An Unusual Lung Mass of Heterotopic Pancreatic Tissue in a Neonate With an Elevated Immunoreactive Trypsinogen on Newborn Screen.

We present a case of a neonate with tracheoesophageal fistula and esophageal atresia along with a suspicious lung mass who had a false-positive newborn screen for cystic fibrosis due to an elevated serum immunoreactive trypsinogen with an additionally elevated serum lipase. The infant's lung mass was found to contain heterotopic pancreatic tissue consisting of acini, ducts, and islet cells, without an associated gastrointestinal duplication cyst. This constellation of congenital abnormalities has not been described in previous literature. Also, this is the first reported case of a neonate with elevated serum pancreatic enzymes in which the underlying etiology was discovered to be heterotopic pancreas.

Impact of simulation-based training on perceived provider confidence in acute multidisciplinary pediatric trauma resuscitation.

PURPOSE: Simulation-based training has the potential to improve team-based care. We hypothesized that implementation of an in situ multidisciplinary simulation-based training program would improve provider confidence in team-based management of severely injured pediatric trauma patients. METHODS: An in situ multidisciplinary pediatric trauma simulation-based training program with structured debriefing was implemented at a free-standing children's hospital. Trauma providers were anonymously surveyed 1 month before (pre-), 1 month after (post-), and 2 years after implementation. RESULTS: Survey response rate was 49% (n = 93/190) pre-simulation, 22% (n = 42/190) post-simulation, and 79% (n = 150/190) at 2-year follow-up. These providers reported more anxiety (p = 0.01) and less confidence (p = 0.02) 1-month post-simulation. At 2-year follow-up, trained providers reported less anxiety (p = 0.02) and greater confidence (p = 0.01), compared to untrained providers. CONCLUSIONS: Implementation of an in situ multidisciplinary pediatric trauma simulation-based training program may initially lead to increased anxiety, but long-term exposure may lead to greater confidence. LEVEL OF EVIDENCE: II, Prospective cohort.

Direct to operating room trauma resuscitation decreases mortality among severely injured children.

BACKGROUND: Expediting evaluation and intervention for severely injured patients has remained a mainstay of advanced trauma care. One technique, direct to operating room (DOR) resuscitation, for selective adult patients has demonstrated decreased mortality. We sought to investigate the application of this protocol in children. METHODS: All DOR pediatric patients from 2009 to 2016 at a pediatric Level I trauma center were identified. Direct to OR criteria included penetrating injury, chest injuries, amputations, significant blood loss, cardiopulmonary resuscitation, and surgeon discretion. Demographics, injury patterns, interventions, and outcomes were analyzed. Observed mortality was compared with expected mortality, calculated using Trauma Injury Severity Score methodology, with two-tailed t tests, and a p value less than 0.5 was considered significant. RESULTS: Of 2,956 total pediatric trauma activations, 82 (2.8%) patients (age range, 1 month to 17 years) received DOR resuscitation during the study period. The most common indications for DOR were penetrating injuries (62%) and chest injuries (32%). Forty-four percent had Injury Severity Score (ISS) greater than 15, 33% had Glasgow Coma Scale (GCS) score of 8 or less, and 9% were hypotensive. The most commonly injured body regions were external (66%), head (34%), chest (30%), and abdomen (27%). Sixty-seven (82%) patients required emergent procedural intervention, most commonly wound exploration/repair (35%), central venous access (22%), tube thoracostomy (19%), and laparotomy (18%). Predictors of intervention were ISS greater than 15 (odds ratio, 14; p = 0.013) and GCS < 9 (odds ratio = 8.5, p = 0.044). The survival rate to discharge for DOR patients was 84% compared with an expected survival of 79% (Trauma Injury Severity Score) (p = 0.4). The greatest improvement relative to expected mortality was seen in the subgroup with penetrating trauma (84.5% vs 74.4%; p = 0.002). CONCLUSION: A selective policy of resuscitating the most severely injured children in the OR can decrease mortality. Patients suffering penetrating trauma with the highest ISS, and diminished GCS scores have the greatest benefit. Trauma centers with appropriate resources should evaluate implementing similar policies. LEVEL OF EVIDENCE: Diagnostic tests or criteria, level II.

Self-assessment of team performance using T-NOTECHS in simulated pediatric trauma resuscitation is not consistent with expert assessment.

BACKGROUND: The Trauma NOn-TECHnical Skills (T-NOTECHS) tool has been used to assess teamwork in trauma resuscitation, but its reliability and validity for self-assessment is unknown. Our purpose was to determine the reliability and validity of self-administered T-NOTECHS in pediatric trauma resuscitation. METHODS: Simulated in situ resuscitations were evaluated using T-NOTECHS in real time by experts and immediately afterwards by team members. Reliability was analyzed with linear-weighted kappa and intra-class correlation. T-NOTECHS scores were compared between expert (gold-standard) and self-assessment. RESULTS: Fifteen simulations were examined. T-NOTECHS scores were similar between self- and expert assessment for leadership. Self-assessment scores were higher than expert for the other domains and total composite score. Inter-rater reliability for total score was similar between the two groups, but differences were observed in the domains. CONCLUSIONS: Self-assessment is not interchangeable with expert rating when using T-NOTECHS. Future studies need to determine how self-assessment can be best utilized. LEVEL OF EVIDENCE: Studies of diagnostic accuracy - Level 2.

