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Despite ongoing efforts to decrease ionizing radiation exposure from computed tomography (CT) use in pediatric appendicitis, high CT utilization rates are still observed across many hospitals. This study aims to identify factors influencing CT use and facilitators and barriers to quality improvement efforts.The Pediatric Surgery Quality Collaborative is a voluntary consortium of 42 children's hospitals participating in the National Surgical Quality Improvement Project - Pediatric. Hospitals were compared based on CT utilization from January 1, 2019, to December 31, 2019. Semi-structured interviews were conducted with surgeons, radiologists, emergency medicine physicians, and clinical data abstractors from 7 hospitals with low CT use rates (high performers) and 6 hospitals with high CT use rates (low performers). A mixed deductive and inductive coding approach for analysis of the interview transcripts was used to develop a codebook based on the Theoretical Domains Framework and subsequently identify prominent barriers and facilitators to CT reduction.Thematic saturation was achieved after 13 interviews. We identified four factors that distinguish high-performing from low-performing hospitals: (1) consistent availability of resources such as ultrasound technicians, pediatric radiologists, and magnetic resonance imaging (MRI); (2) presence of and adherence to protocols guiding imaging modality decision making and imaging execution; (3) culture of inter-departmental collaboration; and (4) presence of a radiation reduction champion.Significant barriers to reducing the use of CT in pediatric appendicitis exist. Our findings highlight that future quality improvement efforts should target resource availability, protocol adherence, collaborative culture, and radiation reduction champions.Level III.

Background/Purpose Comprehensive opioid stewardship programs require collective stakeholder alignment and proficiency. We aimed to determine opioid-related prescribing practices, knowledge, and beliefs among providers who care for pediatric surgical patients. Methods A single-center, cross-sectional survey was conducted of attending physicians, residents, and advanced practice providers (APPs), who managed pediatric surgical patients. Results Of 110 providers surveyed, 75% completed the survey. Over half of respondents (n = 43, 52%) reported always/very often prescribing opioids at discharge, with residents reporting the highest rate (66%). Provider types had varying prescribing patterns, including what types of opioids and non-opioids they prescribed. There was a lack of formal training, particularly among residents, of which only 42% reported receiving formal opioid prescribing education. Finally, although only 28% of providers felt that the opioid epidemic affects children, 48% believed pediatric providers’ prescribing patterns contributed to the opioid epidemic as a whole, and 80% reported changing their prescribing practices in response. Conclusions Significant variability exists in opioid prescribing practices, knowledge, and beliefs among providers who care for pediatric surgical patients. Effective opioid stewardship requires comprehensive policies, pediatric specific guidelines, and education for all providers caring for children to align provider proficiency and optimize prescribing patterns.

Purpose Repetitive painful stimuli and early exposure to opioids places neonates at risk for neurocognitive delays. We aimed to understand opioid utilization for neonates with gastroschisis. Methods We performed a retrospective review of infants with gastroschisis at a tertiary children's hospital (2017–2019). Multivariate linear regression was performed to analyze variations in opioid use. Results Among 30 patients with gastroschisis, 33% were managed by primary suture-less closure, 7% by primary sutured closure, 40% by spring silo, and 20% by handsewn silo. The proportion of pain medication used was: morphine (89%), acetaminophen (8%), and fentanyl (3%). Opioids were used for a median of 6.5 days (range 0–20) per patient. Median total opioid administered across all patients was 2.2 morphine milligram equivalents (MME)/kg (IQR 0.7–3.3). Following definitive closure, median opioid use was 0.2 MME/kg (IQR 0.1–0.8). With multivariate regression, 45% of the variation in MME use was associated with the type of surgery after adjusting for weight, gestational age, and gender, p = 0.02. After definitive fascial closure, there was no significant variations in opioid use. Conclusion There is a significant variation in the utilization of opioid, primarily prior to fascial closure. Understanding pain needs and standardization may improve opioid stewardship in infants with gastroschisis. 197/200 Level of Evidence Level III

Cardiac dysfunction is a key determinant of outcome in congenital diaphragmatic hernia (CDH). Pro-b-type natriuretic peptide (proBNP) is used as a prognosticator in heart failure and cardiomyopathy. We hypothesized that proBNP levels would be associated with ventricular dysfunction and high-risk disease in CDH.Patients in the CDH Study Group (CDHSG) from 2015-2019 with at least one proBNP value were included. Ventricular function was determined using echocardiograms from the first 48 h of life.A total of 2,337 patients were identified, and 212 (9%) had at least one proBNP value. Of those, 3 (1.5%) patients had CDHSG stage A defects, 58 (29.6%) B, 111 (56.6%) C, and 24 (12.2%) D. Patients with high-risk defects (Stage C/D) had higher proBNP compared with low-risk defects (Stage A/B) (14,281 vs. 5,025, p = 0.007). ProBNP was significantly elevated in patients who died (median 14,100, IQR 4,377-22,900 vs 4,911, IQR 1,883-9,810) (p0.001). Ventricular dysfunction was associated with higher proBNP than normal ventricular function (8,379 vs. 4,778, p = 0.005). No proBNP value was both sensitive and specific for ventricular dysfunction (AUC=0.61).Among CDH patients, elevated proBNP was associated with high-risk defects, ventricular dysfunction, and mortality. ProBNP shows promise as a biomarker in CDH-associated cardiac dysfunction.

Randomized controlled trials (RCTs) are challenging, but are the studies most likely to change practice and benefit patients. RCTs investigating neonatal surgical therapies are rare. The Necrotizing Enterocolitis Surgery Trial (NEST) was the first surgical RCT conducted by the Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) Neonatal Research Network (NRN), and multiple lessons were learned. NEST was conducted over a 7.25-year enrollment period and the primary outcome was death or neurodevelopmental impairment (NDI) at 18-22 months corrected age. Surgical investigators designing clinical trials involving neonatal surgical treatments have many considerations to include, including how to study eligible but non-randomized patients, heterogeneity of treatment effect, use of frequentist and Bayesian analyses, assessment of generalizability, and anticipating criticisms during peer review. Surgeons are encouraged to embrace these challenges and seek innovative methods to acquire evidence that will be used to improve patient outcomes.

There is a known association between Cornelia de Lange syndrome (CdLS) and congenital diaphragmatic hernia (CDH), with CDH being the cause of death in 5%-20% of CdLS cases. We aimed to identify and describe patients with CDLS and CDH. We hypothesized that CdLS would be associated with high-risk CDH and poor outcomes.CDH Study Group patients from 1995 to 2019 were included. Those with CdLS were reviewed retrospectively. Rates of repair and outcomes were compared between patients with and without CdLS.We identified 9,251 CDH patients. Of those, 21 had confirmed CdLS. CdLS patients had a lower birth weight (2.2±0.57 kg) than non-CdLS patients (2.9±0.64 kg) (p0.001). 5-min Apgar scores were lower in CdLS patients (6, 4-7) than non-CdLS patients (7, 5-8) (p=0.014). Only 33% of CdLS patients underwent diaphragmatic repair compared to 84.2% of non-CdLS patients (p0.001). Mortality was 76% for CdLS patients compared with 29% for non-CdLS patients (p0.001). Of the 7 CdLS patients who underwent repair, 5 survived to hospital discharge.Infants with CdLS and CDH have a poor prognosis. However, CdLS patients who undergo repair can survive to discharge; therefore, the concomitant diagnosis of CdLS and CDH is not necessarily a contraindication to repair. Early recognition of these anomalies can assist with counseling and prognostication.Retrospective comparative study LEVEL OF EVIDENCE: III.

