Social Determinants of Health are Associated With Postoperative Outcomes in Children With Complicated Appendicitis.

INTRODUCTION: Socioeconomic disadvantage has been associated with increased complicated appendicitis rates. Our purpose was to analyze the complex interactions between social determinants of health (SDOH) and postoperative outcomes in pediatric appendicitis. MATERIALS AND METHODS: Children who underwent appendectomy at our institution (1/2015-12/2020) were retrospectively reviewed. We used home addresses to determine composite measures of neighborhood/area-level socioeconomic advantage (Area Deprivation Index [ADI] and Social Deprivation Index [SDI]), and other area-level indicators. We created a novel, composite outcome score computed as a weighted average of eight outcome measures. Feature selection and exploratory factor analysis were used to create a multivariate model predictive of outcomes. RESULTS: Of 1117 children with appendicitis, 20.59% had complicated (perforated) appendicitis. Factor analysis identified two multivariate latent factors; Factor 1 contained SDI, ADI, and % unemployed in the population, and Factor 2 contained % Hispanic and % foreign-born in the population. Low Factor 2 scores (communities with more Hispanic/foreign-born residents) were associated with increased length of stay, more frequent postoperative percutaneous drainage, and increased postoperative imaging. CONCLUSIONS: Interactions between SDOH and pediatric surgical care go beyond the individual patient and suggest that vulnerable populations are exposed to contextual conditions that may impact outcomes. Specifically, neighborhood-level factors, including the prevalence of Hispanic ethnicity and foreign-born individuals, are associated with outcomes in pediatric patients with complicated appendicitis. Reducing disparities in complicated appendicitis outcomes may involve addressing neighborhood-level SDOH through strategic reallocation of healthcare resources and developing targeted interventions to improve access to pediatric surgical care in underserved communities.

A Pilot Randomized Controlled Trial of Outcomes Associated with Parent-Nurse Controlled Analgesia vs. Continuous Opioid Infusion in the Neonatal Intensive Care Unit.

PURPOSE: Prospectively compare parent/nurse controlled analgesia (PNCA) to continuous opioid infusion (COI) in the post-operative neonatal intensive care unit (NICU) population. DESIGN/METHODS: A randomized controlled trial compared neonates treated with morphine PNCA to those treated with morphine COI. The primary outcome was average opioid consumption up to 3 post-operative days. Secondary outcomes included 1) pain intensity, 2) adverse events that may be directly related to opioid consumption, and 3) parent and nurse satisfaction. RESULTS: The sample consisted of 25 post-operative neonates and young infants randomized to either morphine PNCA (n = 16) or COI (n = 9). Groups differed significantly on daily opioid consumption, with the PNCA group receiving significantly less opioid (P = .02). Groups did not differ on average pain score or frequency of adverse events (P values > .05). Parents in both groups were satisfied with their infant's pain management and parents in the PNCA group were slightly more satisfied with their level of involvement (P = .03). Groups did not differ in nursing satisfaction. CONCLUSIONS: PNCA may be an effective alternative to COI for pain management in the NICU population. This method may also substantially reduce opioid consumption, provide more individualized care, and improve parent satisfaction with their level of participation. CLINICAL IMPLICATIONS: Patients in the NICU represent one of our most vulnerable patient populations. As nurses strive to provide safe and effective pain management, results of this study suggest PNCA may allow nurses to maintain their patients' comfort while providing less opioid and potentially improving parental perception of involvement. STUDY TYPE: Treatment study. LEVEL OF EVIDENCE: I.

Diagnosis and treatment of gastric antral webs in pediatric patients.

