. Two patients suffered skin separation managed with local wound care. All 4 patients experienced satisfactory wound healing without anoplasty stricture. Two different patients developed a stricture of the neo-anus. Age and weight at time of repair, gender, and presence of a genitourinary anomaly were not associated with wound complications. Conclusion Delayed single-stage repair of rectoperineal and rectovestibular fistulae can be performed safely in infants beyond the newborn period. With attentive treatment, satisfactory healing can be anticipated if a wound complication is encountered. Level of Evidence Retrospective Comparative Study, Level III.

A 4-day-old female neonate presented with a black necrotic lesion with circumferential ulceration and erythema located to the left of the umbilicus. ### Prenatal and Birth Histories ### Presentation The infant was admitted to the NICU. An umbilical venous catheter (UVC) was inserted with the use of betadine as the antimicrobial skin preparation and a thin hydrocolloid dressing (DuoDERM®, ConvaTec, Bridgewater, NJ) as a protective skin barrier to secure the UVC. The hydrocolloid dressing was located just left of the umbilicus and placed on intact abdominal skin. The infant was noted to have asymptomatic thrombocytopenia (platelet count 73,000/μL [73×109/L]) and neutropenia (absolute neutrophil count 700/μL [0.70×109/L]), which were attributed to maternal preeclampsia. The infant received 1 dose of granulocyte colony-stimulating factor (G-CSF, 10 μg/kg, subcutaneously) at 2 days of age. Prophylactic antibiotics were not indicated at the time of birth because there were no concerns for infection. The infant underwent extubation to receive nasal noninvasive ventilation within 24 hours of birth. …

Traumatic bronchial avulsion is standardly treated with emergent lobectomy versus primary repair. We reviewed our experience with peri-operative use of extracorporeal membrane oxygenation (ECMO) to manage respiratory failure in two cases of pediatric chest trauma. ECMO can be an effective intervention to overcome respiratory failure, facilitate surgical repair, and promote healing in bronchial injuries as a result of blunt chest trauma in children. Keywords: Pediatric trauma, Respiratory failure, Extracorporeal membrane oxygenation, ECMO, Bronchial avulsion

Introduction Recreation on longboards is gaining in popularity. The purpose of this study is to detail the injury patterns, treatment and management of children with longboarding injuries seen at a level 1 pediatric trauma center. Methods A retrospective review using our trauma registry from 2006 to 2016 of pediatric patients who sustained injuries while riding a longboard. Results Of 12,920 injured children, 64 (0.5%) were treated for injuries that occurred while riding a longboard. Median age was 14.5 years (IQR 13.6, 15.4) and 84% were male. Fifty-one (80%) suffered a traumatic brain injury (TBI) including 32 intracranial hemorrhages (ICH), 17 concussions, and 31 skull fractures. Seven (11%) were wearing helmets. Three patients required neurosurgical intervention. Extremity fractures were the most common reason for surgery. Ninety-six percent of patients were admitted to the hospital with a median length of stay of 1 day (IQR 1, 3). All children survived to discharge. Compared with skateboard injuries during the same period, TBI, ICH, concussion, and skull fractures were all greater. Conclusions TBI ranging from concussion to ICH requiring craniotomy is common in children injured while riding a longboard, and greater than rates after skateboarding injuries. Extremity fracture was the most common reason for operative intervention. Level of evidence III.

Total colonic aganglionosis is a rare phenotype of Hirschsprung disease (HD). While the diagnosis is generally established within the neonatal period there are reports of delayed presentation. In this case, we describe a 9-month old girl with no previous medical or surgical history who presented with a small bowel obstruction. A contrast enema performed as part of the work up demonstrated right sided colonic diverticula with no other anatomical abnormalities. Surgical intervention was required to manage the obstruction and colonic biopsies were consistent with HD. This case highlights the association of the unusual finding of colonic diverticula with total colonic HD in infants.