INTRODUCTION: Biliary dyskinesia (BD) is a common indication for pediatric cholecystectomy. While diagnosis is primarily based on diminished gallbladder ejection fraction (GB-EF), work-up and management in pediatrics is controversial. METHODS: We conducted a multi-institutional retrospective review of children undergoing cholecystectomy for BD to compare perioperative work-up and outcomes. RESULTS: Six hundred seventy-eight patients across 16 institutions were included. There was no significant difference in gender, age, or BMI between institutions. Most patients were white (86.3%), non-Hispanic (79.9%), and had private insurance (55.2%). Gallbladder ejection fraction (EF) was reported in 84.5% of patients, and 44.8% had an EF < 15%. 30.7% of patients were initially seen by pediatric surgeons, 31.3% by pediatric gastroenterologists, and 23.4% by the emergency department with significant variability between institutions (p < 0.001). Symptoms persisted in 35.3% of patients post-operatively with a median follow-up of 21 days (IQR 13, 34). On multivariate analysis, only non-white race and the presence of psychiatric comorbidities were associated with increased risk of post-operative symptoms. CONCLUSION: There is significant variability in evaluation and follow-up both before and after cholecystectomy for BD. Prospective research with standardized data collection and follow-up is needed to develop and validate optimal care pathways for pediatric patients with suspected BD. STUDY TYPE: Case Series, Retrospective Review. LEVEL OF EVIDENCE: Level IV.

Evidence-based guidelines recommend ultrasound (US) over computed tomography (CT) as the primary imaging modality for suspected pediatric appendicitis. Continued high rates of CT use may result in significant unnecessary radiation exposure in children. The purpose of this study was to evaluate variables associated with preoperative CT use in pediatric appendectomy patients.A retrospective cohort study of pediatric patients who underwent appendectomy for acute appendicitis in 2015-2016 at National Surgical Quality Improvement Program for Pediatrics (NSQIP-P) hospitals was conducted. Pediatric (18 years old) patients who underwent appendectomy for acute appendicitis in an NSQIP-P hospital from 2015 to 2016 were included. Patients were excluded if they underwent interval or incidental appendectomy or did not have a final diagnosis of appendicitis. Variables associated with imaging evaluation, including age, body mass index (BMI), race/ethnicity, gender and hospital of presentation (NSQIP-P vs. non-NSQIP-P hospital) were evaluated. The primary outcome was receipt of preoperative CT. Secondary outcomes include reimaging practices and trends over time.22,333 children underwent appendectomies, of which almost all were imaged preoperatively (96.5%) and 36% of whom presented initially to a non-NSQIP-P hospital. Overall, US only was the most common imaging modality (52%), followed by CT only (27%), US+CT (16%), no imaging (3%), MRI +/- CT/US (1%) and MRI only (1%). On regression, older age (11 years), obesity (BMI95th percentile for age), and female gender were associated with increased odds of receiving a CT scan. However, initial presentation to a non-NSQIP-P hospital was the strongest predictor of CT use (OR 9.4, 95% CI 8.1-10.8). Reimaging after transfer was common, especially after US and MRI at a non-NSQIP-P hospital. CT use decreased between 2015 and 2016 in non-NSQIP-P hospitals but remained the same (25%) in NSQIP-P facilities.Though patient characteristics were associated with different imaging practices, presentation at a referral, nonchildren's hospital is the strongest predictor of CT use in children with appendicitis. NSQIP-P hospitals frequently reimage transferred patients and have not reduced their CT use. Novel strategies are required for all hospital types in order to sustain reduction in CT use and mitigate unnecessary imaging.Level III.Retrospective comparative study.

Aim To compare outcomes between prenatally and postnatally diagnosed CDH in a large multicenter database of prospectively collected data and evaluate factors associated with poorer outcome for prenatally diagnosed CDH. Material and Methods We used information from the multicenter, multinational CDH Study Group database on patients born between 2007 and 2015. We compared differences between prenatally and postnatally diagnosed CDH with respect to survival, side, size, ECMO needs, associated major cardiac malformations and liver position. Results 3746 cases of CDH were entered in the registry between 2007 and 2015, with an overall survival of 71%. Of those, 68% had a prenatal diagnosis. Survival rates were significantly better in the postnatally diagnosed group, 83 vs 65%. There was a higher proportion of bigger defect sizes, C and D, in the prenatally diagnosed group, but the survival rates were similar when patients were stratified by defect size. The rate of ECMO utilization was higher overall in the prenatally diagnosed group, 33 vs 22%, but it was similar within similar defect sizes. Right-sided defects are more commonly missed at prenatal screening than left-sided CDH, 53 vs 35% (p Conclusions Prenatally diagnosed CDH is associated with larger defect sizes compared to those with a postnatal diagnosis, and consequently have higher morbidity and mortality. Right-sided CDH are more often missed at prenatal ultrasound. The increasing rate of prenatal detection requires a clear understanding of accurate risk stratification, in order to counsel families and to provide appropriate perinatal management. Level of Evidence I for a Prognosis Study – This is a high-quality, prospective cohort study with 99% of patients followed to the study end point (death or discharge).

Contains fulltext : 202928.pdf (Publisher’s version ) (Open Access) Acetylation of the lysine residues in histones and other DNA-binding proteins plays a major role in regulation of eukaryotic gene expression. This process is controlled by histone acetyltransferases (HATs/KATs) found in multiprotein complexes that are recruited to chromatin by the scaffolding subunit transformation/transcription domain-associated protein (TRRAP). TRRAP is evolutionarily conserved and is among the top five genes intolerant to missense variation. Through an international collaboration, 17 distinct de novo or apparently de novo variants were identified in TRRAP in 24 individuals. A strong genotype-phenotype correlation was observed with two distinct clinical spectra. The first is a complex, multi-systemic syndrome associated with various malformations of the brain, heart, kidneys, and genitourinary system and characterized by a wide range of intellectual functioning; a number of affected individuals have intellectual disability (ID) and markedly impaired basic life functions. Individuals with this phenotype had missense variants clustering around the c.3127G>A p.(Ala1043Thr) variant identified in five individuals. The second spectrum manifested with autism spectrum disorder (ASD) and/or ID and epilepsy. Facial dysmorphism was seen in both groups and included upslanted palpebral fissures, epicanthus, telecanthus, a wide nasal bridge and ridge, a broad and smooth philtrum, and a thin upper lip. RNA sequencing analysis of skin fibroblasts derived from affected individuals skin fibroblasts showed significant changes in the expression of several genes implicated in neuronal function and ion transport. Thus, we describe here the clinical spectrum associated with TRRAP pathogenic missense variants, and we suggest a genotype-phenotype correlation useful for clinical evaluation of the pathogenicity of the variants.