BACKGROUND: Gastric antral webs are mucosal structures, varying from fenestrated diaphragms to mucosal crescents, resulting in varying degrees of foregut obstruction. Patients commonly present with vomiting, failure to thrive, and abdominal pain. Prevalence is unknown, and diagnosis can be difficult. METHODS: We performed an IRB-approved retrospective review of patients from 4/1/2015-4/1/2018 at a Level I Children's Surgery Center undergoing gastric antral web resection. Data obtained included demographics, preoperative workup, surgical repair, and outcomes. RESULTS: Twenty-one patients were identified; 67% were male with an average age of 30 months at diagnosis. Initial diagnosis was established by a combination of fluoroscopy and esophagogastroduodenoscopy (EGD) in all patients. Patients presented with emesis (76%), failure to thrive (57%), need for post-pyloric tube feeds (33%), and abdominal pain (14%). Web localization without intraoperative EGD (n = 3) was initially challenging. As a result, intraoperative EGD was combined with operative antral web resection to facilitate web localization (n = 18). Web marking techniques have evolved from marking with suture (n = 1) and tattoo (n = 2), to endoscopic clip application (n = 12). All 21 patients underwent web resection, 2 were performed laparoscopically. Twenty underwent Heineke-Mikulicz pyloroplasty during the initial surgery. Average length of stay was 5.5 days. There were no intraoperative complications or deaths. Permanent symptom resolution occurred in 90% of patients immediately, with a statistically significant decrease in emesis (p < 0.001), failure to thrive (p < 0.001), and need for post-pyloric tube feeding (p = 0.009) within 6 months of surgery. CONCLUSION: Gastric antral webs should be considered in the differential diagnosis for a child with persistent vomiting. Web resection with the use of intraoperative endoscopic localization can result in permanent symptom resolution in the majority of these patients.

Criteria for Critical Care Infants and Children: PICU Admission, Discharge, and Triage Practice Statement and Levels of Care Guidance.

OBJECTIVES: To update the American Academy of Pediatrics and Society of Critical Care Medicine's 2004 Guidelines and levels of care for PICU. DESIGN: A task force was appointed by the American College of Critical Care Medicine to follow a standardized and systematic review of the literature using an evidence-based approach. The 2004 Admission, Discharge and Triage Guidelines served as the starting point, and searches in Medline (Ovid), Embase (Ovid), and PubMed resulted in 329 articles published from 2004 to 2016. Only 21 pediatric studies evaluating outcomes related to pediatric level of care, specialized PICU, patient volume, or personnel. Of these, 13 studies were large retrospective registry data analyses, six small single-center studies, and two multicenter survey analyses. Limited high-quality evidence was found, and therefore, a modified Delphi process was used. Liaisons from the American Academy of Pediatrics were included in the panel representing critical care, surgical, and hospital medicine expertise for the development of this practice guidance. The title was amended to "practice statement" and "guidance" because Grading of Recommendations, Assessment, Development, and Evaluation methodology was not possible in this administrative work and to align with requirements put forth by the American Academy of Pediatrics. METHODS: The panel consisted of two groups: a voting group and a writing group. The panel used an iterative collaborative approach to formulate statements on the basis of the literature review and common practice of the pediatric critical care bedside experts and administrators on the task force. Statements were then formulated and presented via an online anonymous voting tool to a voting group using a three-cycle interactive forecasting Delphi method. With each cycle of voting, statements were refined on the basis of votes received and on comments. Voting was conducted between the months of January 2017 and March 2017. The consensus was deemed achieved once 80% or higher scores from the voting group were recorded on any given statement or where there was consensus upon review of comments provided by voters. The Voting Panel was required to vote in all three forecasting events for the final evaluation of the data and inclusion in this work. The writing panel developed admission recommendations by level of care on the basis of voting results. RESULTS: The panel voted on 30 statements, five of which were multicomponent statements addressing characteristics specific to PICU level of care including team structure, technology, education and training, academic pursuits, and indications for transfer to tertiary or quaternary PICU. Of the remaining 25 statements, 17 reached consensus cutoff score. Following a review of the Delphi results and consensus, the recommendations were written. CONCLUSIONS: This practice statement and level of care guidance manuscript addresses important specifications for each PICU level of care, including the team structure and resources, technology and equipment, education and training, quality metrics, admission and discharge criteria, and indications for transfer to a higher level of care. The sparse high-quality evidence led the panel to use a modified Delphi process to seek expert opinion to develop consensus-based recommendations where gaps in the evidence exist. Despite this limitation, the members of the Task Force believe that these recommendations will provide guidance to practitioners in making informed decisions regarding pediatric admission or transfer to the appropriate level of care to achieve best outcomes.

Long-term Quality of Life in Neonatal Surgical Disease.

