Hirschsprung-associated enterocolitis: a comprehensive review.

Hirschsprung-associated enterocolitis (HAEC) is an important cause of morbidity and the leading cause of mortality in patients with Hirschsprung disease. The pathophysiology of disease includes dysmotility of the enteric nervous system, dysbiosis of the microbiota, failure of the intestinal barrier, and impaired immunity. Common manifestations include fever, abdominal distension, lethargy, vomiting, and diarrhea. Given the non-specific signs and symptoms of HAEC, high clinical suspicion is warranted, especially in patients with risk factors. Diagnosis and management of HAEC depend on the severity of disease presentation. Several preoperative and postoperative modalities have been explored to prevent HAEC. The current review elaborates on the risk factors, pathogenesis, diagnosis, treatment, and prevention of HAEC.

Prospective Outcomes of Standardized Non-operative Management of Pancreatic Trauma With Ductal Injury in Children: Less is More.

BACKGROUND: Traumatic pancreatic laceration with ductal injury in children can be managed non-operatively (NOM); however, variable management affects outcomes. We hypothesized that a standardized management approach with early feeding and limited resource utilization is safe and improves outcomes. METHOD: Prospective, multicenter study of 13 pediatric trauma centers (2018-2022). Children with blunt pancreatic trauma with ductal injury were managed per a standard NOM pathway. Outcomes were compared to a historical NOM cohort with variable management. RESULTS: Of 22 patients, the median age was 7.5 years (range 1-14 years). Low-fat diet was started at median 4 days [IQR 2-7] and median hospital stay was 8 days [IQR 4-10]. One patient failed NOM and underwent distal pancreatectomy. Of the rest, most (17/21, 81%) had early recovery and discharged in median 6 days [IQR 4-8.25] while 4 (19%) had prolonged recovery (median stay 24 days, IQR 19.8-30.5) and higher peri-pancreatic cyst development (early 23.5% vs prolonged 75%,p = 0.05). Pancreatic ascites at presentation correlated with cyst development (p < 0.0001). Endoscopic stent (optional) was placed in 33% and did not prevent cyst development. Delayed exocrine pancreatic insufficiency was noted in 1 patient. Compared to the historic cohort (32 patients), TPN use was lower (pre-protocol 56% vs post 23%, p = 0.02), days to diet was shorter (pre-protocol 7 vs post 4; p = 0.03), and cyst development was lower (pre-protocol 81% vs post 33%, p < 0.001). CONCLUSION: Children with traumatic pancreatic ductal injury can be safely managed per the PTS NOM clinical pathway and most recover rapidly. Pancreatic ascites may predict pseudocyst formation. LEVELS OF EVIDENCE: IV. STUDY TYPE: therapeutic, comparative.

Pylorus-preserving pancreaticoduodenectomy with superior mesenteric vein resection and reconstruction in a child with recurrent hepatoblastoma after liver transplantation.

Pancreaticoduodenectomy with vascular reconstruction is rarely performed in children. We present a 3-year-old male with stage IV hepatoblastoma and pre-treatment extent of disease (PRETEXT) stage III with tumor into the portal vein and superior mesenteric vein (SMV), and with brain and lung metastases status post chemotherapy and stereotactic radiosurgery to left frontal brain lesion. He then underwent deceased donor liver transplant with Roux-en-Y hepaticojejunostomy complicated by two recurrences to bilateral lungs treated with wedge resections. His course lastly involved a third hepatoblastoma recurrence to the SMV that was managed with pylorus-preserving pancreaticoduodenectomy with SMV resection and reconstruction.

The Story of Dr. Asa G. Yancey and Surgical Innovation in the Face of Discrimination.