Accurate data are essential for the validity of clinical registries. This study aimed to validate NSQIP-P data, assess representativeness, and evaluate risk-adjusted predictive ability at a single institution.A prospective appendectomy-specific pediatric surgery research database (RD) maintained by clinical researchers was compared to the NSQIP-P data for appendectomies performed in 2016 at a tertiary children's hospital. NSQIP-P sampled data collected by trained surgical clinical reviewers (SCRs) were compared to matched RD patients. Both datasets used NSQIP-P definitions. Using χ458 appendectomies for acute appendicitis were performed in 2016, of which 250 (55%) were abstracted by SCRs and matched to RD patients. Patient demographics were similar between datasets. Disease severity (NSQIP-P:50% complicated vs RD:31% complicated) and composite morbidity (NSQIP-P:6.0% vs RD:14.4%) were significantly different (both p 0.01). Demographics and outcomes were similar between matched (n = 250) and unsampled patients in the RD (n = 208). NSQIP-P's risk-adjusted predicted morbidity was significantly lower than morbidity observed in all (n = 458) RD patients (NSQIP-P:9.9% vs RD:14.2%, p 0.01).Though constituting a representative sample, NSQIP-P appendectomy data were inconsistent with department data. Discrepancies appear to be the result of underreporting of outcome variables and disease misclassification.Retrospective comparative review.Level III.

Background Opioid misuse is a public health crisis in the United States. This study aimed to evaluate the discharge opioid prescription practices for pediatric simple appendectomy patients. Methods A retrospective review of pediatric appendectomy patients at a tertiary children's hospital was conducted from October 2016 to January 2018. Only patients with simple appendicitis were included. Written opioid prescriptions were found in the electronic medical record (EMR) or through a statewide prescription monitoring database. All dosing data were converted to oral morphine equivalents (OMEs). Analysis of variance and logistic regression were used. Results During the study, 590 patients underwent appendectomy, of which 371 (62.9%) were diagnosed as having simple acute appendicitis. The majority of patients were prescribed an opioid analgesic (62.5%). Demographics were similar between those who received opioids and those who did not. The OME prescribed per day (range 0.2 to 3.4 mg/kg/day) was highly variable as was duration of prescription (1 to 30 days). Odds of emergency department visit were 3.3 times higher (95% CI 1.3–8.2) in those who received opioids. Conclusion Postdischarge prescription practices for pediatric appendectomy are highly variable. Two-thirds of patients who received narcotics had a higher rate of complications. Greater scrutiny is required to optimize opioid stewardship. Type of study Retrospective comparative study. Level of evidence Level III.

To determine in-hospital morbidities for neonates with right-sided congenital diaphragmatic hernia (R-CDH) compared with those with left-sided defects (L-CDH) and to examine the differential effect of laterality and defect size on morbidities.This retrospective, multicenter, cohort study from the international Congenital Diaphragmatic Hernia Study Group registry collected data from neonates with CDH surviving until hospital discharge from 90 neonatal intensive care units between January 1, 2007, and July 31, 2020. Major pulmonary, cardiac, neurologic, and gastrointestinal morbidities were compared between neonates with L-CDH and R-CDH, adjusted for prenatal and postnatal factors using logistic regression.Of 4123 survivors with CDH, those with R-CDH (n = 598 [15%]) compared with those with L-CDH (n = 3525 [85%]) had an increased odds of pulmonary (1.7; 95% CI, 1.4-2.2, P .0001), cardiac (1.4; 95% CI, 1.1-1.8; P = .01), gastrointestinal (1.3; 95% CI, 1.1-1.6; P = .01), and multiple (1.6; 95% CI, 1.2-2.0; P .001) in-hospital morbidities, with a greater likelihood of morbidity with increasing defect size. There was no difference in neurologic morbidities between the groups.Neonates with R-CDH and a larger defect size are at an increased risk for in-hospital morbidities. Counseling and clinical strategies should incorporate knowledge of these risks, and approach to neonatal R-CDH should be distinct from current practices targeted to L-CDH.

Background Purposeful completion (fidelity) more than simple adherence to items in the surgical safety checklist may improve operating room efficiency and patient safety. The purpose of this study was to evaluate intraoperative delays and correlate them with adherence and fidelity to the preincision surgical safety checklist. Methods Trained observers evaluated surgical safety checklist compliance during 3 observation periods from 2014–2016. Degree of adherence, checkpoint verbalization, fidelity, and meaningful completion were assessed. Delays were categorized as missing or malfunctioning equipment, staff error, and medication issues. Descriptive statistics, analysis of variance, logistic regression, χ2 and Student t test were used to analyze results. Results Of the 591 cases observed, 19% (n = 110) had at least one documented, intraoperative delay. The majority of delays were related to missing (50%) or malfunctioning (30%) equipment. Compared with cases without delays, cases with delays did not have a different mean degree of adherence (96.3 ± 7.6% vs 95.6 ± 5.8%, P = .36). Degree of fidelity was different between cases with and without delays (mean fidelity 77.1 ± 14.9% vs 80.5 ± 7.14.2%, P = .03). Conclusion The preincision SSC is a communication tool offering an opportunity to discuss potential concerns and anticipated intraoperative needs. Fidelity rather than adherence to the surgical safety checklist seems to diminish intraoperative delays.

Background Institutional protocols for preincisional antibiotic prophylaxis can standardize care and improve outcomes. However, challenges remain in compliance with such protocols for urgent or emergent operations. We hypothesized that compliance with an institutional protocol for antibiotic prophylaxis for appendectomy for appendicitis in pediatric patients results in reduced surgical site infections (SSIs) after simple appendectomy. Methods This retrospective study assessed all pediatric patients (≤18 y) who underwent appendectomy for confirmed simple appendicitis at a tertiary children's hospital between 2012 and 2015. Demographic, admission, and outcome data were recorded. Compliance with the protocol was assessed. Univariate analyses were performed to identify factors associated with any SSI and protocol noncompliance. Results Overall compliance with antibiotic prophylaxis occurred in 590 of 697 patients (85%). Compliance was high with timing (91%), spectrum (95%), and protocol-recommended drug (87%). Admission antibiotics alone were administered in 65 patients (9%), preincisional antibiotics alone in 254 patients (36%), and both in 378 patients (55%). Patients included in the analysis received a median of 2 (range 1-6) doses of antibiotics preoperatively. Ten patients (1.4%) developed an SSI. Only receipt of any antibiotics within an hour of incision was associated with decreased odds of SSI (odds ratio 0.22, 95% confidence interval 0.06-0.87). No factors were associated with noncompliance. Conclusions An institutional appendicitis protocol yields high compliance with prophylactic antibiotic administration and associated low SSI rates, but at a cost of antibiotic over-administration. Further efforts are necessary to sustain compliance while also practicing appropriate antibiotic stewardship.

Surgical site infection (SSI) rate in pediatric appendicitis is a commonly used hospital quality metric. We hypothesized that surveillance of organ-space SSI (OSI) using cultures alone would fail to capture many clinically-important events.A prospective, multidisciplinary surveillance program recorded 30-day SSI and hospital length of stay (LOS) for patients18years undergoing appendectomy for perforated appendicitis from 2012 to 2015. Standardized treatment pathways were utilized, and OSI was identified by imaging and/or bacterial cultures.Four hundred ten appendectomies for perforated appendicitis were performed, and a total of 84 OSIs (20.5%) were diagnosed with imaging. Positive cultures were obtained for 39 (46%) OSIs, whereas 45 (54%) had imaging only. Compared to the mean LOS for patients without OSI (5.2±2.9days), LOS for patients with OSI and positive cultures (13.7±5.4days) or with OSI without cultures (10.4±3.7days) was significantly longer (both p0.001). The OSI rate identified by positive cultures alone was 9.5%, whereas the clinically-relevant OSI rate was 20.5%.Using positive cultures alone to capture OSI would have identified less than half of clinically-important infections. Utilizing clinically-relevant SSI is an appropriate metric for comparing hospital quality but requires agreed upon standards for diagnosis and reporting.II.Diagnostic study.