OBJECTIVE: This prospective observational study was designed to assess Pediatric Quality of Life (PedsQL) after surgical treatment for congenital diaphragmatic hernia (CDH), esophageal atresia/tracheoesophageal fistula (EA/TEF), Hirschsprung disease (HD), gastroschisis (GAS), omphalocele (OMP), and necrotizing enterocolitis (NEC). SUMMARY OF BACKGROUND DATA: Improvements in neonatal and surgical care have led to increased survival for many newborn conditions. Quality of life in these patients is seldom explored in a longitudinal manner. We hypothesized that age-adjusted physical and psychosocial scores would improve over time, but with diagnosis-dependent variation. METHODS: Data were collected from 241 patients (CDH = 52; EA/TEF = 62; HD = 46; GAS = 32; OMP = 26; NEC = 23) in an institutional Clinical Outcomes Registry (COR) from 2012 to 2017. Aggregate physical, psychosocial, and overall PedsQL scores were determined for each diagnosis. Spline regression models were created to model scores as a function of age. RESULTS: Physical scores trended up for all diagnoses except CDH and NEC beyond age 10. Psychosocial scores trended up for all diagnoses except NEC and EA/TEF beyond age 10. Beyond age 12, CDH, GAS, and HD patients had overall scores within the normal range, while NEC, OMP, and EA/TEF patients had scores similar to children with chronic medical illness. CONCLUSION: Variation exists in long-term PedsQL scores after neonatal surgery for selected, complex disease. Beyond age 12, quality of life is significantly impaired in NEC, moderately impaired in OMP and EA/TEF, and within normal range for CDH, HD, and GAS patients at the population level. These data are relevant to prenatal and perioperative discussions with patients and families.

Case Volume and Revisits in Children Undergoing Gastrostomy Tube Placement.

OBJECTIVES: Emergency department (ED) visits and hospital readmissions are common after gastrostomy tube (GT) placement in children. We sought to characterize interhospital variation in revisit rates and explore the association between this outcome and hospital-specific GT case volume. PATIENTS AND METHODS: We conducted a retrospective cohort study from 38 hospitals using the Pediatric Health Information System database. Patients younger than 18 years who had a GT placed in 2010 to 2012 were assessed for a GT-related (mechanical or infectious) ED visit or inpatient readmission at 30 and 90 days after discharge from GT placement. Risk-adjusted rates were calculated using generalized linear mixed-effects models accounting for hospital clustering and relevant demographic and clinical attributes, then compared across hospitals. RESULTS: A total of 15,642 patients were included. A median of 468 GTs were placed in all the 38 hospitals during 3 years (range: 83-891), with a median of 11.4 GT placed per 1000 discharges (range: 2.4-16.7). Median ED visit for each hospital at 30 days after discharge was 8.2% (range: 3.7%-17.2%) and 14.8% at 90 days (range: 6.3%-26.1%). Median inpatient readmissions for each hospital at 30 days after discharge was 3.5% (range: 0.5%-10.5%) and 5.9% at 90 days (range: 1.0%-18.5%). Hospital-specific GT placement per 1000 discharges (rate of GT placement) was inversely correlated with ED visit rates at 30 (P = 0.007) and 90 days (P = 0.020). The adjusted 30- and 90-day readmission rate and the adjusted 30- and 90-day ED return rates decreased with increasing GT insertion rate (P < 0.001). CONCLUSION: Higher hospital GT insertion rates are associated with lower ED revisit rates but not inpatient readmissions.

Emergency Department Visits and Readmissions among Children after Gastrostomy Tube Placement.

OBJECTIVES: To define the incidence of 30-day postdischarge emergency department (ED) visits and hospital readmissions following pediatric gastrostomy tube (GT) placement across all procedural services (Surgery, Interventional-Radiology, Gastroenterology) in 38 freestanding Children's Hospitals. STUDY DESIGN: This retrospective cohort study evaluated patients <18 years of age discharged between 2010 and 2012 after GT placement. Factors significantly associated with ED revisits and hospital readmissions within 30 days of hospital discharge were identified using multivariable logistic regression. A subgroup analysis was performed comparing patients having the GT placed on the date of admission or later in the hospital course. RESULTS: Of 15 642 identified patients, 8.6% had an ED visit within 30 days of hospital discharge, and 3.9% were readmitted through the ED with a GT-related issue. GT-related events associated with these visits included infection (27%), mechanical complication (22%), and replacement (19%). In multivariable analysis, Hispanic ethnicity, non-Hispanic black race, and the presence of ≥3 chronic conditions were independently associated with ED revisits; gastroesophageal reflux and not having a concomitant fundoplication at time of GT placement were independently associated with hospital readmission. Timing of GT placement (scheduled vs late) was not associated with either ED revisits or hospital readmission. CONCLUSIONS: GT placement is associated with high rates of ED revisits and hospital readmissions in the first 30 days after hospital discharge. The association of nonmodifiable risk factors such as race/ethnicity and medical complexity is an initial step toward understanding this population so that interventions can be developed to decrease these potentially preventable occurrences given their importance among accountable care organizations.