10024 Background: Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm of intermediate malignancy, predominantly seen in children and young adults. Given its rarity, data are limited. We describe the largest cohort of patients with IMT to date, with an aim to further characterize this poorly understood tumor. Methods: A multi-institutional review was performed at 18 North American hospitals participating in the Pediatric Surgical Oncology Research Collaborative to identify IMT patients ≤39 years, diagnosed from 2000-2018. Descriptive statistics are described as median and interquartile range. Multivariable analysis was used to identify predictors of event free survival (EFS). Results: 182 patients were identified with a median age of 11 years (5-17); 52% were female. 33% of tumors were thoracic, 26% abdominal/pelvic, 20% head/neck, and 14% genitourinary. Common presenting symptoms included pain (29%), respiratory symptoms (24%), weight loss (12%), and a palpable mass (10%). Median tumor size was 3.9 cm (1.9-6.5). Anaplastic lymphoma kinase (ALK) overexpression was identified via immunohistochemistry in 53% of patients tested. One third of patients had abnormal cytogenetics, with 12% of the entire cohort having an ALK mutation. 7% of patients had distant disease at diagnosis. 13% of patients received neoadjuvant therapy including chemotherapy (3%), ALK inhibitor (4%), radiation (0.5%), non-steroidal anti-inflammatories (NSAIDs) (7%), or steroids (2%). Of those who underwent resection with known margin status (n = 158), 66% had complete resection, 20% had microscopic positive margins, and 14% had gross residual disease. Just over 40% of patients had an en bloc resection of involved organs, most commonly lung (26%). 21% of patients received adjuvant therapy, including chemotherapy (3%), ALK inhibitor (9%), radiation (0.5%), NSAIDs (8%), or steroids (5%). 12% of all patients received an ALK inhibitor: 24% neoadjuvant, 62% adjuvant, and 14% without surgery. Median follow-up time was 36 months (14-69). Overall 5-year survival (OS) was 95% and 5-year EFS was 80%. Predictors of recurrence included respiratory symptoms, larger tumor size, or distant disease at diagnosis. Gender, race, age and primary site were not predictive of EFS. Likewise, there was no association of ALK overexpression or ALK mutation with EFS. The presence of gross or microscopic margins following resection was not associated with recurrence. Conclusions: IMT is a rare tumor with favorable OS. Five year recurrence rate was 20%. Presenting with respiratory symptoms, larger tumor size, or metastatic disease was associated with recurrence, while ALK positivity was not. Notably, a positive margin after resection was not associated with increased recurrence, indicating that aggressive attempts at surgical resection that would compromise form or function may not be warranted.

Purpose Awareness of equestrian related injury remains limited. Studies evaluating children after equestrian injury report under-utilization of safety equipment and rates of operative intervention as high as 33%. Methods We hypothesized that helmets are underutilized during equestrian activity and lack of use is associated with increased traumatic brain injury. We queried the trauma database of a level one pediatric trauma center for all cases of equestrian and rodeo related injury from 2005 to 2015. Analysis was conducted using SAS 9.4. Results Of 312 children identified, 142 were assessed for use of a helmet. Only 28 children (19.7%) had documented use of a helmet. Most injuries occurred while riding a horse (83%) or bull (13%) with traumatic brain injury being the most common injury (51%). Helmet use was associated with decreased ISS (7.1 vs. 11.3, p p =0.03), and ICU admission (10.7% vs. 29%, p =0.05). Multivariable analysis reveals lack of helmet use to be an independent predictor of TBI (OR 2.5, 95% CI 1.1–6.3). Conclusion Helmets are underutilized by children during equestrian related activity. Increased awareness of TBI and education encouraging helmet use may decrease morbidity associated with equestrian activities. Level of Evidence Retrospective comparative study, Level III.

BACKGROUND: Optimal management of neutropenic appendicitis (NA) in children undergoing cancer therapy remains undefined. Management strategies include upfront appendectomy or initial nonoperative management. We aimed to characterize the effect of management strategy on complications and length of stay (LOS) and describe implications for chemotherapy delay or alteration. METHODS: Sites from the Pediatric Surgery Oncology Research Collaborative performed a retrospective review of children with NA over a 6-year period. RESULTS: Sixty-six children, with a median age of 11 years (range 1–17), were identified with NA while undergoing cancer treatment. The most common cancer diagnoses were leukemia (62%) and brain tumor (12%). Upfront appendectomy was performed in 41% of patients; the remainder had initial nonoperative management. Rates of abscess or perforation at diagnosis were equivalent in the groups (30% vs 24%; P = .23). Of patients who had initial nonoperative management, 46% (17 of 37) underwent delayed appendectomy during the same hospitalization. Delayed appendectomy was due to failure of initial nonoperative management in 65% (n = 11) and count recovery in 35% (n = 6). Cancer therapy was delayed in 35% (n = 23). Initial nonoperative management was associated with a delay in cancer treatment (46% vs. 22%, P = .05) and longer LOS (29 vs 12 days; P = .01). Patients who had initial nonoperative management and delayed appendectomy had a higher rate of postoperative complications (P CONCLUSIONS: In pediatric patients with NA from oncologic treatment, upfront appendectomy resulted in lower complication rates, reduced LOS, and fewer alterations in chemotherapy regimens compared to initial nonoperative management.