Prolonged Absence of Mechanoluminal Stimulation in Human Intestine Alters the Transcriptome and Intestinal Stem Cell Niche.

BACKGROUND & AIMS: For patients with short-bowel syndrome, intestinal adaptation is required to achieve enteral independence. Although adaptation has been studied extensively in animal models, little is known about this process in human intestine. We hypothesized that analysis of matched specimens with and without luminal flow could identify new potential therapeutic pathways. METHODS: Fifteen paired human ileum samples were collected from children aged 2-20 months during ileostomy-reversal surgery after short-segment intestinal resection and diversion. The segment exposed to enteral feeding was denoted as fed, and the diverted segment was labeled as unfed. Morphometrics and cell differentiation were compared histologically. RNA Sequencing and Gene Ontology Enrichment Analysis identified over-represented and under-represented pathways. Immunofluorescence staining and Western blot evaluated proteins of interest. Paired data were compared with 1-tailed Wilcoxon rank-sum tests with a P value less than .05 considered significant. RESULTS: Unfed ileum contained shorter villi, shallower crypts, and fewer Paneth cells. Genes up-regulated by the absence of mechanoluminal stimulation were involved in digestion, metabolism, and transport. Messenger RNA expression of LGR5 was significantly higher in unfed intestine, accompanied by increased levels of phosphorylated signal transducer and activator of transcription 3 protein, and CCND1 and C-MYC messenger RNA. However, decreased proliferation and fewer LGR5+, OLFM4+, and SOX9+ intestinal stem cells (ISCs) were observed in unfed ileum. CONCLUSIONS: Even with sufficient systemic caloric intake, human ileum responds to the chronic absence of mechanoluminal stimulation by up-regulating brush-border enzymes, transporters, structural genes, and ISC genes LGR5 and ASCL2. These data suggest that unfed intestine is primed to replenish the ISC population upon re-introduction of enteral feeding. Therefore, the elucidation of pathways involved in these processes may provide therapeutic targets for patients with intestinal failure. RNA sequencing data are available at Gene Expression Omnibus series GSE82147.

Utilizing technology to improve intraoperative family communication.

PURPOSE: To create and assess satisfaction with an electronic-medical-record (EMR) integrated communication system designed to optimize perioperative communication with families. METHODS: We built a tool in the EMR's intraoperative nursing navigation screen for sending customized or standardized text pages to families in English or Spanish. Preoperatively, families were given text pagers with instructions and a hospital map to facilitate leaving the waiting area. After 6 months, Press-Ganey™ data and internal surveys from randomly selected families, and all nurses and surgeons were analyzed for satisfaction and effectiveness. RESULTS: Press-Ganey™ data demonstrated 30% improvement in patient satisfaction (p < 0.05). Among families, > 90% indicated pagers were easy to use and provided the desired information during surgery. Of nurses, >90% found the system easy to use and believed it improved families' experience. All surgeons reported improved intraoperative communication and ease of finding families postoperatively. CONCLUSION: Perioperative family communication via EMR-integrated text improves efficiency and family, nurse, and surgeon satisfaction.

Management of pediatric intramuscular venous malformations.

BACKGROUND: Intramuscular venous malformations (VMs) are rare, but can be highly symptomatic. There are few reports on outcomes, particularly pain, functional limitations, and muscle contractures. We aimed to compare results of medical management, sclerotherapy, and surgical resection. METHODS: We retrospectively reviewed 45 patients with an extremity or truncal intramuscular VM between June 2005 and June 2015 at a single institution. Outcomes were compared between treatment modalities with ANOVA and χ2 tests. RESULTS: Six patients (13%) were treated with medical management, 4 (9%) with surgical resection, 23 (51%) with sclerotherapy, and 12 (27%) with both surgery and sclerotherapy. Sclerotherapy alone decreased pain in 72%. Only 20% of patients presented with muscle contracture. For these patients, 33% resolved with sclerotherapy, physical therapy, and aspirin; 22% resolved with surgery, and 45% had persistent contracture. 40% of patients treated with sclerotherapy then surgery developed new muscle contractures, compared to 4% of sclerotherapy only patients and 0% of surgery only patients (p=0.04). CONCLUSIONS: Medical management, surgery and sclerotherapy are effective treatments for intramuscular VMs. Observation and supportive care can be a primary treatment for patients with minimal symptomatology and no functional limitations. Sclerotherapy is more effective for treating pain than contractures and when used alone, rarely causes a new muscle contracture.