Is there an alternative to continuous opioid infusion for neonatal pain control? A preliminary report of parent/nurse-controlled analgesia in the neonatal intensive care unit.

BACKGROUND: Continuous opioid infusion (COI) remains the mainstay of analgesic therapy in the neonatal intensive care unit (NICU). Parent/nurse-controlled analgesia (PNCA) has been accepted as safe and effective for pediatric patients, but few reports include use in neonates. This study sought to compare outcomes of PNCA and COI in postsurgical neonates and young infants. METHODS: Twenty infants treated with morphine PNCA were retrospectively compared with 13 infants treated with fentanyl COI in a Midwestern pediatric hospital in the United States. Outcome measures included opioid consumption, pain scores, frequency of adverse events, and subsequent methadone use. RESULTS: The PNCA group (median 6.4 µg · kg(-1) · h(-1) morphine equivalents, range 0.0-31.4) received significantly less opioid (P < 0.001) than the COI group (median 40.0 µg · kg(-1) · h(-1) morphine equivalents; range 20.0-153.3), across postoperative days 0-3. Average daily pain scores (based on 0-10 scale) were low for both groups, but median scores differed nonetheless (0.8 PNCA vs 0.3 COI, P < 0.05). There was no significant difference in the frequency of adverse events or methadone use. CONCLUSION: Results suggest PNCA may be a feasible and effective alternative to COI for pain management in postsurgical infants in the NICU. Results also suggest PNCA may provide more individualized care for this vulnerable population and in doing so, may potentially reduce opioid consumption; however, more studies are needed.

Negative pressure wound therapy in infants and children: a single-institution experience.

BACKGROUND: Information regarding the use of negative pressure wound therapy (NPWT) in the pediatric population is limited. Because of adverse outcomes in adult patients, the Food and Drug Administration issued a warning in 2011 about the use of NPWT in infants and children. METHODS: We performed an institutional review board-approved, single-institution, retrospective review of pediatric patients who had undergone NPWT from 2007-2011. We collected the types of wounds for which NPWT was initiated, the NPWT outcomes, and the complications encountered. RESULTS: The data from 290 consecutive patients were reviewed. Their average age was 9.3 y (range 12 d to 18 y), and their average weight was 46.5 kg (range 1.1-177). Of the wounds, 66% were classified as acute, 10% as chronic, and 24% as traumatic. The two most common indications were surgical wound dehiscence (n = 47) and skin grafting (n = 41). NPWT was used in 15 wounds containing surgical hardware, with 2 devices requiring eventual removal. NPWT was used for a median of 9 d per patient (two dressing changes). Complications occurred in 5 patients (1.7%). Documentation problems were noted in 44 patients. After NPWT, about one-third of the patients (n = 95 patients) were able to undergo delayed primary closure. CONCLUSIONS: NPWT is an effective adjunct in wound healing and closure in the pediatric population, with no mortality ascribed to NPWT. Also, the complication rates were low.

Bronchogenic cyst in the intradiaphragmatic location.

Bronchogenic cysts are congenital foregut malformations thought to develop due to abnormal budding of tracheal diverticulumand proximal bronchial structures during embryologic development. The cyst is lined by ciliated pseudostratified columnar epithelium and the wall contains cartilage and layers of smooth muscle. These lesions most commonly are seen in the mediastinum, lung, or pleural spaces. The intradiaphragmatic location of the bronchogenic cyst rarely has been reported in the literature. We report the clinical presentation and computed tomography and magnetic resonance imaging findings in a pediatric patient who presented with left-sided chest pain and was found to have a mass in the region of the diaphragm.

Sustaining the gains: Reducing unnecessary computed tomography scans in pediatric trauma patients.

