BACKGROUND:Acute appendicitis in leukopenic pediatric cancer patients is a clinical challenge. While the perceived risks of surgery in this population may delay immediate appendectomy, we sought to identify the consequences of delaying surgery based on the severity of leukopenia at diagnosis. We hypothesized that leukopenic cancer patients are more likely to experience delayed appendectomy and that delay would be associated with worsened outcomes. METHODS:Using ACS NSQIP-P data from 2016-2023, we identified pediatric oncology patients who underwent appendectomy for acute appendicitis. Leukopenic patients (< 4,000 WBC/μL) were stratified by preoperative WBC count as: < 1,000, 1,000-<2,500, and 2,500-<4,000 WBC/μL. Delayed appendectomy was defined as surgery 24 hours or more from admission. The primary outcome was the prevalence of complicated appendicitis. Bivariate comparisons were made using chi-square and nonparametric tests, with p<0.05 considered significant. RESULTS:Of 310 pediatric cancer patients, 84 (27.1%) experienced delayed appendectomy. Median time to operation was significantly longer for leukopenic patients (22.7 hours vs. 11.8 hours, p<0.001). Patients with leukopenia with delayed appendectomy were more likely to have complicated appendicitis (29.2% vs. 2.0%, p<0.001); this association was not observed in non-leukopenic patients. Within each stratum of leukopenic patients, delayed appendectomy was associated with significantly longer length of stay. CONCLUSION:Leukopenic pediatric oncology patients experienced longer delays to appendectomy for acute appendicitis than non-leukopenic patients. Among leukopenic patients, delaying appendectomy by over 24 hours was associated with an increased proportion of complicated appendicitis and prolonged hospitalization, supporting early operative management regardless of leukopenia severity.

Introduction: Intercostal nerve cryoablation reduces postoperative pain in adults undergoing thoracotomy and children undergoing pectus excavatum repair. We hypothesize that cryoablation is associated with decreased post-thoracotomy pain and opioid use in pedi-atric oncology patients.Methods: A single-center retrospective cohort study was performed for oncology patients who underwent thoracotomy from January 1, 2017 to May 31, 2021. Outcomes included postoperative opioid use measured in morphine milligram equivalents per kilogram (MME/ kg), pain scores (scale 0-10), and opioid prescription at discharge. Univariable analysis compared patients who received cryoablation to patients who did not receive cryoablation. Multivariable regression analysis controlling for age and prior thoracotomy evaluated as-sociations between cryoablation and postoperative pain.Results: Overall, 32 patients (19 males:13 females) underwent thoracotomy with 16 who underwent >1 thoracotomy resulting in 53 thoracotomies included for analysis. Cryoa-blation was used in 14 of 53 (26.4%) thoracotomies. Throughout the postoperative hospi-talization, patients receiving cryoablation during thoracotomy consumed less opioids compared to patients who did not receive cryoablation (median 0.38 MME/kg, interquartile range [IQR] 0.20-1.15 versus median 1.47 MME/kg, IQR 0.71-4.02, P < 0.01). Maximum pain scores were lower in cryoablation patients (median 6, IQR 5-8) than noncryoablation pa-tients (median 8, IQR 6-10), with a significant difference observed on postoperative day 4 (P = 0.01). Cryoablation patients were also less frequently prescribed opioids at discharge (21.4% versus 58.97%, P = 0.02). Multivariable regression demonstrated that cryoablation was associated with 2.59 MME/kg less opioid use (95% confidence interval-4.56 to-0.63) and decreased likelihood of opioid prescription at discharge (adjusted odds ratio 0.14, 95% confidence interval 0.03-0.67).Conclusions: Cryoablation is significantly associated with decreased post-thoracotomy pain and opioid use in pediatric cancer patients and should be considered in postoperative pain regimens.@ 2022 Elsevier Inc. All rights reserved.