A cost and outcome analysis of pediatric single-incision appendectomy.

BACKGROUND: For appendicitis, single-incision laparoscopic appendectomy (SIA) has been proposed as an alternative to 3-port appendectomy (3PA). However, there remains controversy regarding outcomes and cost of SIA. We sought to review our experience with these two techniques to identify differences in these factors. MATERIALS AND METHODS: The charts of children (0-17 y) who underwent appendectomy at a tertiary pediatric hospital from 2011-2014 were retrospectively reviewed. Appendectomy was either performed through traditional 3PA or SIA (laparoscopically assisted via externalization through an umbilical incision). Demographic data including age, body mass index, comorbidities, and gender were examined. Information on perforation, operative time and cost, length of stay, and infectious complications for both SIA and 3PA was identified. Data were analyzed using student t tests and chi square analysis. RESULTS: A total of 337 patients underwent appendectomy (141 SIA and 197 3PA), 35.6% of whom (40 SIA, 80 3PA) had perforated appendicitis. For nonperforated appendicitis, SIA had significantly shorter operative times, decreased operative costs, and length of stay. However, these differences were not found for perforated appendicitis. Regardless of appendicitis severity, there was no difference in rates of wound infection, abscess, or readmission between the two techniques. CONCLUSIONS: Our study suggests that SIA is a faster, more cost effective alternative than 3PA for acute appendicitis. SIA did not result in increased infection rates for acute or perforated appendicitis and can be considered an equivalent alternative to 3PA in the surgical management of appendicitis.

The pediatric intestinal mucosal microbiome remains altered after clinical resolution of inflammatory and ischemic disease.

BACKGROUND: The pediatric intestinal microbiome is impacted by many factors, including age, diet, antibiotics, and environment. We hypothesized that in operative patients, alterations to antibiotics and mechanoluminal stimulation would demonstrate measurable changes in the intestinal microbiome and that microbial diversity would be reduced without normal mechanoluminal stimulation and with prolonged antibiotic treatment. METHODS: Bacterial 16s rRNA was extracted from swabbed samples of 43 intestines from 29 patients, aged 5 days to 13 years old. Swabs were obtained during initial resection or later stoma closure. Samples were compared using phylogenetic diversity whole tree alpha diversity and unweighted UniFrac distance beta diversity and by comparing significantly different taxonomic groups. RESULTS: Microbial community structure varied significantly between obstructive and inflammatory diseases (P = .001), with an effect size of 0.99 (0.97, 1.00). This difference persisted even 6 weeks after return to health. Family Enterobacter and Clostridiaceae predominated in patients with necrotizing enterocolitis or focal intestinal perforation; patients with an obstructive pathology had an abundance of Bacteroides. Comparison of UniFrac distance between paired proximal and distal intestines demonstrated that paired samples were significantly closer than any other comparison. CONCLUSION: In infants, inflammatory and ischemic intestinal pathologies treated with prolonged courses of antibiotics durably alter the intestinal mucosal microbiome. Diversion of mechanoluminal stimulation, however, does not.

Sequestration of Vascular Endothelial Growth Factor (VEGF) Induces Late Restrictive Lung Disease.

RATIONALE: Neonatal respiratory distress syndrome is a restrictive lung disease characterized by surfactant deficiency. Decreased vascular endothelial growth factor (VEGF), which demonstrates important roles in angiogenesis and vasculogenesis, has been implicated in the pathogenesis of restrictive lung diseases. Current animal models investigating VEGF in the etiology and outcomes of RDS require premature delivery, hypoxia, anatomically or temporally limited inhibition, or other supplemental interventions. Consequently, little is known about the isolated effects of chronic VEGF inhibition, started at birth, on subsequent developing lung structure and function. OBJECTIVES: To determine whether inducible, mesenchyme-specific VEGF inhibition in the neonatal mouse lung results in long-term modulation of AECII and whole lung function. METHODS: Triple transgenic mice expressing the soluble VEGF receptor sFlt-1 specifically in the mesenchyme (Dermo-1/rtTA/sFlt-1) were generated and compared to littermate controls at 3 months to determine the impact of neonatal downregulation of mesenchymal VEGF expression on lung structure, cell composition and function. Reduced tissue VEGF bioavailability has previously been demonstrated with this model. MEASUREMENTS AND MAIN RESULTS: Triple transgenic mice demonstrated restrictive lung pathology. No differences in gross vascular development or protein levels of vascular endothelial markers was noted, but there was a significant decrease in perivascular smooth muscle and type I collagen. Mutants had decreased expression levels of surfactant protein C and hypoxia inducible factor 1-alpha without a difference in number of type II pneumocytes. CONCLUSIONS: These data show that mesenchyme-specific inhibition of VEGF in neonatal mice results in late restrictive disease, making this transgenic mouse a novel model for future investigations on the consequences of neonatal RDS and potential interventions.