BACKGROUND/PURPOSE: "Pan-scanning" pediatric blunt trauma patients leads to exposure to harmful radiation and increased healthcare costs without improving outcomes. We aimed to reduce computed tomography (CT) scans that are not indicated (NI) by imaging guidelines for injured children. METHODS: In July 2017, our Pediatric Trauma Center prospectively implemented validated imaging guidelines to direct CT imaging for trauma activations and consultations for children younger than 16 years old with blunt traumatic injuries. Patients with suspected physical abuse, CT imaging prior to arrival, penetrating mechanism, and instability precluding CT imaging were excluded. We compared CT scanning rates for pre-implementation (01/2016-06/2017) and post-implementation (07/2017-08/2021) time periods. Guideline compliance was evaluated by chart review and sustained through iterative process improvement cycles. RESULTS: During the pre-implementation era, 61 patients underwent 171 CT scans of which 87 (51%) scans were not indicated by guidelines. Post-implementation, 363 patients had 531 scans and only 134 (25%) CTs were not indicated. Total CTs performed declined after initiation of guidelines (2.80 vs 1.46 scans/patient, p<0.0001). Total NI CTs declined (1.41 vs 0.37 NI scans/patient, p<0.0001) reflected in significant reductions in all anatomic regions: head, cervical spine, chest, and abdomen/pelvis. Charges related to NI scans decreased from $1,490.31/patient to $408.21/patient, saving $218,000 in charges. Based on prior utilization, 146 children were spared excessive radiation with no clinically significant missed injuries since guideline implementation. CONCLUSIONS: Quality improvement and implementation science methodologies to enhance compliance with imaging guidelines for children with blunt injuries can significantly reduce unnecessary CT scanning without compromising care. This practice reduces harmful radiation exposure in a sensitive patient population and may save healthcare systems money and resources.

Value Added from Same-day Discharge after Appendectomy for Children with Simple Appendicitis.

Same-day discharge of children after appendectomy for simple appendicitis is safe and associated with enhanced parent satisfaction. Our general pediatric surgeons aimed to improve the rate of same-day discharge after appendectomy for simple appendicitis. Methods: We implemented a clinical practice guideline in September 2019. A surgeon-of-the-week service model and the urgent operating room started in November 2019 and January 2020, respectively. Data for children with simple appendicitis from our academic medical center were gathered prospectively using National Surgical Quality Improvement Program-Pediatric. Patient outcomes before intervention implementation (n = 278) were compared with patients following implementation (n = 264). Results: The average monthly percentage of patients discharged on the day of surgery increased in the postimplementation group (32% versus 75%). Median postoperative length of stay decreased [16.5 hours (interquartile range, 15.9) versus 4.4 hours (interquartile range, 11.7), P < 0.001], and the proportion of patients discharged directly from the postoperative anesthesia care unit increased (22.8% versus 43.6%; P < 0.001). There were no differences in balancing measures, including the return to the emergency department and readmission. Fewer children were discharged home on oral antibiotics after implementation (6.8% versus 1.5%, P = 0.002), and opioid prescribing at discharge remained low (2.5% versus 1.1%, P = 0.385). Conclusions: Using quality improvement methodology and care standardization, we significantly improved the rate of same-day discharge after appendectomy for simple appendicitis without impacting emergency department visits or readmissions. As a result, our health care system saved 140 hospital days over the first 21 months.

Prehospital factors predict outcomes in pediatric trauma: A principal component analysis.

BACKGROUND: Trauma team activation leveling decisions are complex and based on many variables. Accurate triage decisions improve patient safety and resource utilization. Our purpose was to establish proof-of-concept for using principal component analysis (PCA) to identify multivariate predictors of injury severity and to assess their ability to predict outcomes in pediatric trauma patients. We hypothesized that we could identify significant principal components (PCs) among variables used for decisions regarding trauma team activation and that PC scores would be predictive of outcomes in pediatric trauma. METHODS: We conducted a retrospective review of the trauma registry (January 2014 to December 2020) at our pediatric trauma center, including all pediatric patients (age <18 years) who triggered a trauma team activation. Data included patient demographics, prehospital report, Injury Severity Score, and outcomes. Four significant principal components were identified using PCA. Differences in outcome variables between the highest and lowest quartile for PC score were examined. RESULTS: There were 1,090 pediatric patients included. The four significant PCs accounted for greater than 96% of the overall data variance. The first PC was a composite of prehospital Glasgow Coma Scale and Revised Trauma Score and was predictive of outcomes, including injury severity, length of stay, and mortality. The second PC was characterized primarily by prehospital systolic blood pressure and high PC scores were associated with increased length of stay. The third and fourth PCs were characterized by patient age and by prehospital Revised Trauma Score and systolic blood pressure, respectively. CONCLUSION: We demonstrate that, using information available at the time of trauma team activation, PCA can be used to identify key predictors of patient outcome. While the ultimate goal is to create a machine learning-based predictive tool to support and improve clinical decision making, this study serves as a crucial step toward developing a deep understanding of the features of the model and their behavior with actual clinical data. LEVEL OF EVIDENCE: Diagnostic Test or Criteria; Level III.