To examine surgical outcomes of children with pancreaticoduodenal injuries at a Quaternary Level I pediatric trauma center. We queried a prospectively maintained trauma database of a level one pediatric trauma center for all cases of pancreatic and/or duodenal injury from 2002 to 2017. Analysis was conducted using JMP 13.1.0. 170 children presented with pancreatic and/or duodenal injury. 13 (7.7%) suffered a combined injury and this group forms the basis for this report with mean ISS of 22.8 (± 15.1), RTS2 of 6.4(± 2.1), and median age of 6.6 (1.3–13.5) years. Child abuse (31%) and bicycle injuries (23%) were the most common mechanisms. 8/13 (61.5%) required operative intervention. Higher AAST pancreatic and duodenal injury grade (2.9 vs. 1.2, p = 0.05 and 3.6 vs. 1.4, p =

Data support use of video-assisted thoracoscopic surgery (VATS) for safe and effective resection of paraspinal masses in children. Our aim was to describe outcomes following this operation using a novel technique and to compare its use with that of other established techniques.We performed a retrospective chart review of all children (18 years old) undergoing thoracoscopic resection of paraspinal masses in 2000-2011. Demographics, operative details, and clinical outcomes were summarized and compared between those undergoing resection using a Harmonic (Ethicon Endo-Surgery, Blue Ash, OH) blade scalpel (HBS) and those who did not.Sixteen cases were identified (median age, 57.5 months; range, 2-204 months). Six cases (37%) underwent VATS with use of the HBS, and 10 (63%) did not. Demographic and clinical factors were well matched. Median tumor diameter was larger in the HBS group (49.2 cm(3) versus 18.7 cm(3); P=.07). Operative time was similar between groups (121 versus 138 minutes; P=.25), as was the estimated blood loss (10 mL versus 30 mL; P=.91) and chest tube duration (2.1 versus 1.8 days; P=.78). Three cases of Horner's syndrome developed in the standard resection group, and one complication occurred in the HBS group.The Harmonic blade scalpel can be used as a simple alternative to standard dissection techniques for thoracoscopic resection of paraspinal masses in children.

The intestinal barrier becomes compromised during systemic inflammation, leading to the entry of luminal bacteria into the host and gut origin sepsis. Pathogenesis and treatment of inflammatory gut barrier failure is an important problem in critical care. In this study, we examined the role of cyclooxygenase-2 (COX-2), a key enzyme in the production of inflammatory prostanoids, in gut barrier failure during experimental peritonitis in mice. I.p. injection of LPS or cecal ligation and puncture (CLP) increased the levels of COX-2 and its product prostaglandin E2 (PGE2) in the ileal mucosa, caused pathologic sloughing of the intestinal epithelium, increased passage of FITC-dextran and bacterial translocation across the barrier, and increased internalization of the tight junction (TJ)-associated proteins junction-associated molecule-A and zonula occludens-1. Luminal instillation of PGE2 in an isolated ileal loop increased transepithelial passage of FITC-dextran. Low doses (0.5-1 mg/kg), but not a higher dose (5 mg/kg) of the specific COX-2 inhibitor Celecoxib partially ameliorated the inflammatory gut barrier failure. These results demonstrate that high levels of COX-2-derived PGE2 seen in the mucosa during peritonitis contribute to gut barrier failure, presumably by compromising TJs. Low doses of specific COX-2 inhibitors may blunt this effect while preserving the homeostatic function of COX-2-derived prostanoids. Low doses of COX-2 inhibitors may find use as an adjunct barrier-protecting therapy in critically ill patients.

