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Background: There is no consensus on the appropriate duration of postoperative antibiotics for complicated appendicitis in children. Commonly used antibiotic endpoints include normalization of white blood cell count (WBC) or completion of a minimum number of prespecified treatment days. We compared clinical outcomes resulting from varying postoperative antibiotic protocols for complicated appendicitis in children., Methods: National Surgical Quality Improvement Program Pediatric (NSQIP-P) data from nine children's hospitals was used to identify a retrospective cohort of children (<18 years) who underwent laparoscopic appendectomy from 2021 to 2023 with intraoperative findings of complicated appendicitis. Participating hospitals were classified into four groups based on discharge protocol: 1) no discharge antibiotics, 2) oral antibiotics for elevated WBC on the day of discharge, 3) oral antibiotics to complete a minimum number of total antibiotic days, and 4) routine discharge antibiotics regardless of inpatient antibiotic duration. Univariate analysis was completed between groups., Results: We identified 1342 patients with complicated appendicitis who underwent laparoscopic appendectomy. Patients were similar by age and BMI. Median length of stay (5 days) and rate of post-discharge percutaneous drainage (9.4 %) were highest at the center with a standardized minimum duration of discharge antibiotics. There were no statistical differences among treatment groups for surgical site infection (5.7-9.8 %), emergency department visits (9.0-15.6 %), or readmissions within 30 days (2.9-7.6 %)., Conclusion: The incidence of SSI and readmission following appendectomy did not differ based on the discharge antibiotic protocol, however, the incidence of post-discharge drainage was highest in the center with protocolized discharge antibiotics. These findings highlight an opportunity to minimize unnecessary blood draws and extended postoperative antibiotics., Level of Evidence: III., (Copyright © 2025 The Authors. Published by Elsevier Inc. All rights reserved.)

Introduction: We sought to understand the impact of locum tenens surgeons on pediatric surgical care delivery., Methods: We conducted a cross-sectional survey of Children's Hospital Association pediatric surgical practices. Anonymous electronic surveys were used to investigate locum tenens utilization, primary reason for use, limitations on clinical activities, and variations in practice standards or quality. Bivariate analysis and multivariable logistic regression were performed to evaluate for associations between practice characteristics and locum tenens use., Results: Of 172 practices, 71% (n = 122) completed the survey. Median hospital size was 203 beds (interquartile range = 130-350). Median number of surgeons per practice was 5 (interquartile range = 3-8). Thirty-seven practices (30%) employed locum tenens at primary (n = 27) or satellite (n = 12) sites. Locum tenens utilization was higher in suburban (odds ratio [OR] = 3.78, P = 0.006) and rural (OR = 4.96, P = 0.041) locations and lower at sites with a level 4 neonatal intensive care unit (OR = 0.35, P = 0.035). Most (51%) used locum tenens ≥ 1 time monthly but < 1 time weekly and for ongoing or interim coverage (87%). In total, 14% of practices reported clinical restrictions for locum tenens surgeons, including limitations on extracorporeal membrane oxygenation, neonatal index cases, and operative trauma. Most (76%) practices using locum tenens reported variations in practice standards or quality; all were perceived as negative (57%) or neutral (43%)., Conclusions: Locum tenens providers are utilized most commonly in suburban and rural sites and hospitals without the highest level of neonatal intensive care. While locum tenens surgeons may help maintain access to pediatric surgical care where gaps exist, there may be a need to improve the quality and reliability of care rendered., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Hirschsprung associated enterocolitis (HAEC) is a challenging problem in a subset of children with Hirschsprung disease (HD). In refractory cases, fecal diversion may be required. The aim of this study was to characterize patients who require fecal diversion for HAEC management and examine their long-term outcomes., Methods: A retrospective review of prospectively collected data within the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry was performed. All children with a history of a corrective procedure for HD and documented post-operative HAEC were included. Our primary outcome was diversion to manage HAEC after pull-through and secondary outcomes included incidence of recurrent HAEC, fecal continence following ostomy closure, and bowel management needs at last follow-up., Results: 951 patients were identified with a history of HD and 852 had undergone pull-through. 339/852 (39.8 %) had documented HAEC and 75/339 (22.1 %) required an ostomy, 54 (72 %) of which underwent reversal at a median of 239.5 days. After reversal, 10/54 (18.5 %) required repeat diversion for recurrent HAEC. 30/75 (40 %) had their pull-through revised. Median age at last follow-up was 5.3 [3.4, 9] years. At time of last follow-up, 40.3 % were toilet-trained for stool and 42.9 % required long term enema-based therapy., Conclusion: Fecal diversion for refractory HAEC was common in our study. Slightly more than half of the patients underwent successful closure of their stoma during over 4.5 years of follow-up, while less than half were toilet-trained for stool at their last visit. These children may represent a unique cohort that warrants further investigation., Level of Evidence: Level VI., Competing Interests: Conflict of interest The authors of this paper have no disclosures or conflicts of interest to report relevant to this project., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare tumor for which there are few evidence-based guidelines. The aim of this study was to define current management strategies and outcomes for these patients using a multi-institutional dataset curated by the Pediatric Surgical Oncology Research Collaborative., Methods: Data were collected retrospectively for patients with UESL treated across 17 children's hospitals in North America from 1989 to 2019. Factors analyzed included patient and tumor characteristics, PRETEXT group, operative details, and neoadjuvant/adjuvant regimens. Event-free and overall survival (EFS, OS) were the primary and secondary outcomes, respectively., Results: Seventy-eight patients were identified with a median age of 9.9 years [interquartile range [IQR): 7-12]. Twenty-seven patients underwent resection at diagnosis, and 47 patients underwent delayed resection, including eight liver transplants. Neoadjuvant chemotherapy led to a median change in maximum tumor diameter of 1.6 cm [IQR: 0.0-4.4] and greater than 90% tumor necrosis in 79% of the patients undergoing delayed resection. R 0 resections were accomplished in 63 patients (81%). Univariate analysis found that metastatic disease impacted OS, and completeness of resection impacted both EFS and OS, while multivariate analysis revealed that R 0 resection was associated with decreased expected hazards of experiencing an event [hazard ratio (HR): 0.14, 95% confidence interval (CI): 0.04-0.6]. At a median follow-up of 4 years [IQR: 2-8], the EFS was 70.0% [95% CI: 60%-82%] and OS was 83% [95% CI: 75%-93%]., Conclusion: Complete resection is associated with improved survival for patients with UESL. Neoadjuvant chemotherapy causes minimal radiographic response, but significant tumor necrosis., (© 2024 Wiley Periodicals LLC.)