Reduction of surgical site infections in pediatric patients with complicated appendicitis: Utilization of antibiotic stewardship principles and quality improvement methodology.

BACKGROUND: The rate of surgical site infection (SSI) after appendectomy for complicated appendicitis (CA) was high at our children's hospital. We hypothesized that practice standardization, including obtaining intra-operative cultures of abdominal fluid in patients with CA, would improve outcomes and reduce healthcare utilization after appendectomy. METHODS: A quality improvement team designed and implemented a clinical practice guideline for CA that included obtaining intra-operative culture of purulent fluid, administering piperacillin/tazobactam for at least 72 h post-operatively, and transitioning to oral antibiotics based on intraoperative culture data. We compared outcomes before and after guideline implementation. RESULTS: From July 2018-October 2019, 63 children underwent appendectomy for CA compared to 41 children from January-December 2020. Compliance with our process measures are as follows: Intra-operative culture was obtained in 98% of patients post-implementation; 95% received at least 72 h of piperacillin-tazobactam; and culture results were checked on all patients. Culture results altered the choice of discharge antibiotics in 12 (29%) of patients. All-cause morbidity (SSI, emergency department visit, readmission to hospital, percutaneous drain, unplanned return to operating room) decreased significantly from 35% to 15% (p=0.02). Surgical site infections became less frequent, occurring on average every 27 days pre-implementation and every 60 days after care pathway implementation (p=0.03). CONCLUSIONS: Utilization of a clinical practice guideline was associated with reduced morbidity after appendectomy for CA. Intra-operative fluid culture during appendectomy for CA appears to facilitate the selection of appropriate post-operative antibiotics and, thus, minimize SSIs and overall morbidity.

Pediatric surgery milestones 2.0: A primer.

More than twenty years ago, the Accreditation Council for Graduate Medical Education and the American Board of Medical Specialties began the conversion of graduate medical education from a structure- and process-based model to a competency-based framework. The educational outcomes assessment tool, known as the Milestones, was introduced in 2013 for seven specialties and by 2015 for the remaining specialties, including pediatric surgery. Designed to be an iterative process with improvements over time based on feedback and evidence-based literature, the Milestones started the evolution from 1.0 to 2.0 in 2016. The formation of Pediatric Surgery Milestones 2.0 began in 2019 and was finalized in 2021 for implementation in the 2022-2023 academic year. Milestones 2.0 are fewer in number and are stated in more straightforward language. It incorporated the harmonized milestones, subcompetencies for non-patient care and non-medical knowledge that are consistent across all medical and surgical specialties. There is a new Supplemental Guide that lists examples, references and links to other assessment tools and resources for each subcompetency. Milestones 2.0 represents a continuous process of feedback, literature review and revision with goals of improving patient care and maintaining public trust in graduate medical education's ability to self-regulate. LEVEL OF EVIDENCE: V.

Making a definitive diagnosis: successful clinical application of whole exome sequencing in a child with intractable inflammatory bowel disease.