Laparoscopic repair of congenital duodenal obstruction has become popularized over the past decade. Comparative data on outcomes, however, are sparse. We hypothesized that laparoscopic repair of congenital duodenal obstruction could be performed with similar outcomes to traditional open repair.Medical records for all cases of congenital duodenal obstruction from 2005 to 2011 at three academic teaching hospitals were retrospectively reviewed. Patients were excluded from the analysis if they had confounding surgical diseases, did not have duodenoduodenostomy during the first hospital admission, had the repair performed before transfer from a referring hospital, or weighed less than 1.7 kg at the time of surgery. Analysis was performed as intention to treat, with laparoscopic converted to open cases included in the laparoscopic group.Sixty-four cases were included in the analysis (44 open, 20 laparoscopic). Baseline characteristics were similar between the two groups with the exception that the open group, on average, underwent repair later than the laparoscopic group (6 days versus 4 days, respectively). Seven laparoscopic cases were converted to an open procedure (35%), most commonly for difficulty in exposing the decompressed distal duodenum. Laparoscopic repair did take significantly longer than open repair (145 minutes versus 96 minutes, respectively), but clinical outcomes were similar. Complications were rare and were similar between methods of repair. Two patients in the laparoscopic group required subsequent open revision.Laparoscopic duodenoduodenostomy for congenital duodenal obstruction is a technically challenging procedure with a steep learning curve. Despite a relatively high conversion rate, clinical outcomes remained similar to the traditional open repair in selected patients.

Gender may influence outcomes following traumatic brain injury (TBI) although the mechanism is unknown. Animal TBI studies suggest that gender differences in endogenous hormone production may be the source. Limited retrospective clinical studies on gender present varied conclusions. Pediatric patients represent a unique population as pubescent children experience up-regulation of endogenous hormones that varies dramatically by gender. Younger children do not have these hormonal differences. The aim of this study was to compare pubescent and prepubescent females with males after isolated TBI to identify independent predictors of mortality.We performed a retrospective review of the National Trauma Data Bank Research Data Sets from 2007 and 2008 looking at all blunt trauma patients 18 years or younger who required hospital admission after isolated, moderate-to-severe TBI, defined as head Abbreviated Injury Scale (AIS) score 3 or greater. We excluded all individuals with AIS score of 3 or greater for any other region to limit the confounding effect of comorbidities. Based on the median age of menarche, we defined two age groups as follows: prepubescent (0-12 years) and pubescent (12 years). Analysis was performed to compare trauma profiles and outcomes between groups. Our primary outcome measure was in-hospital mortality.A total of 20,280 patients met inclusion criteria; 10,135 were prepubescent, and 10,145 were pubescent. Overall mortality was 6.9%, and lower mortality was noted among prepubescent patients compared with pubescent (5.2% vs. 8.6%, p0.0001). Although female gender did not predict reduced mortality in the prepubescent cohort (adjusted odds ratio, 1.05; 95% confidence interval, 0.85-1.30; p = 0.63), female gender was associated with reduced mortality in the pubescent (adjusted odds ratio, 0.78; 95% confidence interval, 0.65-0.93; p = 0.007).In contrast to prepubescent female gender, pubescent female gender predicts reduced mortality following isolated, moderate-to-severe TBI. Endogenous hormonal differences may be a contributing factor and require further investigation.Prognostic study, level III.

Hirschsprung disease (HSCR) is a multigenic condition with variable presentation. Most commonly, it presents in the neonatal period as a functional intestinal obstruction secondary to failure of caudal migration of the enteric nervous system. Classically, this manifests as dilated proximal bowel and constricted distal bowel with absent ganglia and hypertrophic nerve trunks. When recognized early, medical and surgical therapies can be instituted to minimize associated morbidity and mortality. This article reviews current understanding of the etiology of HSCR, its multigenic associations, the historical evolution of HSCR diagnosis and treatment, and current HSCR therapies.