Introduction The Accreditation Council for Graduate Medical Education mandates patient safety education without specific curricular guidelines. We hypothesized that a dedicated, adjunctive resident safety workshop (SW) led by surgical faculty compared with an online curriculum (OC) for hospital personnel alone would improve residents’ patient safety perceptions and behaviors. Materials and Methods A pilot randomized controlled trial was performed from 2014 to 2015 within a university-based general surgery residency. Control and intervention groups, stratified by postgraduate year, participated in a hospital-based OC; the intervention group participated in an additional SW. Primary outcomes were perceptions of safety culture, teamwork, and speaking up as per the validated safety attitudes questionnaire (SAQ) at 6 and 12 months postintervention. Secondary outcomes included behavioral scores from blinded surgical faculty using the Oxford NonTechnical Skills scale. Results A total of 51 residents were enrolled (control = 25, intervention = 26). SAQ response rates were 100%, 100%, and 76% at baseline, 6 months, and 12 months, respectively. SAQ scores were similar at baseline between groups and did not change significantly at 6 or 12 months, independent of postgraduate year (PGY) level. Overall NonTechnical Skills scores were similar between groups, but senior residents (≥PGY 4) in the OC + SW group scored significantly higher in teamwork, decision-making, and situation awareness (all p Conclusion An adjunctive, dedicated resident SW compared with a hospital-based OC alone did not significantly improve overall perceptions of patient safety. However, senior residents participating in the SW demonstrated improved patient safety perceptions and had significantly better intraoperative safety behaviors than senior residents in the OC group. Future curricular enhancements should include PGY-level specific education, iterative reviews, and increased faculty involvement. A larger randomized trial may be warranted.

Surgical wound classification (SWC) is widely utilized for surgical site infection (SSI) risk stratification and hospital comparisons. We previously demonstrated that nearly half of common pediatric operations are incorrectly classified in eleven hospitals. We aimed to improve multicenter, intraoperative SWC assignment through targeted quality improvement (QI) interventions.A before-and-after study from 2011-2014 at eleven children's hospitals was conducted. The SWC recorded in the hospital's intraoperative record (hospital-based SWC) was compared to the SWC assigned by a surgeon reviewer utilizing a standardized algorithm. Study centers independently performed QI interventions. Agreement between the hospital-based and surgeon SWC was analyzed with Cohen's weighted kappa and chi square.Surgeons reviewed 2034 cases from 2011 (Period 1) and 1998 cases from 2013 (Period 2). Overall SWC agreement improved from 56% to 76% (p0.01) and weighted kappa from 0.45 (95% CI 0.42-0.48) to 0.73 (95% CI 0.70-0.75). Median (range) improvement per institution was 23% (7-35%). A dose-response-like pattern was found between the number of interventions implemented and the amount of improvement in SWC agreement at each institution.Intraoperative SWC assignment significantly improved after resource-intensive, multifaceted interventions. However, inaccurate wound classification still commonly occurred. SWC used in SSI risk-stratification models for hospital comparisons should be carefully evaluated.

Appendicitis is the most common operative disease in children, yet few data exist to support specialized care for childhood appendicitis. We hypothesized that children treated with specialized care are younger, have more severe disease, and experience equal or better outcomes.Texas Blue Cross/Blue Shield claims data were reviewed for all children18 years who underwent appendectomy for acute appendicitis from 2008 to 2012. Primary outcomes included adverse events, 30-day readmissions, and hospital duration of stay. χ(2), Fisher exact test, Student t test, and multivariate regression were performed.Of the 3,886 pediatric appendectomies, 894 (23%) were performed by pediatric surgeons and 1,558 (40%) were performed in children's hospitals. Children treated by pediatric surgeons were younger, had more severe disease, underwent fewer computed tomography (CT) examinations, and were hospitalized longer (all P.05). Similarly, children treated in children's hospitals were younger, underwent fewer CTs, were more likely to undergo laparoscopic appendectomy, and were hospitalized longer (all P.05). Specialized care did not predict adverse events or 30-day readmissions; however, specialized care was associated with longer duration of stay (P.01).Privately insured children in Texas treated for appendicitis by pediatric surgeons or in children's hospitals were younger, had more severe disease, and were hospitalized longer but were less likely to undergo preoperative CT.

Background/purpose The contrast enema (CE) is commonly utilized for suspected Hirschsprung disease (HD) patients. We set out to determine the utility of the CE in the newborn for clinically suspicious HD. Methods All CEs performed for suspicion of HD in neonates from January 2004 to December 2013 were reviewed by two pediatric radiologists who were blinded to the original interpretations and final diagnoses. A standardized scoring sheet was utilized to document essential radiographic findings. Definitive diagnoses were determined by pathology. Descriptive statistics, likelihood ratios, and interrater agreement were determined. Results 158 CEs were reviewed. Interrater agreement was 89% with kappa (95% CI) of 0.63 (0.47–0.76). Common indications for CE were similar between non-HD and HD groups. The positive, inconclusive, and negative likelihood ratios (95% CI) were 38 (10–172), 3.2 (1.3–9.1), and 0.15 (0.06–0.47), respectively, leading to posttest probabilities for positive, inconclusive, and negative tests of 83%, 32%, and 2.5%, respectively. Conclusions Although radiographic positive CE for HD portends a high probability of HD, inconclusive studies still represent a significant increased risk. In clinically suspicious infants for HD, those with inconclusive studies may benefit from a lower threshold to perform follow-up rectal biopsy.

Surgical wound class (SWC) is used to risk-stratify surgical site infections (SSI) for quality reporting. We previously demonstrated only 8% agreement between hospital-based SWC and diagnosis-based SWC for acute appendicitis. We hypothesized that education and process-based interventions would improve hospital-based SWC reporting and the validity of SSI risk stratification.Patients (18 years old) who underwent appendectomies for acute appendicitis between January 2011 and December 2013 were included. Interventions entailed educational workshops regarding SWC for perioperative personnel and inclusion of SWC as a checkpoint in the surgical safety checklist. Thirty-day postoperative SSIs were recorded. Chi-square, Fisher's exact test, and kappa statistic were utilized.995 cases were reviewed (pre-intervention=478, post-intervention=517). Weighted interrater agreement between hospital-based and diagnosis-based SWC improved from 50% to 81% (p0.01), and weighted kappa increased from 0.16 (95% CI 0.004-0.03) to 0.29 (95% CI 0.25-0.34). Hospital-based dirty wounds were significantly associated with SSI in the post-intervention period only (p0.01).Agreement between hospital-based SWC and diagnosis-based SWC significantly improved after simple interventions, and SSI risk stratification became consistent with the expected increase in disease severity. Despite these improvements, there were still substantial gaps in SWC knowledge and process.