PURPOSE: We report a male child who presented at 15 months with perianal abscesses and proctitis, progressing to transmural pancolitis with colocutaneous fistulae, consistent with a Crohn disease-like illness. The age and severity of the presentation suggested an underlying immune defect; however, despite comprehensive clinical evaluation, we were unable to arrive at a definitive diagnosis, thereby restricting clinical management. METHODS: We sought to identify the causative mutation(s) through exome sequencing to provide the necessary additional information required for clinical management. RESULTS: After sequencing, we identified 16,124 variants. Subsequent analysis identified a novel, hemizygous missense mutation in the X-linked inhibitor of apoptosis gene, substituting a tyrosine for a highly conserved and functionally important cysteine. X-linked inhibitor of apoptosis was not previously associated with Crohn disease but has a central role in the proinflammatory response and bacterial sensing through the NOD signaling pathway. The mutation was confirmed by Sanger sequencing in a licensed clinical laboratory. Functional assays demonstrated an increased susceptibility to activation-induced cell death and defective responsiveness to NOD2 ligands, consistent with loss of normal X-linked inhibitor of apoptosis protein function in apoptosis and NOD2 signaling. CONCLUSIONS: Based on this medical history, genetic and functional data, the child was diagnosed as having an X-linked inhibitor of apoptosis deficiency. Based on this finding, an allogeneic hematopoietic progenitor cell transplant was performed to prevent the development of life-threatening hemophagocytic lymphohistiocytosis, in concordance with the recommended treatment for X-linked inhibitor of apoptosis deficiency. At >42 days posttransplant, the child was able to eat and drink, and there has been no recurrence of gastrointestinal disease, suggesting this mutation also drove the gastrointestinal disease. This report describes the identification of a novel cause of inflammatory bowel disease. Equally importantly, it demonstrates the power of exome sequencing to render a molecular diagnosis in an individual patient in the setting of a novel disease, after all standard diagnoses were exhausted, and illustrates how this technology can be used in a clinical setting.

Left congenital diaphragmatic hernia and gastroschisis in a term male infant.

A term male infant was born to a healthy 24-year-old mother with antenatally diagnosed liver-up, left congenital diaphragmatic hernia (CDH) and gastroschisis. The infant was stabilised in the neonatal intensive care unit and then underwent primary repair of the CDH via left subcostal incision and silo placement for the gastroschisis. Serial silo reductions were started postoperatively and umbilical flap closure for the gastroschisis was performed on day of life 6. The patient was weaned from respiratory support, started on enteral feeds, and discharged home at 1 month of age. He was weaned from supplemental nasogastric feeds by 6 weeks of age and is currently well and thriving at 11 months of age.

The APSA Board of Governors enthusiastically endorses the position paper ``Diversity, Equity, and Inclusion: A Strategic Priority for the American Pediatric Surgical Association''.

This Commentary by the APSA Board of Governors enthusiastically endorses the position paper "Diversity, Equity, and Inclusion: A Strategic Priority for the American Pediatric Surgical Association".

Splitting hairs and challenging guidelines: Defining the role of perioperative antibiotics in pediatric appendicitis patients.

BACKGROUND: Ambiguity regarding timing and duration of perioperative IV antibiotics in AA and CA exists. We wanted to determine if an association existed between surgical site infections (SSI) in children with acute (AA) or complicated appendicitis (CA) and timing and duration of perioperative antibiotics. METHODS: We performed a single institution, case control observational study of patients with (n = 988) and CA (n = 561) from 2013 to 2017. The exposure was the timing and/or duration of pre- and postoperative antibiotics. The outcome measure was SSI development within 60 days of surgery. RESULTS: SSI occurred in 2.5% AA and 19.1% CA patients. We identified 18-70 min before incision (MBI) as the best interval for preoperative antibiotic administration with regards to SSI occurrence with SSI OR = 3.0 (95% CI 1.35, 6.68) p = 0.0356 for antibiotics given 0-17 MBI and OR = 3.21 (95% CI 1.45, 7.09) p = 0.0108) for antibiotics given >70 MBI. Postoperative antibiotics did not confer protection from SSI in AA patients (p = 0.718). CA patients who achieved normal physiologic indices within ≤6 days (Early Responders, ER) had 8.8% SSI while the Late Responders (LR, normal by >6 days) had 49.3% SSI rate (p < 0.001). ER patients who received IV antibiotics for 1-2 postoperative days had higher SSI rates compared to 3, 4, 5, or 6 days, but higher odds of SSI were found only with 1 day. Additional oral antibiotics decreased SSI for ER (OR 0.36, 95% CI 0.159, 0.87; p = 0.0145), but not LR patients (OR 1.25, 95% CI 0.55, 2.85, p = 0.5951). CONCLUSIONS: Antibiotics given within 18-70 MBI for appendectomy may be associated with decreased SSI. Postoperative antibiotics should not be given for AA. In ER CA patients, additional oral antibiotics may decrease SSI. LEVEL OF EVIDENCE: Level III. TYPE OF STUDY: Retrospective comparative study.