INTRODUCTION: Dr. Asa G. Yancey published a surgical technique describing a pull - through of normal colon through a cuff of aganglionic colon to treat Hirschsprung disease in 1952, 12 y before Dr. Franco Soave whose name is attached to the procedure. Yancey and his pioneering operation went unrecognized for over half a century because of discriminatory segregation in the publishing practices of academic medicine dating back to the 1950s. MATERIALS AND METHODS: We performed a literature review on the surgical therapies for Hirschsprung disease. This history was supplemented with first-hand accounts provided by Yancey's children. Further information by leaders of the American Pediatric Surgical Association Hirschsprung interest group regarding the future of surgical nomenclature for the endorectal pull-through procedure was acquired through interviews. RESULTS: A review of the literature revealed that Yancey's description of the pull-through technique for Hirschsprung disease was published 12 y prior to Soave's publication and yet, Yancey received little to no recognition for his work. Yancey's children describe a surgeon who was persistent in his endeavor to create a more inclusive field of academic surgery as well as a man who prioritized his family and the education of future surgeons. Conversations with the American Pediatric Surgical Association Hirschsprung interest group suggest active changes to give posthumous credit to Yancey, including renaming the procedure to the Yancey - Soave pull-through technique. CONCLUSIONS: Here, we provide a historical review of Yancey's career as well as insights on the man behind the surgery and how he persevered in academic surgery despite experiencing discrimination during the civil rights movement.

Evaluation and Management of Total Colonic Hirschsprung Disease: A Comprehensive Review From the American Pediatric Surgical Association (APSA) Hirschsprung Disease Interest Group.

BACKGROUND: Children with total colonic Hirschsprung disease (TCHD) are a unique group of patients with pre- and postoperative management challenges. This review provides a rational, expert-based approach to diagnosing and managing TCHD. METHODS: The guidelines were developed by the Hirschsprung Disease Interest Group members established by the American Pediatric Surgical Association (APSA) Board of Governors. Group discussions, literature review, and expert consensus were used to summarize the current knowledge regarding diagnosis, staged approach, the timing of pull-through, and pre-and postoperative management in children with TCHD. RESULTS: This paper presents recommendations for managing TCHD before and after reconstruction, including diagnostic criteria, surgical approaches, bowel management, diet, antibiotic prophylaxis, colonic irrigations, and post-surgical considerations. CONCLUSIONS: A clear understanding of the unique challenges posed by TCHD and consensus on its treatment are lacking in the literature. This review standardizes this patient group's pre- and postoperative management. LEVEL OF EVIDENCE: V.

The Impact of Low Workplace Support During Pregnancy on Surgeon Distress and Career Dissatisfaction.

OBJECTIVE: To describe the impact of lack of workplace support (LOWS) for obstetric health on surgeon distress and career satisfaction. BACKGROUND: Although most pregnant surgeons desire clinical duty reductions to mitigate obstetric risk, few modify their schedules due to low workplace support. METHODS: US surgeons with at least one live birth completed an electronic survey. LOWS during pregnancy was defined as (1) disagreeing that colleagues/leadership were supportive of obstetric-mandated bedrest; (2) feeling unable to reduce clinical duties despite health concerns due to risk of financial penalties, requirement to make up missed call shifts, being perceived as "weak", burdening colleagues, or accommodations being denied by the workplace. Multivariate logistic regression determined the association between LOWS and burnout, low quality of life, plans to leave clinical practice or to reduce work hours, and likelihood of recommending a surgical career to one's child. RESULTS: Of 557 surgeons, the 360 (64.6%) who reported LOWS during pregnancy were more likely to report burnout (OR:2.57; 95%CI:1.60-4.13), low quality of life (OR:1.57; 95%CI:1.02-2.41), a desire to leave their practice (OR:2.74; 95%CI: 1.36-5.49), plans to reduce clinical hours in the next year (OR:4.25; 95%CI:1.82-9.90), and were less likely to recommend their career to their child (OR:0.44; 95%CI:0.28-0.70). CONCLUSIONS: LOWS for maternal-fetal health concerns is associated with burnout, low quality of life, and career dissatisfaction. The work environment is a modifiable factor requiring system-level interventions to limit clinical work during pregnancy and provide fair compensation for covering surgeons. Supporting surgeons during pregnancy is a short-term investment with long-term implications for improving longevity and diversity of the workforce.