Adherence to surgical safety checklists remains challenging. Our institution demonstrated acceptable rates of checklist utilization but poor adherence to all checkpoints. We hypothesized that stepwise, multifaceted interventions would improve checklist adherence.From 2011 to 2013, adherence to the 14-point, pre-incision checklist was assessed directly by trained observers during three, 1-year periods (baseline, observation #1, and observation #2) during which interventions were implemented. Interventions included safety workshops, customization of a stakeholder-derived checklist, and implementation of a report card system. Chi-square and Kruskal-Wallis tests were utilized.Checklist performance was assessed for 873 cases (baseline, n = 144; observation #1, n = 373; observation #2, n = 356). Total checkpoint adherence increased (from 30% to 78% to 96%; P.001), as did cases with correct completion of all checkpoints (from 0% to 19% to 61%; P.001). The median (interquartile range) number of checkpoints completed per case improved from 4 (3-5) to 11 (10-12) to 14 (13-14; P.001).A strategic, multifaceted approach to perioperative safety significantly improved checklist adherence over 2 years. Checklist content and process need to reflect local interests and operative flow to achieve high adherence rates. Successful checklist implementation requires efforts to change the safety culture, stakeholder buy-in, and sustained efforts over time.

Purpose Operative repair of large abdominal wall defects in infants and children can be challenging. Component separation technique (CST) is utilized in adults to repair large abdominal wall defects but rarely used in children. The purpose of this report is to describe our experience with the CST in pediatric patients including the first description of CST use in newborns. Methods After IRB approval, we reviewed all patients who underwent CST between June 1, 2010 and December 31, 2012 at a large children's hospital. CST included dissection of abdominal wall subcutaneous tissue from the muscle and fascia and an incision of the external oblique aponeurosis one centimeter lateral to the rectus sheath. Biologic mesh onlay or underlay was used to reinforce this closure. Patients were followed for complications. Results Nine children, two patients with gastroschisis and seven with omphalocele, were repaired with CST at median (range) 1.1 years (5 days–10.1 years) of age. CST was the first surgical intervention for five children. There were minor wound complications and no recurrences after a median (range) follow up of 16 months (3–34 months). Conclusion CST can be a very useful technique to repair large abdominal wall defects in children with a loss of abdominal domain.

This study was conducted to determine the influence of age on disease presentation and evaluate the change in pediatric melanoma incidence between 1998 and 2007.We performed a retrospective review of all children ≤18 years with cutaneous melanoma who were included in the 2007 National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) database between 1988 and 2007.We identified a total of 1447 patients with cutaneous melanoma. The overall average annual melanoma incidence was 5.4 per 1 million children and adolescents in the U.S., which increased throughout the study period. Most patients (89%) were at least 10 years of age (average age 15 years). Melanoma in situ (21%), thin (1 mm) lesions (37%), stage I disease (46%), and superficial spreading histology (25%) were common at presentation. Only 1% of patients presented with distant metastases. Preadolescents younger than age 10 were ethnically more diverse and more likely to present with non-truncal primaries and advanced disease (P.01) compared to adolescents.The incidence of pediatric melanoma in the U.S. is increasing. There are significant differences between children and adolescents which suggest age-based inherent differences in the biology of the disease may exist.

Purpose The purpose of this study was to characterize the complications associated with surgical treatment of pediatric melanoma. Methods We retrospectively reviewed all pediatric patients who received surgical treatment for melanoma at our institution between 1992 and 2010. We compared complications between three groups: wide local excision only (WLE), WLE and sentinel lymph node biopsy (SLNB), and WLE and completion lymph node dissection (CLND). Results One hundred twenty-five patients were identified: 37 patients received WLE only, 47 received WLE and SLNB, and 41 patients had WLE and CLND. Complication rates differed between the three groups: 19% in WLE, 11% in WLE+SLNB, and 39% in WLE+CLND ( P =.006). The risk of complications was significantly lower among patients having WLE+SLNB versus WLE+CLND (OR 0.19, 95% CI 0.06–0.57, P =.0032). Lymphedema was a common complication with a higher incidence in the CLND group compared to the SLNB group (19.5% vs. 2.1%, P =.01). Complications were more frequent in inguinal compared to axillary dissections (52.0% vs. 17.1%, P =.006). Conclusions In the surgical treatment of pediatric melanoma, the addition of a completion lymph node dissection significantly increases complication risk. Thus, it is critical to determine which patients truly benefit from this procedure.

PURPOSE The aim of our study was to elucidate if the defect size reflects the magnitude of the developmental defect in patients with CDH. METHODS All patients recorded in the CDH Study Group registry between January 1, 2007, and December 31, 2010, and with defect classification were included in the study. They were divided according to defect size (A-D) and compared for: gestational age (GA), birth weight (BW), number of abnormal organ systems, prevalence of associated anomalies, cardiovascular malformations (CVM), chromosomal anomalies, liver in the chest, and hernia sac. RESULTS A total of 1350 of 1778 patients had defect classification: 173 A, 557 B, 438 C, and 182 D. Mortality rate was 0.6%, 5.3%, 22.6%, and 45.6% in group A, B, C, and D, respectively, (p

Purpose Chylothorax is a known complication in neonates after congenital diaphragmatic hernia (CDH) repair. This report uses a large international registry to evaluate risk factors, treatment, morbidity, and survival associated with chylothorax in a prospective cohort of neonates after CDH repair. Methods From January 2007 to January 2010, live-born neonates with repaired, unilateral CDHs were evaluated from a prospective database for chylothorax development. Chylothorax was diagnosed based on pleural fluid examination. Study variables included patient characteristics, CDH defect and disease severity characteristics, chylothorax treatment, and survival. In addition, the temporal relationship between timing of CDH repair and extracorporeal membrane oxygenation (ECMO) therapy was evaluated as a risk factor for chylothorax. Univariate and multivariate regression analyses were utilized. Results Among the 1383 patients evaluated, chylothorax was diagnosed in 4.6% of the cohort. Patch repair and ECMO were statistically significant risk factors for chylothorax. The odds of developing a chylothorax were significantly increased in patients with CDH repair on ECMO (aOR 2.6; 95% CI: 1.3–4.9) or after ECMO (aOR 3.1; 95% CI: 1.7–5.8). Most chylothoraces (83.1%) were successfully treated without surgery. Chylothorax patients had significant morbidity including increased oxygen use at 30days and longer length of stay. Survival was not significantly affected by chylothorax. Conclusions Chylothorax is a known but uncommon complication of neonatal CDH repair. In this very large series of chylothorax in association with CDH, major risk factors appear to be related to increased disease severity with the highest risk in patients repaired on or after ECMO. Chylothoraces usually improve with conservative therapy and lead to significant morbidity but not increased mortality.

To evaluate the evidence for interventions to decrease surgical site infections (SSIs) in colorectal operations using Bayesian meta-analysis.Interventions other than appropriate administration of prophylactic antibiotics to prevent SSIs have not been adopted widely, in part because of lack of recommendations for these interventions based on traditional meta-analyses. Bayesian methods can provide probabilities of specific thresholds of benefit, which may be more useful in guiding clinical decision making. We hypothesized that Bayesian meta-analytic methods would complement the interpretation of traditional analyses regarding the effectiveness of interventions to decrease SSIs.We conducted a systematic search of the Cochrane database for reviews of interventions to decrease SSIs after colorectal surgery other than prophylactic antibiotics. Traditional and Bayesian meta-analyses were performed using RevMan (Nordic Cochrane Center, Copenhagen, Denmark) and WinBUGS (MRC Biostatistics Unit, Cambridge, UK). Bayesian posterior probabilities of any benefit, defined as a relative risk of1, were calculated using skeptical, neutral, and enthusiastic prior probabilities. Probabilities were also calculated that interventions decreased SSIs by ≥10%, and ≥20% using neutral prior probability distributions.A total of 9 Cochrane reviews met the search criteria. Using traditional meta-analysis methods, only laparoscopic colorectal surgery resulted in a significant reduction in SSIs and a recommendation for use of the intervention. Using Bayesian analysis, several interventions that did not result in "significant" decreases in SSIs using traditional analytic methods had a85% probability of benefit. Also, nonuse of 2 interventions (mechanical bowel preparation and adhesive drapes) had a high probability of decreasing SSIs compared with their use.Bayesian probabilities and traditional point estimates of treatment effect yield similar information in terms of potential effectiveness. Bayesian meta-analysis, however, provides complementary information on the probability of a large magnitude of effect. The clinical impact of using Bayesian methods to inform decisions about which interventions to institute first or which interventions to combine requires further study.