BackgroundThoracotomy is considered the standard surgical approach for the management of pulmonary metastases in osteosarcoma (OST). Several studies have identified the advantages of a thoracoscopic approach, however, the clinical significance of thoracotomy compared to thoracoscopy is yet to be evaluated in a randomized trial.AimsThe primary aim was to determine the survival outcomes in OST patients based on surgical approach for pulmonary metastasectomy (PM) and secondary aim was to assess the post-operative morbidities of OST PM through various surgical approaches.Materials and MethodsWe conducted a single institution retrospective study to compare survival outcomes and surgical morbidity according to the surgical approach of the management of pulmonary metastases in patients with OST.ResultsSixty-one patients with OST underwent PM. Twenty-one patients were metastatic at diagnosis and underwent PM during primary treatment; nine had thoracotomy, six thoracoscopy, and six combined thoracoscopy with thoracotomy (CTT). Forty-three patients with first pulmonary relapse or progression underwent PM; 18 had thoracotomy, 16 thoracoscopy and nine CTT. There was no difference in survival between surgical approaches. There were significantly more postoperative morbidities associated with thoracotomy for initial PM (pain and postoperative chest tube placement), and for PM at first relapse (pneumothoraces, pain, Foley catheter use and prolonged hospitalizations).ConclusionOur study demonstrates that patients with OST pulmonary metastases have comparable poor outcomes despite varying surgical approaches for PM. There were significantly more postoperative morbidities associated with thoracotomy for PM. Surgical bias and other competing risks could not be assessed given the limitations of a retrospective study and may be addressed in a prospective trial evaluating surgical approach for PM in OST.

Introduction: Wilms' tumor (WT) is the most common renal malignancy in children and requires an extensive laparotomy for resection. Epidural analgesia (EA) is commonly used in postoperative pain management, but previous literature suggests it may prolong length of stay (LOS). We hypothesized that EA is associated with prolonged LOS but decreased postoperative opioid use in children undergoing WT resection.Materials and methods: A retrospective chart review was performed for all WT patients who underwent nephrectomy between January 1, 1998, and December 31, 2018, at a tertiary children's hospital. Patients with incomplete records, bilateral WT, caval or cardiac tumor extension, or intubation postoperatively were excluded. Outcomes included postoperative opioid consumption measured in oral morphine equivalents per kilogram, receipt of opioid prescription at discharge, and postoperative LOS. Mann-Whitney and multivariable regression analyses were performed.Results: Overall, 46/77 children undergoing WT resection received EA. Children with EA used significantly less inpatient opioids than children without EA (median 1.0 vs. 3.3 oral morphine equivalents per kilogram; P < 0.001). Comparing patients with EA to patients without, there was no significant difference in opioid discharge prescriptions (57% vs. 39%; P = 0.13) or postoperative LOS (median 5 d vs. 6 d; P = 0.10). Controlling for age and disease stage, EA was associated with shorter LOS by multivariable regression (coefficient-0.73, 95% confidence interval:-1.4,-0.05; P = 0.04).

Accurate diagnosis and treatment of hepatocellular neoplasm, not otherwise specified (HCN-NOS), poses significant challenges. Our study aimed to investigate the clinicopathologic and genomic similarities and differences between HCN-NOS and hepatoblastoma (HB) to guide diagnostic and treatment strategies. The clinicopathologic characteristics of 16 patients with HCN-NOS and 23 patients with HB were compared. Molecular studies, including the OncoKids DNA- and RNA-based next-generation sequencing panel, chromosomal microarray, and targeted Sanger sequencing analyses of CTNNB1 and TERT promoters, were employed. We found that patients with HCN-NOS were older (P < .001) and more frequently classified as high risk (P < .01), yet they showed no significant differences in alpha fetoprotein levels or survival outcomes compared with those with HB. HCN-NOS and HB had a comparable frequency of sequence variants, with CTNNB1 mutations being predominant in both groups. Notably, TERT promoter mutations (37.5%) and rare clinically significant variants (BRAF, NRAS, and KMT2D) were exclusive to HCN-NOS. HCN-NOS demonstrated a higher prevalence of gains in 1q, encompassing the MDM4 locus (17/17 vs 11/24; P < .001), as well as loss/loss of heterozygosity (LOH) of 1p (11/17 vs 6/24; P < .05) and chromosome 11 (7/17 vs 1/24; P < .01) when compared with HB. Furthermore, the recurrent loss/LOH of chromosomes 3, 4p, 9, 15q, and Y was only observed in HCN-NOS. However, no significant differences were noted in gains of chromosomes 2, 8, and 20, or loss/LOH of 4q and 11p between the 2 groups. Notably, no clinically significant gene fusions were detected in either group. In conclusion, our study reveals that HCN-NOS exhibits high-risk clinicopathologic features and greater structural complexity compared with HB. However, patients with HCN-NOS exhibit comparable alpha fetoprotein levels at diagnosis, CTNNB1 mutation rates, and survival outcomes when subjected to aggressive treatment, as compared with those with HB. These findings have the potential to enhance diagnostic accuracy and inform more effective treatments for HCN-NOS.