Perianal perforating disease (PF) has been reported in approximately 15% of children with Crohn's disease (CD). It is unknown whether children who present with PF at the time of diagnosis have a different course than those that develop PF while on therapy.From a prospective, single institution observational registry of children diagnosed with CD, we identified children with perianal perforating CD, defined as perianal abscesses and/or fistulae. Patients who presented with perianal perforating CD (PF-CD0) were compared to those who developed perianal perforating CD (PF-CD1) after initial diagnosis.Thirty-eight of 215 (18%) children with CD had PF-CD during a median follow up of 4.5 years. Patients with PF-CD0 (n=26) tended to be more likely male (81% vs. 50%, p=0.07) and younger (9.3 yrs vs. 12.5 yrs, p=0.02). PF-CD1 (n=12) patients were more likely to require diverting ileostomy (42% vs. 8%, p=0.02) and colectomy (33% vs. 4%, p=0.03). Multivariable analysis predicted increased rate of diverting ileostomy in the PF-CD1 group (p=0.007, OR 19.1, 95% CI 1.6-234.8).Pediatric CD patients who develop PF while on therapy for CD have a more severe phenotype and are more likely to require diverting ileostomy or colectomy compared to those who present with PF-CD.

Our aim was to compare outcomes of children undergoing laparoscopic cholecystectomy with laparoscopic common bile duct exploration (LC+CBDE) to those undergoing laparoscopic cholecystectomy with adjunctive endoscopic retrograde cholangiopancreatography (LC+ERCP).We performed a two-center retrospective chart review of all children (18 years) undergoing LC+CBDE or LC+ERCP between January 2000 and July 2011. Wilcoxon test was performed on continuous variables and logistic regression modeling on categorical data. A P value0.05 was considered significant. Outcomes with a P value0.2 were selected for multivariable analysis.Forty-two patients were identified. Twenty-four (57%) underwent LC+ERCP, and eighteen (43%) underwent LC+CBDE. Demographic and clinical factors were well matched between groups. Total operative time was similar between groups (157 min vs. 152 min, P = .26). LC+CBDE patients had zero major complications and five minor complications (retained stone: 3, pancreatitis: 1, late recurrence: 1). LC+ERCP patients experienced two major complications (duodenal perforation: 1, bleeding requiring transfusion: 1), and four minor complications (pancreatitis: 2, retained stone: 2, P = .57). Median length of stay was significantly longer (15.7 days vs. 6.6 days, P = .02), and median hospital cost was significantly higher ($18,132 vs. $12,735, P.01) in the LC+ERCP group. Multivariable analysis revealed that cost was significantly lower in patients undergoing LC+CBDE (P = .05, OR= 0.71; 95% CI: 0.51-0.97).LC+CBDE at the time of cholecystectomy is associated with decreased length of stay, decreased cost, and has similar or improved morbidity compared to LC+ERCP.

Alcohol intoxication in pediatric trauma is underappreciated. The aim of this study was to characterize alcohol screening rates in pediatric trauma.The Los Angeles County Trauma System Database was queried for all patients aged ≤ 18 years who required admission between 2003 and 2008. Patients were compared by age and gender.A total of 18,598 patients met the inclusion criteria; 4,899 (26.3%) underwent blood alcohol screening, and 2,797 (57.1%) of those screened positive. Screening increased with age (3.3% for 0-9 years, 15.1% for 10-14 years, and 45.4% for 15-18 years; P.01), as did alcohol intoxication (1.9% for 0-9 years, 5.8% 10-14 years, and 27.3% for 15-18 years; P.01). Male gender predicted higher mortality in those aged 15 to 18 years (adjusted odds ratio, 1.7; P.01), while alcohol intoxication did not (adjusted odds ratio, .97; P = .84).Alcohol intoxication is common in adolescent trauma patients. Screening is encouraged for pediatric trauma patients aged ≥10 years who require admission.

Hirschsprung-associated enterocolitis remains the greatest cause of morbidity and mortality in children with Hirschsprung disease. This chapter details the various approaches used to treat and prevent this disease process. This includes prevention of complications, such as stricture formation, prophylaxis with rectal washouts, and identification of high-risk individuals. The chapter also details approaches to diagnose Hirschsprung-associated enterocolitis as well as to exclude other etiologies.