Little is known about potentially modifiable risk factors associated with infectious complications (IC) acquired during extracorporeal life support (ECLS).The Extracorporeal Life Support Organization registry was accessed, and data on patient demographics, run characteristics, infections, and outcomes were collected. Patients who developed IC while on ECLS were compared to those that did not. Regression analysis was performed. Results are expressed as odds ratios, with P.05 considered significant.Infectious complications developed in 10.2% of 38,661 patients and was associated with increased odds of death. Risk factors for IC included increasing age, diagnosis, more remote decade, complications, presence of multiple complications, and ECLS mode. The risk of IC increased with the number of complications (P.001). Patients with positive cultures before ECLS also had increased odds of IC (OR 2.12, 95% CI 1.92-2.34, P.001). Those with IC were more likely to have cultures grow aggressive organisms (non-lactose fermenting gram negative rods, methicillin resistant Staphylococcus aureus, and fungi).Strategies to reduce IC while on ECLS should be aimed at prevention of complications and treatment of pre-existing infections. Future studies should address whether broader spectrum antibiotic prophylaxis and/or empiric coverage for suspected sepsis is indicated in ECLS patients.

Ewing sarcoma (ES) is the second most common bone tumor in children, and survival of those with metastatic ES has not improved. Previous studies have shown a survival benefit to whole lung irradiation in patients with pulmonary metastases and may be given either before, after, or instead of surgical pulmonary metastasectomy (PM). The contribution of surgery compared with irradiation in ES has not previously been studied.A retrospective review of patients younger than 21 years (median age, 16 years) treated at a single institution (1990-2006) was performed. Kaplan-Meier survival curves were compared using log-rank test and a multivariate Cox proportional hazards model. P ≤ .05 was regarded as significant.Eighty patients with ES were identified. Of these, 31 (39%) had pulmonary metastases. Nine patients had incomplete details of their full treatment regimen, but the following groups could be defined from the remainder: resection alone (n = 5), radiation alone (n = 3), radiation and resection (n = 3), or chemotherapy alone (n = 11). There were 24 deaths overall, with a median overall survival (OS) of 2.7 (95% confidence interval [CI], 1.7-5.2) years. Patients who had PM had the best OS (80%), whereas those who underwent radiation to the lung without PM compared with chemotherapy only for pulmonary metastasis both had similar OS of 0% at 5 years (P = .002). Patients who had radiation followed by PM for lung metastasis had a 5-year OS of 65%. Patients with PM had a longer OS compared with those without lung resection (P.0001).These data suggest a possible benefit for ES patients who undergo surgical resection of lung metastases.

Background The purpose of this study was to assess predictive factors and compliance with surgical site infection (SSI) prevention guidelines at 2 county hospitals. Design Chart review and analysis of laparotomy patients undergoing colorectal, hysterectomy, or abdominal vascular procedures over two 6-month periods 1 year apart and evaluation of safety climate using the Safety Attitudes Questionnaire (SAQ). Results Overall compliance with all antibiotic prophylaxis guidelines was 62% (n = 442). Gynecologic surgery was an independent predictor of compliance with antibiotic prophylaxis guidelines in elective cases, and nonemergency status was an independent predictor when all cases were considered. Postoperative normothermia was predicted by hospital, procedure length, initial intraoperative temperature, and service. The SAQ had a 91% response rate. Contrary to expected, safety domain scores and agreement with statements on collaboration and teamwork were not predictive of compliance. Conclusion Interventions to improve poor compliance with infection prevention guidelines must be multifaceted, hospital- and service-specific, and resilient during emergencies. Good safety and teamwork climate are not sufficient.

Congenital diaphragmatic hernia (CDH) remains a significant cause of death in newborns. With advances in neonatal critical care and ventilation strategies, survival in the term infant now exceeds 80% in some centers. Although prematurity is a significant risk factor for morbidity and mortality in most neonatal diseases, its associated risk with infants with CDH has been described poorly. We sought to determine the impact of prematurity on survival using data from the Congenital Diaphragmatic Hernia Registry (CDHR).Prospectively collected data from live-born infants with CDH were analyzed from the CDHR from January 1995 to July 2009. Preterm infants were defined as37 weeks estimated gestational age at birth. Univariate and multivariate logistic regression analysis were performed.During the study period, 5,069 infants with CDH were entered in the registry. Of the 5,022 infants with gestational age data, there were 3,895 term infants (77.6%) and 1,127 preterm infants (22.4%). Overall survival was 68.7%. A higher percentage of term infants were treated with extracorporeal membrane oxygenation (ECMO) (33% term vs 25.6% preterm). Preterm infants had a greater percentage of chromosomal abnormalities (4% term vs 8.1% preterm) and major cardiac anomalies (6.1% term vs 11.8% preterm). Also, a significantly higher percentage of term infants had repair of the hernia (86.3% term vs 69.4% preterm). Survival for infants that underwent repair was high in both groups (84.6% term vs 77.2% preterm). Survival decreased with decreasing gestational age (73.1% term vs 53.5% preterm). The odds ratio (OR) for death among preterm infants adjusted for patch repair, ECMO, chromosomal abnormalities, and major cardiac anomalies was OR 1.68 (95% confidence interval [CI], 1.34-2.11).Although outcomes for preterm infants are clearly worse than in the term infant, more than 50% of preterm infants still survived. Preterm infants with CDH remain a high-risk group. Although ECMO may be of limited value in the extremely premature infant with CDH, most preterm infants that live to undergo repair will survive. Prematurity should not be an independent factor in the treatment strategies of infants with CDH.

BACKGROUND: Severe congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) is associated with high mortality. Timing of CDH repair relative to ECMO therapy remains controversial. Our hypothesis was that survival would significantly differ between those who underwent repair during ECMO and those who underwent repair after ECMO therapy. METHODS: We examined de-identified data from the CDH Study Group (CDHSG) registry from 1995–2005 on patients who underwent repair and ECMO therapy (n=636). We used Cox regression analysis to assess differences in survival between those repaired during and after ECMO. RESULTS: Five covariates were significantly associated with mortality: timing of repair relative to ECMO (P=0.03), defect side (P=0.01), ECMO run length (P

The Congenital Diaphragmatic Hernia Registry (CDHR) was established to collect data on all infants treated at participating institutions with congenital diaphragmatic hernia (CDH) to assess therapy and improve outcome. Since 1995, the CDHR has accumulated data on over 4000 infants. The collective efforts of the CDH Study Group have provided valuable information regarding the efficacy of various therapeutic interventions and have tried to establish predictors of outcome. Because CDH is an uncommon, heterogeneous structural anomaly with a wide spectrum of severity, individual institutions may vary significantly in their experiences and treatment approaches. International multicenter registries, like the CDHR, are useful to provide clinically relevant direction by accumulating far more data on a large patient population than any single institution might.