Background: Pediatric colorectal specialists care for patients with a variety of defecation disorders. Anorectal (AR) manometry testing is a valuable tool in the diagnosis and management of these children. This paper provides a summary of AR manometry techniques and applications as well as a review of AR manometry findings in pediatric patients with severe defecation disorders referred to a pediatric colorectal center. This is the first study describing multi-year experience using a portable AR manometry device in pediatric patients., Methods: An electronic medical record review was performed (1/2018 to 12/2023) of pediatric patients with defecation disorders who had AR manometry testing. Demographics, diagnostic findings, and outcomes are described., Key Results: A total of 297 unique patients (56.9% male, n = 169) had AR manometry testing. Of these, 72% (n = 188) had dyssynergic defecation patterns, of which 67.6% (n = 127) had fecal soiling prior to treatment. Pelvic rehabilitation (PR) was administered to 35.4% (n = 105) of all patients. A total of 79.5% (n = 58) of the 73 patients that had fecal soiling at initial presentation and completed PR with physical therapy and a bowel management program were continent after therapy. AR manometry was well tolerated, with no major complications., Conclusions: AR manometry is a simple test that can help guide the management of pediatric colorectal surgical patients with defecation disorders. As a secondary finding, PR is a useful treatment for patients with dyssynergic stooling.

Objective: The purpose of this study was to describe management and outcomes from a contemporary cohort of children with Wilms tumor complicated by inferior vena caval thrombus., Background: The largest series of these patients was published almost 2 decades ago. Since then, neoadjuvant chemotherapy has been commonly used to manage these patients, and outcomes have not been reported., Methods: Retrospective review of 19 North American centers between 2009 and 2019. Patient and disease characteristics, management, and outcomes were investigated and analyzed., Results: Of 124 patients, 81% had favorable histology (FH), and 52% were stage IV. IVC thrombus level was infrahepatic in 53 (43%), intrahepatic in 32 (26%), suprahepatic in 14 (11%), and cardiac in 24 (19%). Neoadjuvant chemotherapy using a 3-drug regimen was administered in 82% and postresection radiation in 90%. Thrombus level regression was 45% overall, with suprahepatic level showing the best response (62%). Cardiopulmonary bypass (CPB) was potentially avoided in 67%. The perioperative complication rate was significantly lower after neoadjuvant chemotherapy [(25%) vs upfront surgery (55%); P =0.005]. CPB was not associated with higher complications [CPB (50%) vs no CPB (27%); P =0.08]. Two-year event-free survival was 93% and overall survival was 96%, higher in FH cases (FH 98% vs unfavorable histology/anaplastic 82%; P =0.73). Neither incomplete resection nor viable thrombus cells affected event-free survival or overall survival., Conclusions: Multimodal therapy resulted in excellent outcomes, even with advanced-stage disease and cardiac extension. Neoadjuvant chemotherapy decreased the need for CPB to facilitate resection. Complete thrombectomy may not always be necessary., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc.)