Key Points Question Is Children’s Hepatic Tumor International Collaboration–Hepatoblastoma Stratification (CHIC-HS) risk group prognostic of outcomes and are tumor histological characteristics associated with risk stratification? Findings In this cohort of 96 pediatric patients with hepatoblastoma from a single institution who were independent of the CHIC-HS discovery cohort and treated with more recent therapy, CHIC-HS group was significantly associated with both event-free survival and overall survival. Data on the contribution of hepatoblastoma histologic subtype to risk stratification were also generated. Meaning These findings validate the CHIC-HS and suggest that incorporating pretreatment tumor histological characteristics into CHIC-HS may provide additional prognostic value.

Background: Central hepatectomy (CH) is an uncommon surgical technique that is an option for resection of centrally located tumors, with the advantage of sparing normal hepatic parenchyma. Few studies have described outcomes in children undergoing CH. Materials and methods: An IRB-approved, retrospective chart review of patients who underwent CH at Children's Hospital Los Angeles between 2005 and 2016 was performed. Data included patient demographics, peri-operative factors, and post-operative outcomes. The IRB approved waiver of consent. Results: Eight patients (4F:4M) with median age of 1.9 Y underwent CH: 7 patients for HB and 1 patient for focal nodular hyperplasia. Two of the seven HB patients had metastatic disease at diagnosis. Six of the seven HB patients received a median of 4 rounds (3-7 rounds) of pre-operative chemotherapy. The median operative time was 197.5 Min (143-394 Min) with median blood loss of 175 mL (100-1200 mL). Complications included a bile fluid collection requiring aspiration. Seven patients had negative margins on pathology. One patient with a positive margin successfully completed therapy, without recurrent disease. All patients survived to follow-up, with a median follow-up duration of 1.1 Y (0.1-12.1 Y). Two patients developed recurrent disease requiring formal hepatic lobectomy and orthotopic liver transplantation. These patients had negative pathologic margins, with tumor within 1 mm of resection margins. Conclusion: CH is an effective alternative to extended hepatectomy for patients with centrally located liver tumors and is associated with good clinical and pathologic outcomes. (C) 2021 Elsevier Inc. All rights reserved.

BACKGROUND: Specialized neonatal transport teams provide efficient, critical care services to the community with decreased mortality and morbidity. During our pre-intervention period, our transport team’s 30-minute mobilization time was met in only 80% of emergent neonatal cases …

BACKGROUND:Although longer ECMO run times for patients with congenital diaphragmatic hernia (CDH) have been associated with worse outcomes, a large study has not been conducted to examine the risk factors for long ECMO runs.METHODS:The Extracorporeal Life Support Organization (ELSO) Registry from 2000 to 2015 was used to identify predictors of long ECMO runs in CDH patients. A long run was any duration of ≥14 days. Multivariable logistic regression models were used to examine the association between demographics, pre-ECMO blood gas/ventilator settings, comorbid conditions, and therapies on long ECMO runs.RESULTS:There were 4730 CDH-infants examined. The largest association with long ECMO runs was on-ECMO repair (OR: 3.72, 95% CI: 3.013-4.602, p < 0.001) and the use of THAM (OR: 1.463, 95% CI: 1.062-2.016, p = 0.02). Each drop in pH quartile was associated with an increased risk of long ECMO run: pH ≥ 7.3 (reference), pH 7.2-7.9 (OR 1.24, 95% CI: 0.98-1.57, p = 0.07), pH 7.08-7.19 (OR 1.46, 95% CI: 1.17-1.84, p = 0.001), pH ≤ 7.07 (OR 1.64, 95% CI: 1.29-2.07, p < 0.001).CONCLUSIONS:We found a correlation between both pre-ECMO demographics/timing of repair and the subsequent risk of long ECMO runs, providing insight for both providers and parents about the risk factors for longer runs.TYPE OF STUDY:Treatment Study.LEVEL OF EVIDENCE:Level III.