Although survival without resection of pulmonary metastases from osteosarcoma is unlikely, not all surgeons agree on an aggressive surgical approach. We have taken an approach to attempt surgical resection if at all feasible regardless of number of metastases and disease-free interval (DFI). This study presents information on long-term follow-up after this aggressive approach to resection.A single-institution retrospective cohort study of osteosarcoma patients younger than 21 years with pulmonary metastases, limited to the contemporary chemotherapeutic period (1980-2000), was conducted.In 137 patients, synchronous (23.4%) or metachronous (76.6%) pulmonary nodules were identified. The median follow-up was 2.0 years (5 days to 20.1 years) for all patients. Overall survival among patients who had pulmonary nodules was 40.2% and 22.6% at 3 and 5 years, respectively. Ninety-nine patients underwent attempted pulmonary metastasectomy (mean survival, 33.6 months; 95% confidence interval, 25.1-42.1) and 38 patients did not (mean survival, 10.1 months; 95% confidence interval, 6.5-13.6; P.001, t test). Characteristics that were associated with an increased likelihood of 5-year overall survival after pulmonary resection were primary tumor necrosis greater than 98% after neoadjuvant chemotherapy (P.05) and DFI before developing lung metastases more than 1 year (P.001). No statistically significant difference in overall survival or disease-free survival was found based on the number of pulmonary metastases resected. Characteristics including primary tumor size, site, or extension; chemotherapy; early vs late metastases; unilateral vs bilateral metastases; and resection margins did not significantly affect survival.Most patient and tumor characteristics commonly used by surgeons to determine utility of resection of pulmonary metastases among patients with osteosarcoma are not associated with outcome. Biology of the particular tumor (response to preoperative chemotherapy, measured by tumor necrosis percentage, and DFI), as opposed to tumor burden, appears to influence survival more significantly. We would advocate considering repeat pulmonary resection for patients with recurrent metastases from osteosarcoma.

Background Late-presenting congenital diaphragmatic hernia (CDH) is a rare subset of CDH, most of the information derived from small series or case reports. The aim of this study was to document the clinical manifestations of late-presenting CDH using a large multicenter database. Methods Information about late-presenting CDH (diagnosed at later than 30 days of age) was identified from the database of the CDH Study Group (3098 cases collected during 1995-2004) and reviewed retrospectively. Results Seventy-nine cases (2.6%) from 30 centers met the inclusion criteria. Seven cases had a Morgagni hernia. There were 50 males (65%) and 27 females (35%). The mean age at diagnosis was 372 days (32 days to 15 years). Major associated anomalies (10 cardiac and 7 chromosomal abnormalities) were identified in 12 cases (15%). Presenting symptoms were respiratory in 20 (43%), gastrointestinal in 15 (33%), both in 6 (13%), and none (asymptomatic) in 5 (11%). The hernia was left-sided in 53 (69%), right-sided in 21 (27%), and central or bilateral in 3 (4%). Patients with gastrointestinal symptoms invariably had left-sided hernias (n = 19), whereas patients with respiratory symptoms (n = 24) seemed equally likely to have right- or left-sided lesions. A primary repair without patch was done in all cases with 100% survival. Conclusions Presenting symptoms of late-onset CDH can be respiratory or gastrointestinal, but presentation with gastrointestinal problems was more common in left-sided hernias, whereas respiratory symptoms predominated in right-sided lesions. The prognosis is excellent once the correct diagnosis is made.

The mortality rate associated with congenital diaphragmatic hernia (CDH) varies widely between centers and remains relatively high despite widespread use of new therapeutic modalities. Many of these have been implemented without properly controlled studies. Over the past 10 to 15 years, only 9 randomized trials enrolling a total of approximately 250 infants with CDH have been published. The limited evidence available suggests that better outcomes are observed by delivering infants with CDH at experienced centers, by delaying surgical repair until hemodynamic and respiratory stability is achieved, and by the judicious utilization of nonaggressive mechanical ventilation and permissive hypercapnea. Other therapeutic modalities, such as high frequency oscillatory ventilation, inhaled nitric oxide, and ECMO, may provide additional advantages for selected infants. There is a dire need to establish networks of centers that manage enough infants with CDH, to conduct appropriately sized randomized trials that can answer some of the critical questions about the management and long-term outcome of these infants.

Background: Surgical wound infection and intra-abdominal abscess remain common infectious complications after appendectomy, especially in the setting of a perforated or gangrenous appendix. We therefore developed a clinical protocol for the management of appendicitis to decrease postoperative infectious complications. Methods: Between January 1, 1999, and December 31, 1999, 206 patients with appendicitis were treated on protocol. Retrospectively, the charts were reviewed for all protocol patients as well as for 232 patients with appendicitis treated in the year prior to protocol initiation. Data were collected on surgical wound infections and intra-abdominal abscesses. Results: There were significantly fewer infectious complications in the protocol group than in the nonprotocol group (20 [9%] versus 8 [4%]; P

Postoperative wound infections are one of the most common nosocomial infections in surgical patients and the third most common nosocomial infection in all hospitalized patients. Surgical wound infections commonly increase the need for antibiotics and increase the length of stay and hospital costs. Although this subject has been discussed frequently in the adult literature, fewer than 10 articles exist on the subject in the pediatric patient population, despite the rate of surgical wound infection, which ranges from 3 to 20 percent. Surgical site infections are potentially preventable complications that increase hospital costs as well as patient morbidity and discomfort. Recognizing the patient who is at high risk for a surgical site infection and providing appropriate antibiotic prophylaxis to those patients is an important step in decreasing surgical site infections. This article discusses the risks of surgical site infection specific to pediatric surgical procedures, as well as appropriate antibiotic prophylaxis and treatment. Copyright © 2001 by W.B. Saunders Company

Background/Purpose: The benefit of extracorporeal membrane oxygenation (ECMO) in improving survival of neonates with congenital diaphragmatic hernia (CDH) has never been clearly demonstrated. This may be due to comparisons made between treatment groups of unequal illness severity and the low statistical power of analyses from previous studies. The authors analyzed the data from the multicenter CDH registry to determine if ECMO improves survival in CDH neonates with a high risk of mortality. Methods: A total of 730 neonates were enrolled in the CDH Registry from January 1995 to November 1997. Of these, 632 neonates had a complete data set and were eligible for ECMO by the weight criterion of greater than 2.0 kg. Multivariate logistic regression analysis was used to assess mortality risk for each neonate based on previously validated independent predictors of survival: birth weight and 5-minute Apgar. Five quintile groups were defined based on increasing predictive mortality risk. Multivariate logistic regression and X 2 analyses with birth weight, Apgar score at 5 minutes, and predictive mortality risk as covariates were then performed to assess survival benefit of ECMO compared with conventional therapy alone. Patient survival rate was defined as survival to discharge from hospital. Results: When analyzing all 632 neonates, ECMO neonates (n = 289) had a decidedly lower survival rate (52.9% v77.3%, P