Purpose: The utilization of home antibiotic therapy following surgery for complicated pediatric appendicitis is highly variable. In 2019, we stopped home antibiotic therapy in this cohort at our institution. We sought to evaluate our outcomes following this protocol change., Methods: We queried our institutional NSQIP Pediatrics data for all children undergoing appendectomy for complicated appendicitis between January 2015 and May 2022. We identified two cohorts: those discharged with home antibiotics (1/1/15-4/30/19) and those discharged with no home antibiotics (5/1/19-4/30/22). Both groups were treated with response based parenteral antibiotics while hospitalized and discharged when clinically well. Our primary outcome was postoperative deep organ space infection requiring intervention (drainage, aspiration, reoperation, or antibiotics). Secondary outcomes included length of stay, superficial site infection, Clostridium difficile colitis, ER visits, post-operative CT imaging, and readmission., Results: There were 185 patients in the home antibiotic group (83% discharged with antibiotics) and 121 patients in the no home antibiotic group (8.3% discharged with antibiotics). There were no significant differences in deep organ space infection requiring intervention (7% vs. 7.4%, p = 1.0). Our length of stay was not different (4.5 days vs. 3.95 days, p = 0.32), nor were other secondary outcomes or patient characteristics. All patients had documented follow-up., Conclusions: We did not identify differences in deep organ space infections, length of stay or other events after eliminating home antibiotic therapy in our complicated appendicitis cohort. The use of home antibiotics following surgery for complicated appendicitis should be reconsidered., Level of Evidence: III., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: The Coronavirus Disease 2019 pandemic provided a natural experiment to study the effect of social distancing on the risk of developing Hirschsprung's Associated Enterocolitis (HAEC)., Methods: Using the Pediatric Health Information System (PHIS), a retrospective cohort study of children (<18 years) with Hirschsprung's Disease (HSCR) across 47 United States children's hospitals was performed. The primary outcome was HAEC admissions per 10,000 patient-days. The exposure (COVID-19) was defined as April 2020-December 2021. The unexposed (historical control) period was April 2018-December 2019. Secondary outcomes included sepsis, bowel perforation, intensive care unit (ICU) admission, mortality, and length of stay., Results: Overall, we included 5707 patients with HSCR during the study period. There were 984 and 834 HAEC admissions during the pre-pandemic and pandemic periods, respectively (2.6 vs. 1.9 HAEC admissions per 10,000 patient-days, incident rate ratio [95% confidence interval]: 0.74 [0.67, 0.81], p < 0.001). Compared to pre-pandemic, those with HAEC during the pandemic were younger (median [IQR]: 566 [162, 1430] days pandemic vs. 746 [259, 1609] days pre-pandemic, p < 0.001) and more likely to live in the lowest quartile of median household income zip codes (24% pandemic vs. 19% pre-pandemic, p = 0.02). There were no significant differences in rates of sepsis (6.1% pandemic vs. 6.1% pre-pandemic, p > 0.9), bowel perforation (1.3% pandemic vs. 1.2% pre-pandemic, p = 0.8), ICU admissions (9.6% pandemic vs. 12% pre-pandemic, p = 0.2), mortality (0.5% pandemic vs. 0.6% pre-pandemic, p = 0.8), or length of stay (median [interquartile range]: 4 [(Pastor et al., 2009; Gosain and Brinkman, 2015) 2,112,11 days pandemic vs. 5 [(Pastor et al., 2009; Tang et al., 2020) 2,102,10 days pre-pandemic, p = 0.4)., Conclusions: The COVID-19 pandemic was associated with significantly decreased incidence of HAEC admissions across US children's hospitals. Possible etiologies such as social distancing should be explored., Level of Evidence: II., Competing Interests: Conflict of interest The authors declare that they have NO affiliations with or involvement in any organization or entity with any financial interest in the subject matter or materials discussed in this manuscript., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: The incidence and optimal management of rectal prolapse following repair of an anorectal malformation (ARM) has not been well-defined., Methods: A retrospective cohort study was performed utilizing data from the Pediatric Colorectal and Pelvic Learning Consortium registry. All children with a history of ARM repair were included. Our primary outcome was rectal prolapse. Secondary outcomes included operative management of prolapse and anoplasty stricture following operative management of prolapse. Univariate analyses were performed to identify patient factors associated with our primary and secondary outcomes. A multivariable logistic regression was developed to assess the association between laparoscopic ARM repair and rectal prolapse., Results: A total of 1140 patients met inclusion criteria; 163 (14.3%) developed rectal prolapse. On univariate analysis, prolapse was significantly associated with male sex, sacral abnormalities, ARM type, ARM complexity, and laparoscopic ARM repairs (p < 0.001). ARM types with the highest rates of prolapse included rectourethral-prostatic fistula (29.2%), rectovesical/bladder neck fistula (28.8%), and cloaca (25.0%). Of those who developed prolapse, 110 (67.5%) underwent operative management. Anoplasty strictures developed in 27 (24.5%) patients after prolapse repair. After controlling for ARM type and hospital, laparoscopic ARM repair was not significantly associated with prolapse (adjusted odds ratio (95% CI): 1.50 (0.84, 2.66), p = 0.17)., Conclusion: Rectal prolapse develops in a significant subset of patients following ARM repair. Risk factors for prolapse include male sex, complex ARM type, and sacral abnormalities. Further research investigating the indications for operative management of prolapse and operative techniques for prolapse repair are needed to define optimal treatment., Type of Study: Retrospective cohort study., Level of Evidence: II., Competing Interests: Conflicts of interest The authors have no conflicts of interest to declare., (Copyright © 2023. Published by Elsevier Inc.)