Background: Pelvic neuroblastomas are rare and often present in children as massive tumors whose surgical resection can be associated with significant morbidity, given sacral nerve root involvement and close proximity to pelvic vascular structures. We sought to examine the characteristics of patients with pelvic neuroblastoma and the effect of extent of surgical resection on survival and surgical outcomes. Materials and methods: After institutional review board approval, a retrospective chart review was performed at Children's Hospital Los Angeles from 2000 to 2018. Collected data included tumor location, size, image-defined risk factors histology, stage and risk classification, amplification of the oncogene MYCN or N-myc, use of preoperative chemotherapy, and extent of surgical resection. Outcome variables included postoperative complications and survival. Results: Ten patients with primary pelvic neuroblastoma tumors were identified. The median age at diagnosis was 4.2 y (3 mo to 11 y). Four patients presented with a localized pelvic tumor (stage I or stage II) and underwent upfront tumor resection. Six patients presented with advanced disease (stage III or stage IV) and underwent neoadjuvant chemotherapy, followed by partial resection (30%-90% debulked). One patient experienced a complication: lower extremity hypotonia after tumor resection. One patient died from extensive metastatic disease for which no resection was attempted. The mean postoperative follow-up was 3.9 y with 90% overall survival. Conclusions: Our data show that patients who undergo gross total resection for localized pelvic neuroblastoma or neoadjuvant chemotherapy, followed by partial resection for advanced disease have excellent survival. We recommend that small localized pelvic neuroblastoma undergo gross total resection and large unresectable tumors undergo neoadjuvant chemotherapy, followed by partial debulking resection to avoid neurovascular morbidity. (C) 2019 Elsevier Inc. All rights reserved.

Background: Management of perforated appendicitis in children remains controversial. Nonoperative (NO) and immediate operative (IO) strategies are used with varying outcomes. We hypothesized that IO intervention for patients with perforated appendicitis would be more cost-effective than NO management. Methods: A retrospective chart review of all patients with appendicitis from 2012 to 2015 was performed. Patients with perforated appendicitis were defined by evidence of perforation on imaging. We excluded patients who presented with sepsis, organ failure, and ventriculoperitoneal shunts. NO management was determined by surgeon preference. Univariate and multivariate analyses were performed. Results: IO was performed on 145 patients with perforated appendicitis, whereas 83 were treated with NO management. Compared to IO patients, NO patients incurred higher overall costs, greater length of stay, more readmissions, complications, peripherally inserted central venous catheter lines, interventional radiology drains, and unplanned clinic and emergency department visits (P < 0.0001 for all). Multivariate analysis adjusting for age, days of symptoms, admission C-reactive protein and white blood cell count revealed that NO management was independently associated with increased costs (OR 1.35, 1.12-1.62, 95% CI). Cost curves demonstrated that total cost for IO surpasses that of NO management when patients present with greater than 6.3 d of symptoms (P = 0.01). Conclusions: Our data suggest that IO is more cost-effective than NO management for patients with perforated appendicitis who present with less than 6.3 d of symptoms, after which point, NO management is more cost-effective. (C) 2019 Elsevier Inc. All rights reserved.

Purpose: Studying the timing of repair in CDH is prone to confounding factors, including variability in disease severity and management. We hypothesized that delaying repair until post-ECMO would confer a survival benefit. Methods: Neonates who underwent CDH repair were identified within the ELSO Registry. Patients were then divided into on-ECMO versus post-ECMO repair. Patients were 1:1 matched for severity based on pre-ECMO covariates using the propensity score (PS) for the timing of repair. Outcomes examined included mortality and severe neurologic injury (SNI). Results: After matching, 2,224 infants were included. On-ECMO repair was associated with greater than 3-fold higher odds of mortality (OR 3.41, 95% CI: 2.84-4.09, p<0.01). The odds of SNI was also higher for on-ECMO repair (OR 1.49, 95% CI: 1.13-1.96, p<0.01). A sensitivity analysis was performed by including the length of ECMO as an additional matching variable. On-ECMO repair was still associated with higher odds of mortality (OR 2.38, 95% CI: 1.96-2.89, p<0.01). Results for SNI were similar but were no longer statistically significant (OR 1.33, 95% CI: 0.99-1.79, p=0.06). Conclusions: Of the infants who can be liberated from ECMO and undergo CDH repair, there is a potential survival benefit for delaying CDH repair until after decannulation. Type of Study: Treatment Study (C) 2019 Elsevier Inc. All rights reserved.