Management and Outcomes of Hepatoblastoma in Patients With Trisomy 18: A Systematic Review and Pooled Analysis of 70 Patients.

INTRODUCTION: Predicted 1-year survival of children with trisomy 18 (T18) has increased to 59.3%. We aimed to systematically review the characteristics, management, and outcomes of children with T18 and hepatoblastoma. METHODS: A systematic literature review of the PubMed, Embase, Scopus, Web of Science, and Cochrane Library databases was performed according to the PRISMA 2020 statement (end-of-search date: 03/03/2024). RESULTS: Fifty studies reporting on 70 patients were included. The median age at diagnosis was 11.5 months, 85.9% were female (n = 55/64), and 15.0% had mosaic T18 (n = 6/40). Diagnosis was made during symptom evaluation (most commonly hepatomegaly or abdominal mass) in 45.5% (n = 15/33), incidentally in 24.2% (n = 8/33), during surveillance with abdominal ultrasound in 18.2% (n = 6/33), and at autopsy in 12.1% (n = 4/33). The median tumor size was 6.4 cm, 33.3% had multiple tumors (n = 14/42), and metastasis was present in one patient (3.8%; n = 1/26). Neoadjuvant chemotherapy was administered in 42.6% (n = 26/61) and adjuvant chemotherapy in 31.6% (n = 18/57). Surgical treatment was performed in 64.2% (n = 43/67). Of the patients not diagnosed on autopsy, overall mortality was 35.5% (n = 22/62) over a median follow-up of 11.0 months. Among the 26 deceased patients (including those diagnosed on autopsy), the most common causes of death were cardiopulmonary disease (38.5%, n = 10/26) and tumor progression (30.8%, n = 8/26). CONCLUSIONS: T18 does not preclude resection with curative intent for hepatoblastoma. Combination of surgery and chemotherapy should be considered in children on an individualized basis depending on tumor characteristics and underlying cardiopulmonary comorbidities. Locoregional modalities may have a role in the setting of severe comorbidities. LEVEL OF EVIDENCE: Level IV evidence.

Current Management Strategies and Outcomes in Children With Adrenocortical Carcinoma.

INTRODUCTION: Adrenocortical carcinoma (ACC) is a rare but aggressive pediatric endocrine tumor. However, there is no recent US national report on the management or outcomes of pediatric ACC. We aimed to examine the clinical characteristics, current management strategies, and outcomes of pediatric ACC. METHODS: In this retrospective National Cancer Database study between 2004 and 2019, children (<18 y) with ACC were included. Overall survival was examined by means of Kaplan-Meier method, log-rank tests, and Cox regression modeling. RESULTS: Seventy-eight children with ACC were included. The median age was 10 y, the median tumor size was 10.2 cm, and 35.9% had metastasis at diagnosis. Most patients underwent surgical treatment (84.6%), 56.4% received chemotherapy, and 7.7% received radiation. The 1-, 3-, and 5-y overall survival rates were 87.0%, 62.0%, and 60.1%, respectively. In unadjusted analysis, surgical treatment was associated with improved overall survival (log-rank test, P < 0.001). In multivariable Cox regression, metastasis at diagnosis was associated with inferior overall survival (hazard ratio: 2.72, 95% confidence interval: 1.15-6.40, P = 0.02), when adjusting for age, tumor size, receipt of surgical treatment, and chemotherapy. In patients with nonmetastatic ACC, increasing age was associated with inferior overall survival (hazard ratio: 1.12, 95% confidence interval: 1.00-1.24, P = 0.04), when adjusting for tumor size, receipt of surgical treatment, and chemotherapy. CONCLUSIONS: Most children with ACC in the USA undergo surgical treatment with about half of these also receiving chemotherapy. Metastasis at diagnosis was independently associated with inferior overall survival; in patients with nonmetastatic ACC, increasing age was independently associated with inferior overall survival.