Background: Numerous studies have demonstrated a variety of social inequalities within pediatric and young adult patients with solid tumors. This systematic review examines and consolidates the existing literature regarding disparities in pediatric and young adult solid tumor oncology., Procedure: A MeSH search was performed on the following databases: MEDLINE, PubMed, OvidSP Cochrane, Central, Embase, Cinhal, and Scopus. The systematic review was performed using Rayyan QCRI., Results: Total 387 articles were found on the initial search, and 34 articles were included in final review. Twenty-seven studies addressed racial and ethnic disparities; 23 addressed socioeconomic disparities. Patients with Hispanic ethnicity, Black race, and lower socioeconomic status were more likely to present at later stages, have differences in treatments and higher mortality rates., Conclusion: This qualitative systematic review identified both racial and socioeconomic disparities in pediatric cancer patients across a variety of solid tumor types. Patients with Hispanic ethnicity, Black race, and lower socioeconomic status are associated with disparities in stage at presentation, treatment, and outcome. Characterization of existing disparities provides the evidence necessary to support changes at a systemic level., (© 2023 Wiley Periodicals LLC.)

Introduction: This study evaluated North American pediatric surgeons' opinions and knowledge of business and economics in medicine and their perceptions of trends in their healthcare delivery environment., Methods: We conducted an elective online survey of 1119 American Pediatric Surgical Association members. Over 8 mo, we iteratively developed the survey focused on four areas: opinion, knowledge, current practice environment, and trends in practice environment over the past 5 y., Results: We received 227 (20.3%) complete surveys from pediatric surgeons. One hundred ninety four (85.5%) perceive healthcare as a business and most (85.9%) believe healthcare decisions may affect patients' out-of-pocket expenses. More than half (51.1%) of surgeons believe it has become more challenging to perform emergent cases and most believe staff quality has decreased for elective (56.4%) and emergent (63.0%) cases over the past 5 y., Conclusions: Pediatric surgeons recognize that medicine is a business and have concerns regarding the decreasing quality of operating room staff and the increasing difficulty providing surgical care over the last 5 y., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Purpose: Surgical site infection (SSI) remains a significant source of patient morbidity and resource utilization in children undergoing colorectal surgery. We examined the utility of a protocolized perioperative care bundle in reducing SSI in pediatric patients undergoing colorectal surgery., Methods: We conducted a prospective cohort study of patients ≤18 years of age undergoing colorectal surgery at ten United States children's hospitals. Using a perioperative care protocol comprising eight elements, or "colon bundle", we divided patients into low (1-4 elements) or high (5-8 elements) compliance cohorts. Procedures involving colorectal repair or anastomosis with abdominal closure were included. Demographics and clinical outcomes were compared between low and high compliance cohorts. Compliance was compared with a retrospective cohort. The primary outcome was superficial SSI incidence at 30 days., Results: Three hundred and thirty-six patients were included in our analysis: 138 from the low compliance cohort and 198 from the high compliance cohort. Age and gender were similar between groups. Preoperative diagnosis was similar except for more patients in the high compliance cohort having inflammatory bowel disease (18.2% versus 5.8%, p<0.01). The most common procedure performed was small bowel to colorectal anastomosis. Wound classification and procedure acuity were similar between groups. Superficial SSI at 30 days occurred less frequently among the high compliance compared to the low compliance cohort (4% versus 9.7%, p = 0.036). Median postoperative length of stay and 30-day rates of readmission, reoperation, intra-abdominal abscess and anastomotic leak requiring operation were not significantly different between groups. None of the individual colon bundle elements were independently protective against superficial SSI., Conclusion: Standardization of perioperative care is associated with a reduction in superficial SSI in pediatric colorectal surgery. Expansion of standardized protocols for children undergoing colorectal surgery may improve outcomes and decrease perioperative morbidity., Type of Study: Clinical Research Paper LEVEL OF EVIDENCE: Level II., Competing Interests: Declaration of Competing Interest The authors have no financial disclosures., (Copyright © 2022 Elsevier Inc. All rights reserved.)