Background: Traumatic injury and the presence of a central venous catheter are 2 of the strongest risk factors for venous thromboembolism in children. The purpose of this study was to determine the incidence of symptomatic, catheter-associated thrombosis in critically injured children. We hypothesized that femoral venous catheters are associated with a greater rate of thrombotic complications when compared with all other central venous access points. Methods: We reviewed a retrospective cohort (2006-2016) of injured children (<= 18 years) admitted to a pediatric intensive care unit with central access placed <= 7 days from admission. Symptomatic, catheter-associated thrombosis was determined by radiographic evidence. Poisson regression was used to compare the incidence of catheter-associated thrombosis per 1,000 catheter days between femoral and nonfemoral catheters. All comparisons were 2-tailed with alpha = 0.05. Results: We examined 209 pediatric trauma patients with central access (65% femoral, 19% subclavian, 11% arm vein, and 5% internal jugular). Femoral catheters were removed earlier (median [interquartile range] 4 [2-7] vs 8 [3-12] days, P < .001) and were larger in diameter (5 Fr [4-7] vs 4 Fr [4-4], P < .001) when compared with all other catheters. Catheter-associated thrombosis was more frequent in femoral versus nonfemoral catheters (18.4 vs 3.5 per 1,000 catheter days, P = .01). Conclusion: Femoral venous catheters are associated with a greater incidence of symptomatic, catheter-associated thrombosis in pediatric trauma patients. When central venous access is indicated for injured children, the femoral site should be avoided. If a femoral venous catheter is necessary, use of a smaller catheter should be considered. (C) 2019 Published by Elsevier Inc.

Etiologies of fetal lung anomalies include congenital pulmonary airway malformation (CPAM), intra- or extralobar pulmonary sequestration, congenital high airway obstruction syndrome (CHAOS), bronchogenic cyst, and bronchial atresia. Fetal tracheobronchoscopy has been reported both as a diagnostic and therapeutic procedure in the setting of severe congenital lung lesions. In this case report, prenatal imaging of a fetus with a large chest mass was suspicious for an obstructive bronchial lesion. The absence of visible normal lung tissue on the right side and mass effect on the left side raised the concern for pulmonary hypoplasia. After antenatal betamethasone and a period observation, hydropic changes developed. Fetal tracheobronchoscopy was then performed in an effort to identify and decompress the suspected obstructive bronchial lesion. Other than release of bronchial debris, no anatomical abnormalities were visualized. However, the right lung lesion and mediastinal shift both decreased after the fetal bronchoscopy. The newborn underwent postnatal resection of a CPAM Type II and is doing well. We hypothesize that fetal tracheobronchoscopy provided the following potential diagnostic and therapeutic benefits: (1) exclusion of an obstructive bronchial lesion; (2) disimpaction of bronchial debris from the saline lavage that we posit may have contributed to the rapid reduction in CPAM size.

Delaplain, Patrick MD; Zhang, Lishi MS; Harting, Matthew T. MD, MS, FAAP, FACS; Jancelewicz, Tim MD, MA, MS, FACS; Yu, Peter T. MD; Stein, James E. MD, MSc, FACS; Ford, Henri R. MD, MHA, FACS, FAAP, FRCSEng(Hon); Nguyen, Danh V. PhD; Guner, Yigit S. MD, FACS Author Information