Lack of Association of Pediatric Surgical Patient Outcomes With Increased Disposable Operating Room Supply Costs.

INTRODUCTION: There is wide variation in the cost of disposable operating room supplies between surgeons performing the same operation at the same institution. The general relationship between variation in disposable supply cost and patient outcomes is unknown. We aimed to evaluate the relationship between disposable supply cost and patient outcomes for sixteen common operations. METHODS: Cost data were reviewed for the most common procedures performed by five surgical divisions at a single children's hospital over a six-month period in 2021. For procedure, the median disposable OR costs were calculated. Each operation performed was categorized as low cost (below the group median) or high cost (above the group median. We compared the rates of adverse events (clinic visit within 5 days, 30-day emergency department visit, unplanned reoperation, unplanned readmission, anesthesia complications, prolonged hospital length of stay, need for blood product transfusion, or death) between procedures with low and high disposable supply costs. RESULTS: 1139 operations performed by 48 unique surgeons from five specialties were included; 596 (52%) were low-cost and 543 (48%) high-cost. The low and high-cost groups did not differ regarding most demographic characteristics. Overall, 21.9% of children suffered any adverse outcome; this rate did not differ between the low and high-cost groups when evaluated individually or in aggregate (20.5% vs 23.6%, p = 0.23). CONCLUSION: Our data demonstrate that across a wide range of pediatric surgical procedures, the cost of disposable operating room supplies was not associated with the risk of adverse outcomes. LEVEL OF EVIDENCE: Level 3.

Effect of Early vs Late Inguinal Hernia Repair on Serious Adverse Event Rates in Preterm Infants: A Randomized Clinical Trial.

Importance: Inguinal hernia repair in preterm infants is common and is associated with considerable morbidity. Whether the inguinal hernia should be repaired prior to or after discharge from the neonatal intensive care unit is controversial. Objective: To evaluate the safety of early vs late surgical repair for preterm infants with an inguinal hernia. Design, Setting, and Participants: A multicenter randomized clinical trial including preterm infants with inguinal hernia diagnosed during initial hospitalization was conducted between September 2013 and April 2021 at 39 US hospitals. Follow-up was completed on January 3, 2023. Interventions: In the early repair strategy, infants underwent inguinal hernia repair before neonatal intensive care unit discharge. In the late repair strategy, hernia repair was planned after discharge from the neonatal intensive care unit and when the infants were older than 55 weeks' postmenstrual age. Main Outcomes and Measures: The primary outcome was occurrence of any prespecified serious adverse event during the 10-month observation period (determined by a blinded adjudication committee). The secondary outcomes included the total number of days in the hospital during the 10-month observation period. Results: Among the 338 randomized infants (172 in the early repair group and 166 in the late repair group), 320 underwent operative repair (86% were male; 2% were Asian, 30% were Black, 16% were Hispanic, 59% were White, and race and ethnicity were unknown in 9% and 4%, respectively; the mean gestational age at birth was 26.6 weeks [SD, 2.8 weeks]; the mean postnatal age at enrollment was 12 weeks [SD, 5 weeks]). Among 308 infants (91%) with complete data (159 in the early repair group and 149 in the late repair group), 44 (28%) in the early repair group vs 27 (18%) in the late repair group had at least 1 serious adverse event (risk difference, -7.9% [95% credible interval, -16.9% to 0%]; 97% bayesian posterior probability of benefit with late repair). The median number of days in the hospital during the 10-month observation period was 19.0 days (IQR, 9.8 to 35.0 days) in the early repair group vs 16.0 days (IQR, 7.0 to 38.0 days) in the late repair group (82% posterior probability of benefit with late repair). In the prespecified subgroup analyses, the probability that late repair reduced the number of infants with at least 1 serious adverse event was higher in infants with a gestational age younger than 28 weeks and in those with bronchopulmonary dysplasia (99% probability of benefit in each subgroup). Conclusions and Relevance: Among preterm infants with inguinal hernia, the late repair strategy resulted in fewer infants having at least 1 serious adverse event. These findings support delaying inguinal hernia repair until after initial discharge from the neonatal intensive care unit. Trial Registration: ClinicalTrials.gov Identifier: NCT01678638.