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 of 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 and 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 (interquartile range = 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 posteriorrib 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 after 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., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

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., (© 2022 UICC.)

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., (© 2022 UICC.)

Background: Recent studies in children with idiopathic rectal prolapse report up to 48% require surgical intervention to manage refractory disease. We sought to examine outcomes of our non-surgical approach to managing rectal prolapse using a bowel management program., Methods: A retrospective review was performed for all children with the diagnosis of rectal prolapse between 2011 and 2020. Children with a rectal polyp or hemorrhoid were excluded., Results: 47 children with rectal prolapse were identified (median age at diagnosis of 4 years (IQR 3,7.75); age ≤ 4 years n = 30; age > 4 years n = 17). Associated diagnoses included constipation (n = 45, 96%) and psychiatric diagnoses (n = 7, 14%). Children underwent a bowel management program including stimulant laxatives in 44 (94%) and osmotic laxatives in 2 (4%). Median follow-up time was 181 days (IQR 77, 238). Median time to resolution of rectal prolapse was 9 months (IQR 4, 13) with a maximum time to resolution of 31 months. We compared children ≤ 4 years old (Group A) to those > 4 years old (Group B). Psychiatric diagnoses were less common in Group A (3.5 vs. 38.9%, p = 0.003). Median time to spontaneous resolution was 6.5 months (IQR 3.5, 9.5) in Group A versus 13.5 (IQR 4, 16) months in Group B, p = 0.13. No differences in surgical intervention were identified. Three (6.4%) patients required surgery for prolapse., Conclusions: A bowel management program is an effective treatment for most children with rectal prolapse. This data suggests that surgical intervention is unnecessary in most children., Level of Evidence: III., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Introduction: 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., Materials and Methods: 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., Results: 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., Conclusion: 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., Level of Evidence: III., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: 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., Methods: 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., Results: 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., Conclusions: 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., Lay Summary: 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., (© 2022 American Cancer Society.)

Background: 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., Methods: 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., Results: 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)., Conclusions: 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., Level of Evidence: Retrospective review, Level 3., (Copyright © 2022 Elsevier Inc. All rights reserved.)

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., (Copyright © 2022 Elsevier Inc. All rights reserved.)

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., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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., (Copyright © 2021. Published by Elsevier Inc.)