Purpose: Previous studies comparing extracorporeal membrane oxygenation (ECMO) modality for congenital diaphragmatic hernia (CDH) have not accounted for confounding by indication. We therefore hypothesized that using a propensity score (PS) approach to account for selection bias may identify outcome differences based on ECMO modality for infants with CDH. Methods: We utilized ELSO Registry data (2000-2016). Patients with CDH were divided to either venoarterial (VA) or venovenous (VV) ECMO. Patients were matched by PS to control for nonrandom treatment assignment. Subgroup analyses were conducted based on timing of CDH repair relative to ECMO. Primary analysis was the Intent-to-treat" cohort based on the initial ECMO mode. Mortality was the primary outcome, and severe neurologic injury (SNI) was a secondary outcome. Results: PS matching (3:1) identified 3304 infants (VA = 2470, VV 834). In the main group, mortality was not different between VA and VV ECMO (OR = 1.01, 95% CI: 0.86-1.18) and there was no difference in SNI between VA and VV (OR = 0.80; 95% CI: 0.63-1.01). For the pre-ECMO CDH repair subgroup, 175 VA cases were matched to 70 W. In these neonates, mortality was higher for VV compared to VA (OR = 2.10, 95% CI: 1.19-3.69), without any difference in SNI (OR = 1.48; 95% CI: 0.59-3.71). For the subgroup that did not have pre-ECMO CDH repair, 2030 VA cases were matched to 683 VV cases. In this subgroup, W was associated with 27% lower risk of SNI relative to VA (OR = 0.73, 95% CI: 056-0.95) without any difference in mortality (OR = 0.94, 95% CI: 0.79-1.11). Conclusion: This study revalidates that ECMO mode does not significantly affect mortality or SNI in infants with CDH. In the subset of infants who require pre-ECMO CDH repair, VA favors survival, whereas, in the subgroup of infants that did not have pre-ECMO CDH repair, W favors lower rates of SNI. We conclude that neither mode appears consistently superior across all situations, and clinical judgment should remain a multifactorial decision. (C) 2018 Elsevier Inc. All rights reserved.

Apprehension in taking independent care of children with medical devices may lead to unnecessary visits to the ED and/or acute clinic (AC). To address these concerns, our institution implemented a gastrostomy tube (GT) class in 2011 for caretakers. We hypothesized that in-appropriate GT-related ED/AC visits would be lower in preoperatively educated caregivers. We performed a retrospective cohort study of all patients aged 0 to 18 who received GT (surgical or percutaneous) at our institution between 2006 and 2015 (n = 1340). Class attendance (trained vs untrained) and unscheduled GT-related ED/AC visits one year after GT placement were reviewed. Gastrostomy-related ED/AC visits were classified as appropriate (hospital-based intervention) or inappropriate (site care and education/reassurance). Occurrence of ED/AC visits was compared between trained and untrained cohorts. We found that 59 per cent of patients had an unscheduled GT-related ED/AC visit within one year of placement. The trained cohort had 27 per cent less unplanned ED/AC visits within one year (mean 1.21 (SD 1.82) vs untrained 1.65 (2.24), P < 0.001). On multivariate analysis, GT education independently decreased one-year GT-related health care utilization (Odds Ratio 0.75, 95% Confidence Interval 0.59-0.95). Formal education seems to decrease GT-related health care utilization within one year of placement and should be integrated into a comprehensive care plan to improve caregiver self-efficacy.

Introduction Extracorporeal membrane oxygenation (ECMO) requires a multidisciplinary healthcare team. The Extracorporeal Life Support Organization publishes training guidelines but leaves specific requirements up to each institution. Simulation training has shown promise, but it is unclear how many institutions have incorporated simulation techniques into ECMO training to date. Methods We sent an electronic survey to ECMO coordinators at Extracorporeal Life Support Organization sites in the United States. Participants were asked about training practices and the use of simulation for ECMO training. Descriptive results were reported as the percentage of total responses for each question. Logistic regression was used to identify characteristics associated with simulation use. Results Of 94 responses (62% response rate), 46% had an ECMO simulation program, whereas 26% report a program is in development. Most (61%) have been in operation for 2 to 5 years. Sixty-three percent use simulation for summative assessment, and 76% have multidisciplinary training. Access to a simulation center [odds ratio (OR) = 4.7, 95% confidence interval (CI) = 1.7–12.5], annual ECMO caseload of greater than 20 (OR = 2.5, 95% CI = 1.5–5.8), and having a pediatric cardiothoracic intensive care unit (OR = 2.8, 95% CI = 1.2–6.7) are each associated with increased likelihood of mannequin-based ECMO simulation. Common scenarios include pump failure (93%), oxygenator failure (90%), and circuit rupture (76%). Discussion Extracorporeal membrane oxygenation simulation is growing but remains in its infancy. Centers with access to a simulation center, higher caseloads, and pediatric cardiothoracic intensive care units are more likely to have ECMO simulation programs. Extracorporeal membrane oxygenation simulation is felt to be beneficial, and further work is needed to delineate best training practices for ECMO providers.