Association of Sociodemographic Factors with Surgical Management of Hepatoblastoma and Hepatocellular Carcinoma in Children.

OBJECTIVE: To evaluate for disparities in surgical care among US children with hepatoblastoma (HB) and hepatocellular carcinoma (HCC). STUDY DESIGN: In this retrospective National Cancer Database study (2004-2015), children aged <18 years with HB or HCC were included. Multivariable mixed-effects logistic regression was used to evaluate the association of sociodemographic factors (age, sex, race and ethnicity, insurance status, income, proximity to treating hospital) with the odds of undergoing surgical treatment after adjusting for disease-related factors (tumor size, metastasis, comorbidities) and hospital-level effects. Subgroup analyses by tumor histology were performed. RESULTS: A total of 811 children were included (HB: 80.9%; HCC: 19.1%), of which 610 (75.2%) underwent surgical treatment. Following adjustment, decreased odds of undergoing surgical treatment were associated with Black race (OR: 0.46 vs White, 95% CI [95% CI]: 0.26-0.80, P = .01), and having Medicaid (OR: 0.58 vs private, 95% CI: 0.38-0.88, P = .01) or no insurance (OR: 0.33 vs private, 95% CI: 0.13-0.80, P = .02). In children with HB, Black race was associated with decreased odds of undergoing surgical treatment (OR: 0.47 vs White, 95% CI: 0.25-0.89, P = .02). In children with HCC, Medicaid (OR: 0.10 vs private, 95% CI: 0.03-0.35, P < .001), or no insurance status (OR: 0.10 vs private, 95% CI: 0.01-0.83, P = .03) were associated with decreased odds of undergoing surgical treatment. Other than metastatic disease, no additional factors were associated with likelihood of surgical treatment in any group. CONCLUSIONS: Black race and having Medicaid or no insurance are independently associated with decreased odds of surgical treatment in children with HB and HCC, respectively. These children may be less likely to undergo curative surgery for their liver cancer.

Hepatic Metastasectomy in Pediatric Patients: An Observational Study.

BACKGROUND: The role of hepatectomy for metastatic disease in children is controversial. Rationales include potential cure, obtaining a diagnosis, and guiding chemotherapy decisions. This study examines the safety and utility of hepatic metastasectomy for children at a single institution. METHODS: After IRB approval (#22-1258), medical records were reviewed from 1995 to 2022 for children undergoing hepatic metastasectomy. En-bloc hepatectomies during primary tumor resection were excluded. RESULTS: Hepatic metastasectomy was performed in 16 patients for a variety of histologies. Median patient age was 12.2 years [IQR 6.9-22.6], and 13/16 patients were female (81 %). Number of hepatic metastases ranged from 1 to 23 and involved between 1 and 8 Couinaud segments. Anatomic resections included 4 hemihepatectomies and 1 sectionectomy. All other resections were nonanatomic. 3/6 resections for germ cell tumor (GCT) revealed only mature teratoma, driving adjuvant therapy decisions. When indicated, median time to adjuvant chemotherapy was 19 days [IQR 11-22]. No patients had Clavien-Dindo Class III or higher perioperative morbidity. Three patients (1 GCT, 1 adrenocortical carcinoma (ACC), and 1 gastric neuroendocrine tumor (GNET) experienced hepatic relapse. The patients with relapsed GCT and GNET are alive with disease at 17 and 135 months, respectively. The patient with ACC died of disease progression and liver failure. One patient with Wilms tumor experienced extrahepatic, retroperitoneal recurrence and died. With a median follow-up of 38 months, 10-year disease-specific and disease-free survival were 77 % and 61 %, respectively. CONCLUSIONS: Hepatic metastasectomy can be accomplished safely in children, may guide adjuvant therapy decisions, and is associated with long-term survival in selected patients. LEVEL OF EVIDENCE: Level IV. TYPE OF STUDY: Treatment Study, Case series with no comparison group.