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 <21 years of age who underwent pneumonectomy from 1990 to 2017 for primary or metastatic tumors at 12 institutions. Clinical information was collected; outcomes included operative complications, long-term function, recurrence, and survival. Univariate log rank, and multivariable Cox analyses determined factors associated with survival., Results: Thirty-eight patients (mean 12 ± 6 yrs) were identified; median (IQR) follow-up was 19 (5-38) months. Twenty-six patients (68%) underwent pneumonectomy for primary tumors and 12 (32%) for metastases. The most frequent histologies were osteosarcoma (n = 6), inflammatory myofibroblastic tumors (IMT; n = 6), and pleuropulmonary blastoma (n = 5). Median postoperative ventilator days were 0 (0-1), intensive care 2 (1-3), and hospital 8 (5-16). Early postoperative complications occurred in 10 patients including 1 death. Of 25 (66%) patients alive at 1 year, 15 reported return to preoperative pulmonary status. All IMT patients survived while all osteosarcoma patients died during follow-up. On multivariable analysis, metastatic indications were associated with nonsurvival (HR = 3.37, P = 0.045)., Conclusion: This is the largest review of children who underwent pneumonectomy for cancer. There is decreased procedure-related morbidity and mortality than reported for adults. Survival is worse with preoperative metastatic disease, especially osteosarcoma., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Complete surgical resection of pulmonary metastatic disease in patients with osteosarcoma is crucial to long-term survival. Open thoracotomy allows palpation of nodules not identified on imaging but the impact on survival is unknown. The objective of this study was to compare overall survival (OS) and pulmonary disease-free survival (DFS) in children who underwent thoracotomy vs thoracoscopic surgery for pulmonary metastasectomy. A multi-institutional collaborative group retrospectively reviewed 202 pediatric patients with osteosarcoma who underwent pulmonary metastasectomy by thoracotomy (n = 154) or thoracoscopy (n = 48). Results were analyzed by Kaplan-Meier survival estimates and multivariate Cox proportional hazard regression models. With median follow-up of 45 months, 135 (67.5%) patients had a pulmonary relapse and 95 (47%) patients were deceased. Kaplan-Meier analysis showed no significant difference in 5-year pulmonary DFS (25% vs 38%; P = .18) or OS (49% vs 42%, P = .37) between the surgical approaches of thoracotomy and thoracoscopy. In Cox regression analysis controlling for other factors impacting outcome, there was a significantly increased risk of mortality (HR 2.11; P = .027; 95% CI 1.09-4.09) but not pulmonary recurrence (HR 0.96; P = .90; 95% CI 0.52-1.79) with a thoracoscopic approach. However, in the subset analysis limited to patients with oligometastatic disease, thoracoscopy had no increased risk of mortality (HR 1.16; P = .62; 0.64-2.11). In conclusion, patients with metastatic osteosarcoma and limited pulmonary disease burden demonstrate comparable outcomes after thoracotomy and thoracoscopy for metastasectomy. While significant selection bias in these surgical cohorts limits the generalizability of the conclusions, clinical equipoise for a randomized clinical trial in patients with oligometastatic disease is supported., (© 2020 Union for International Cancer Control.)

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., (Copyright © 2020. Published by Elsevier Inc.)

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 < .01)., 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., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2021 by the American Academy of Pediatrics.)

Background: 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., Methods: 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., Results: 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., Conclusions: Children undergoing early pouch creation have increased exposure to steroids and immune suppressants; nevertheless, no differences in adverse events were identified., Level of Evidence: II., (Copyright © 2020 Elsevier Inc. All rights reserved.)

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., (Copyright © 2020 Elsevier Inc. All rights reserved.)

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., (© 2020 Wiley Periodicals, Inc.)

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 < .05; and 58.0% vs. 88.5%, P < .05). Complications did not differ between groups (2.9 % vs 3.3%, P = 1.000), though the PCNB group had fewer blood transfusions and lower opioid usage. Efficacy of PCNB was improved for loss of heterozygosity when a pediatric pathologist evaluated the fresh specimen for adequacy., Conclusions: PCNB is a less invasive alternative to open biopsy for primary diagnosis and MYCN oncogene status in patients with neuroblastoma. Our data suggest that PCNB could be optimized for complete genetic analysis by standardized protocols and real-time pathology assessment of specimen quality., (© 2020 Wiley Periodicals, Inc.)