Background/Purpose: The duration of postoperative cardiorespiratory monitoring of premature infants after inguinal herniorrhaphy is uncertain. Prolonged observation requiring hospital admission may be unnecessary and increases costs. Methods: This study was a retrospective review of 191 inguinal herniorrhaphies performed between 1993 and 1996 at the Hermann Children's Hospital. The authors reviewed their experience to identify factors associated with postoperative apnea and bradycardia and determine a safe period of observation. Results: Among 191 elective inguinal herniorrhaphies performed, 57 (29.8%) were in expremature infants (≤60 weeks postconception). Five (8.8%) infants either failed extubation or were unable to extubate (group 1). The average age for this group was 41.0 ± 1.2 weeks compared with 47.2 ± 1.0 ( P = .06) for those who were successfully extubated (group II). Preoperative apnea-bradycardia was found in four (80%) infants in group I compared with 32 (61.5%) in group II ( P = 0.67). All group I and 21 (40.4%, P = .09) group II infants with a history of preoperative apnea required intubation for an average of 24.4 ± 7.8 days and 8.2 ± 2.4 days, respectively ( P = .04). American Society of Anesthesia (ASA) scores were 2.6 ± 0.4 for group I compared with 1.8 ± 0.1 for group II ( P = .01). The use of both intraoperative narcotics (three [60%] in group I v six [12%] in group II, P = .01]) and vecuronium (four [80%] in group I v 16 [31%] in group II, P = .03) were significantly more common in group I infants. Operating room time was 46.4 ± 4.1 minutes for group I compared with 60.6 ± 3.9 minutes for group II ( P = .27). Postoperative apnea-bradycardia occurred in all five group I infants and two (3.8%, P = .001) group II infants. Group II infants were treated successfully with supplemental oxygen. Conclusions: All instances of postoperative apnea-bradycardia and laryngospasm occurred within 4 hours after operation without significant differences between groups. The risk of postoperative cardiorespiratory distress requiring reintubation in premature infants who undergo inguinal herniorrhaphy is not insignificant (8.8%). The judicious use of narcotics and vecuronium, and limiting patient selection to those with ASA score of less than 3 may lessen the need for reintubation. When present cardiorespiratory distress occurs early; therefore we recommend outpatient inguinal herniorrhaphy as a safe and cost-effective choice.

Background: Congenital diaphragmatic hernia (CDH) has been cited to have a mortality rate of 50%. There have been multiple studies at individual institutions demonstrating potential benefits from various strategies including extracorporeal life support (ECLS), delayed repair, and lower levels of ventilator support. There has been no multicenter survey of institutions offering these modalities to describe the current use of ECLS and survival of these infants. In addition, the relationship between the number of patients with CDH managed at an individual institution and outcome has not been evaluated. Methods: We queried 16 level III neonatal intensive care centers on the use of ECLS and survival of infants with CDH who were treated during 2 consecutive years (1993 to 1995). Data are presented as mean ± SEM, median, and range. Results: Data were collected on 411 patients. Of these, 71% ± 8% were outborn and 8% ± 3% were considered nonviable. Overall survival of CDH infants was 69% ± 4% (range, 39% to 95%). The survival rate of infants on ECLS was 55% ± 4%, whereas survival of infants not requiring ECLS was significantly increased at 81% ± 5% (p = 0.005). The mean rate of ECLS use was 46% ± 2%. There was no correlation between the number of cases per year at an individual institution and overall survival, ECLS survival, or ECLS use (r = 0.341, 0.305, and 0.287, respectively). There was also no correlation between case volume at an individual institution and ECLS survival (r = 0.271). Conclusions: The current survival rate and rate of ECLS use in infants with CDH at level III neonatal intensive care units in the United States are 69% ± 4% and 46% ± 2%, respectively. There is no correlation between the yearly individual center experience with managing CDH and rate of ECLS use or outcome. (Surgery 1998;123:305-10.)

Currently, approximately 67% of children diagnosed with cancer can be expected to survive more than 5 years. Among the most significant late effects of cancer therapy is the development of second malignant neoplasm (SMN). This study was performed to identify the factors associated with the development of second malignant neoplasms after treatment for soft tissue sarcomas in childhood. Retrospectively the charts of 20 children who developed second malignant neoplasms after treatment for primary childhood soft tissue sarcoma were reviewed. Presentation, age at diagnosis, tumor histology, extent of tumor, treatment, family histories (when available), and outcome were recorded. The mean age of the patients (10 boys, 10 girls) was 8.5 years of age (range, 1 to 20 years). Most primary tumors were rhabdomyosarcoma (14/20) and occurred in an extremity (10/20). Ninety percent of the patients (18/20) had a complete response to treatment of the primary cancer. Eleven out of 20 received combined chemotherapy and radiation therapy. The most common secondary malignancy was a bone sarcoma (6/20), followed by brain tumors (n = 3), leukemia (n = 2), and other sarcomas (n = 2). Four of the bone sarcomas developed in the field of radiation treatment. Median follow-up was 16 years (range, 1 to 26 years). The median time to development of a SMN was 11.4 years (range, 1.5 to 21 years). Survival after a second malignancy was only 30%. Two patients developed a third malignant neoplasm. The occurrence of a secondary malignancy represents a serious complication of childhood cancer. Certain tumors are related directly to treatment such as osteosarcoma within irradiated fields and secondary leukemias or lymphomas after certain chemotherapy regimens. Combined radiotherapy and chemotherapy may play an additive role in the development of second malignant neoplasms. Genetic factors may predispose affected patients to the development of both primary and secondary malignancies. Close surveillance of children previously treated for childhood cancers is warranted.

Gastric adenocarcinoma is a rare diagnosis in children. Modern treatment includes chemotherapy, radiotherapy, and surgery and has evolved over the past decade. The authors report a case of an 8-year-old girl with gastric adenocarcinoma. The case will be followed by a brief discussion of the presentation, current management guidelines, and prognosis of this rare pediatric entity.

Necrotizing enterocolitis (NEC) is a serious condition affecting predominantly the premature infant. The purpose of this study is to report a multicenter experience of complications in 252 infants requiring surgical therapy for NEC. Data from eight institutions for the years 1980 through 1990 were collected and analyzed for infants undergoing surgical therapy for NEC. Records were reviewed for gestational age, birth weight, age at operation, indications for operation, degree of intestinal involvement, operation(s) performed, complications, and 30-day mortality rates. A total of 264 infants underwent surgical intervention for NEC during the study period. Complete information was available for 252 patients. The mean gestational age was 31 +/- 5 weeks and the mean birth weight was 1,552 +/- 823 g. The mean age at operation was 18 +/- 35 days. Pneumoperitoneum was the most common indication for operation (42%). The 30-day survival rate was 72%. Eighty-one percent of patients underwent primary laparotomy, whereas peritoneal drainage was performed in 48 (19%) patients. Postoperative complications were identified in 119 (47%) patients. The most common postoperative complications were sepsis (9%), intestinal strictures (9%), and short gut (9%). Wound infections occurred in 6%, and the incidence of intraabdominal abscess formation was only 2.3%. Gestational age27 weeks (P.005) and birth weight1,000 g (P.005) were associated with significantly increased mortality but no increase in postoperative morbidity. The incidence of complications was similar in the very low birth weight (1,000 g) infants (51%) compared with infantsor = 1,000 g (46%).(ABSTRACT TRUNCATED AT 250 WORDS)