Aim Our hypothesis is that an improvised low-cost peripheral nerve stimulator is equally effective compared with a high-cost muscle stimulator for anorectal malformation surgery. Methods A modified Micro Stim (MS) peripheral nerve stimulator was compared with the Pena Muscle Stimulator (PS2) on identification of muscle groups during surgery, tissue energy delivery, and cost. A consecutive series of 6 patients (5 boys) with weight 5.9 to 11 kg underwent posterior sagittal anorectoplasty (PSARP). The types of malformations included four prostatic fistulae, one bulbar fistula, and one vestibular fistula. In the course of each operation, both MS and PS2 devices were used to identify muscle groups in a head-to-head comparison with video documentation. The energy delivery was determined using a digital oscilloscope at settings used during the operations (#9 on the MS and 120 mA on the PS2). Price quotes for each device were obtained from commercial vendors. Results The subjective assessment of the surgical team was that both devices demonstrated equivalent utility in identifying critical muscle groups, although the MS probe was less convenient to use. There were no intraoperative complications, including tissue burns with either device, and no postoperative complications. The energy delivery of the devices was similar, 23.5 mW and 25.3 mW for the MS and PS2, respectively. The cost of the MS was US$ 162 and the PS2 was US$ 12,371. Conclusion The MS nerve stimulator demonstrated equivalent effectiveness compared with the PS2 muscle stimulator at 75-fold lower cost. The MS device may serve as a low-cost effective tool for PSARP.

Background and Objectives Optimal adjuvant treatment for patients with Stage I/II colon cancer with micrometastases (MM) is unknown. Because there is no known adjuvant treatment-related benefit, we evaluated whether MM influenced treatment decisions. Method Review of a national survey from members of the SSO and ASCO. Results Of 602 survey responses, 305 (51%) stated that MM had significant prognostic value, 250 (42%) were unsure, and 47 (7%) did not believe that MM held prognostic value. Three hundred seventy-four (63%) would offer adjuvant therapy in the setting of MM, while 222 (37%) would not. Only 15% routinely performed IHC on lymph nodes. Medical oncologists were more likely to recommend adjuvant therapy compared to surgical oncologists (68% vs. 51%, P = 0.001). Conclusions MM in colon cancer apparently influenced adjuvant treatment decisions absent known prognostic benefit. Prospective trials are needed to improve the selection of patients for systemic chemotherapy in early, node-negative colon cancer. J. Surg. Oncol. 2012; 106:119–122. © 2012 Wiley Periodicals, Inc.

We hypothesize that use of postoperative heparin deep vein thrombosis prophylaxis influences development of surgical site infection (SSI) after colorectal surgery. From July 2008 to June 2009, patients undergoing an abdominal operation by colorectal surgeons at a single university-affiliated teaching hospital were prospectively followed and more than 80 variables collected. One hundred eighty-one patients were identified. Forty-five per cent (n = 82) received heparin prophylaxis and 55 per cent (n = 99) did not. SSI occurred in 23 per cent (n = 19) of patients receiving heparin versus 9 per cent (n = 9) who did not ( P = 0.02). Univariate analysis found SSI to be associated with heparin ( P = 0.02) and increased operative time ( P = 0.03). Multivariate analysis showed that SSI was associated only with heparin use ( P = 0.04; OR, 2.6; 95% CI, 1.1 to 6.6).

Smartphones are one of the fastest-growing sectors in the technology industry, and they continue to evolve to combine faster processors, better memory, and more efficient operating systems into a compact handheld device. Smartphones also offer a dynamic tool for use in personal and professional environments.1 The role of smartphones in medicine continues to expand as additional uses and applications emerge. An estimated 80% of physicians, trainees, and medical students use smartphones, and this percentage is expected to increase.2–,4 Smartphones provide a multifaceted platform for mobile health care, allowing users to access a vast amount of information and interact with resources conveniently and quickly.5 Applications range from patient monitoring to use as a tool for diagnosis, to communication and medical education. Recent advances in smartphone technology have led many educators to extend their teaching methods into the mobile learning environment, providing an “anytime, anywhere” approach to learning. Mobile learning has been shown to have efficacy within the traditional classroom environment, and brief communications via short message service (SMS) supplement interactive classroom sessions, resulting in enhanced interest in and attention to classroom activity.6 Given the nature of graduate medical education (GME), where trainees are expected to assimilate a vast amount of information that is constantly evolving, and often are away from traditional classroom settings, the benefits of mobile learning with its uninterrupted access to educational resources can be particularly advantageous. In this perspective, we characterize the current and potential uses of smartphone technology in GME and provide recommendations for future studies on incorporating smartphone technology as an educational platform.

Pyloric atresia with epidermolysis bullosa (EB) dystrophica is a rare entity that may not be immediately recognized. We describe the fourth confirmed case of pyloric atresia associated with the dystrophic subtype of EB diagnosed by standard pathologic measures, and discuss the clinical disease features and recent advances in the pathophysiology.