Introduction: Hospital readmissions have become a quality metric for both hospital systems and individual surgeons. The medical literature is replete with studies describing readmission rates and factors contributing to readmissions following surgical procedures. Relatively little, however, has been done to define potential solutions to these problems. Over the past decade there has been a movement toward the development of multidisciplinary colorectal centers at high volume children's hospitals. We hypothesized that the development of a colorectal center at our children's hospital decreased readmissions in our colorectal surgery population., Materials and Methods: A retrospective review was performed including all patients with the diagnosis of anorectal malformation (ARM) or Hirschsprung disease (HD) at our institution between the years of 2005-2017. Patient level outcomes were compared between the cohort treated prior to (2005-2010) and the cohort treated after the development of the colorectal center (2012-2017)., Results: A total of 354 patients were identified. One hundred seventy-eight patients (113 ARM, 65 HD) were treated prior to and 176 patients (110 ARM, 66 HD) were treated after the development of the colorectal center. Forty-five (25.3%) patients underwent neonatal repair prior to development of the center compared to 15 (8.5%) after. 111 (62.4%) patients underwent colostomy prior to the colorectal center comparted to 95 (54%) after. The rate of readmission within 120 days of discharge in the early group was 63% compared to 52% in those managed in the multidisciplinary colorectal center (p = 0.04). Conversely, the rate of emergency room visits increased from 8.4% to 27.3% (p = 0.01). The decrease in readmission rates was more pronounced in the ARM group, while the HD cohort had similar readmission rates before and after the establishment of the center. Multivariate logistic regression revealed an odds ratio of 0.59 (95% CI 0.37-0.92) for readmission following the development of the multidisciplinary colorectal center., Discussion: The development of a multidisciplinary colorectal center at our institution was associated with decreased hospital readmissions, but an increase in emergency department resource utilization. These findings suggest improved efficiency in patient care with the implementation of a multispecialty, patient centered approach while also identifying areas of focus for future quality improvement initiatives., Level of Evidence: Level III, retrospective comparative study., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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., (Copyright © 2018. Published by Elsevier Inc.)

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., (Copyright © 2018 Elsevier Inc. All rights reserved.)

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., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: To examine surgical outcomes of children with pancreaticoduodenal injuries at a Quaternary Level I pediatric trauma center., Methods: 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., Results: 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 = < 0.01), lower RTS2 (7.84 vs. 5.49, p < 0.01), and lower GCS (9.6 vs. 15, p = 0.03) predicted operative intervention. 6/8 (75%) undergoing surgery survived to discharge with only (2/6) survivors suffering postoperative complications. Both mortalities were secondary to severe traumatic brain injury., Conclusion: Surgical management of complex pancreaticoduodenal injury is an uncommon traumatic event that is associated with high injury severity, but survival occurs in most scenarios.

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<0.01), TBI (32.4% vs. 55.3%, 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., (Copyright © 2017 Elsevier Inc. All rights reserved.)

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.

Introduction: Congenital duodenal obstruction (DO) is frequently associated with congenital heart disease (CHD). Operative repair of DO is often postponed until an echocardiogram is completed, which may result in unnecessary delays. We aimed to identify and characterize CHD in children with DO to determine if appropriately selected patients could forego preoperative echocardiogram., Methods: A two-center retrospective review of all infants with DO undergoing operative repair with completed echocardiograms was included (2003-2011). Demographics, co-morbid conditions, clinical exam findings, radiologic imaging, and need for cardiac surgery were recorded., Results: 67 children were identified. 47 (70.1%) had CHD on echocardiogram of which 19 (40.5%) had significant CHD. Children without clinical findings, abnormalities on physical examination, and/or abnormal chest x-ray were unlikely to have CHD; i.e., no asymptomatic child had significant CHD. Sensitivity and specificity of clinical findings, physical exam, and/or chest x-ray for significant CHD were 100% (95% CI 0.79-1.0) and 37.5% (95% CI 0.24-0.53), respectively, for major CHD and 87.2% (0.74-0.95) and 60% (0.36-0.80) for any CHD., Conclusion: Careful clinical assessment, evaluation with pulse oximetry, and chest x-ray may be sufficient to exclude significant CHD in children with DO. Identifying children at low risk for cardiac lesions may prevent unnecessary delays to operative intervention and may limit medical expenses.

Purpose: The effect of timing of onset of necrotizing enterocolitis (NEC) on outcomes has not been determined for the full-term infant. In this study we aimed to characterize the full-term NEC population and to evaluate onset of NEC., Methods: We performed a two-center retrospective review of all full-term infants (≥ 37weeks) with a diagnosis of NEC between 1990 and 2012. Patients were identified by ICD-9 and age. Early onset for NEC was ≤7days and late onset after 7days of life. Demographics, comorbidities, maternal factors, clinical factors, surgical intervention, complications, and mortality were evaluated. Wilcoxon's test was performed on continuous variables and Fisher's exact test on categorical data. A p-value<0.05 was considered significant. Univariate outcomes with a p-value<0.1 were selected for multivariable analysis., Results: Thirty-nine patients (24 boys, 15 girls) with median EGA of 39weeks were identified. Overall mortality was 18%. Univariate predictors of mortality included congenital heart disease and placement of an umbilical artery (UA) catheter. Multivariate analysis revealed late onset of NEC to be an independent predictor of mortality (OR 90.8, 95% CI 2.6-3121)., Conclusion: Full-term infants who develop NEC after 7days of life, have congenital heart disease, and/or need UA catheterization have increased mortality., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Introduction: 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., Subjects and Methods: 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., Results: 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., Conclusions: The Harmonic blade scalpel can be used as a simple alternative to standard dissection techniques for thoracoscopic resection of paraspinal masses in children.

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.

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.

Objective: The aim of this study was to determine patient factors that predict diagnostic failure or increased risk of bleeding complications following percutaneous liver biopsy in children., Methods: A retrospective review of all children undergoing percutaneous liver biopsy at a single institution between July 2008 and July 2011 was performed. Demographics, comorbid conditions, preprocedural diagnoses/indications, procedural details, laboratory data, pathologic diagnosis, and complications were recorded. Continuous data were analyzed by Wilcoxon test and categorical data by Fisher exact test to determine statistical significance., Results: Two hundred thirteen children (104 girls) with a median age of 7 years (range 1 week-22 years) underwent 328 percutaneous liver biopsies. Nine (4.2%) experienced a decrease in hemoglobin >2 g/dL, 7 required transfusion (3.3%), and 1 patient died (0.5%). Younger age (1.8 vs 84 months, P = 0.05) and lower preprocedural hematocrit (29.3 vs 34.3, P = 0.05) predicted bleeding complications, whereas the number of biopsies, comorbid conditions, and coagulopathy did not. Sixty-three (19.2%) biopsies were insufficient for definitive histologic evaluation on initial biopsy in 57 patients. Twenty-one of 57 patients (37%) underwent repeat percutaneous biopsy and 3 of 57 (8%) underwent surgical biopsy. Biopsy provided definitive diagnosis in 86% of cases when repeat biopsy was performed. Shorter specimen length (1.4 vs 1.7 cm, P < 0.01) and biopsies performed for unexplained elevation of liver function tests (34.9% vs 16.7%, P < 0.01) were predictive of nondiagnosis., Conclusions: Percutaneous liver biopsy is safe with a low rate of bleeding-related complications. Ensuring adequate sample length and careful patient selection may further increase the diagnostic yield.

Background: 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., Methods: 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., Results: 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%, p < 0.0001). 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)., Conclusion: 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., Level of Evidence: Prognostic study, level III.

Background: 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., Patients and Methods: 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., Results: 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., Conclusions: 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.

Background: In adult trauma, mortality varies with race and insurance status. In the elderly, insurance type has little impact on mortality after trauma and the influence of race is reduced. How race and insurance affect pediatric trauma requires further attention. We hypothesized that mortality after pediatric trauma is influenced by insurance type and not race., Methods: We reviewed all cases of blunt trauma in children ≤13 y requiring admission, using the National Trauma Data Bank Research Data Sets for 2007 and 2008. Exclusions included an Abbreviated Injury Score of 6 for any body region, dead on arrival, and missing data. Our primary outcome measure was in-hospital mortality., Results: We identified 831 Asian (1.2%), 10,592 black (15.5%), 45,173 white (66.2%), and 8498 Hispanic (12.5%) children, and 3161 children (4.6%) classified as other race. Mean age was 7.4 ± 4.5 y, 11.9% were uninsured, and overall in-hospital mortality was 1.4%. Multivariable modeling indicated that race was not associated with increased mortality (Asian versus white, adjusted odds ratio [AOR] 1.05, P = 0.88; black versus white, AOR 0.92, P = 0.42; Hispanic versus white, AOR 0.87, P = 0.26; and other race versus white, AOR 1.01, P = 0.96). In contrast, insurance status (any insurance versus no insurance, AOR 0.6, P < 0.01) and insurance type (private insurance versus no insurance, AOR 0.47, P < 0.01; Medicaid versus no insurance, 0.67, P < 0.01) predicted reduced mortality., Conclusions: Insurance status and insurance type are important predictors of mortality after pediatric trauma while, in contrast, race is not., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: 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., Methods: 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., Results: 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)., Conclusion: 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., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: 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)., Methods: 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 value < 0.05 was considered significant. Outcomes with a P value < 0.2 were selected for multivariable analysis., Results: 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)., Conclusion: 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., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: Alcohol intoxication in pediatric trauma is underappreciated. The aim of this study was to characterize alcohol screening rates in pediatric trauma., Methods: 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., Results: 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)., Conclusions: Alcohol intoxication is common in adolescent trauma patients. Screening is encouraged for pediatric trauma patients aged ≥10 years who require admission., (Copyright © 2012 Elsevier Inc. All rights reserved.)