Delphi Process for Validation of Fluid Treatment Algorithm for Critically Ill Pediatric Trauma Patients.

INTRODUCTION: While intravenous fluid therapy is essential to re-establishing volume status in children who have experienced trauma, aggressive resuscitation can lead to various complications. There remains a lack of consensus on whether pediatric trauma patients will benefit from a liberal or restrictive crystalloid resuscitation approach and how to optimally identify and transition between fluid phases. METHODS: A panel was comprised of physicians with expertise in pediatric trauma, critical care, and emergency medicine. A three-round Delphi process was conducted via an online survey, with each round being followed by a live video conference. Experts agreed or disagreed with each aspect of the proposed fluid management algorithm on a five-level Likert scale. The group opinion level defined an algorithm parameter's acceptance or rejection with greater than 75% agreement resulting in acceptance and greater than 50% disagreement resulting in rejection. The remaining were discussed and re-presented in the next round. RESULTS: Fourteen experts from five Level 1 pediatric trauma centers representing three subspecialties were included. Responses were received from 13/14 participants (93%). In round 1, 64% of the parameters were accepted, while the remaining 36% were discussed and re-presented. In round 2, 90% of the parameters were accepted. Following round 3, there was 100% acceptance by all the experts on the revised and final version of the algorithm. CONCLUSIONS: We present a validated algorithm for intavenous fluid management in pediatric trauma patients that focuses on the de-escalation of fluids. Focusing on this time point of fluid therapy will help minimize iatrogenic complications of crystalloid fluids within this patient population.

Hirschsprung disease.

Hirschsprung disease (HSCR) is a rare congenital intestinal disease that occurs in 1 in 5,000 live births. HSCR is characterized by the absence of ganglion cells in the myenteric and submucosal plexuses of the intestine. Most patients present during the neonatal period with the first meconium passage delayed beyond 24 h, abdominal distension and vomiting. Syndromes associated with HSCR include trisomy 21, Mowat-Wilson syndrome, congenital central hypoventilation syndrome, Shah-Waardenburg syndrome and cartilage-hair hypoplasia. Multiple putative genes are involved in familial and isolated HSCR, of which the most common are the RET proto-oncogene and EDNRB. Diagnosis consists of visualization of a transition zone on contrast enema and confirmation via rectal biopsy. HSCR is typically managed by surgical removal of the aganglionic bowel and reconstruction of the intestinal tract by connecting the normally innervated bowel down to the anus while preserving normal sphincter function. Several procedures, namely Swenson, Soave and Duhamel procedures, can be undertaken and may include a laparoscopically assisted approach. Short-term and long-term comorbidities include persistent obstructive symptoms, enterocolitis and soiling. Continued research and innovation to better understand disease mechanisms holds promise for developing novel techniques for diagnosis and therapy, and improving outcomes in patients.

Altered hepatic and intestinal homeostasis in a neonatal murine model of short-term total parenteral nutrition and antibiotics.

Parenteral nutrition (PN) prevents starvation and supports metabolic requirements intravenously when patients are unable to be fed enterally. Clinically, infants are frequently provided PN in intensive care settings along with exposure to antibiotics (ABX) to minimize infection during care. Unfortunately, neonates experience extremely high rates of hepatic complications. Adult rodent and piglet models of PN are well-established but neonatal models capable of leveraging the considerable transgenic potential of the mouse remain underdeveloped. Utilizing our newly established neonatal murine PN mouse model, we administered ABX or controlled drinking water to timed pregnant dams to disrupt the maternal microbiome. We randomized mouse pups to PN or sham surgery controls +/- ABX exposure. ABX or short-term PN decreased liver and brain organ weights, intestinal length, and mucosal architecture (vs. controls). PN significantly elevated evidence of hepatic proinflammatory markers, neutrophils and macrophage counts, bacterial colony-forming units, and evidence of cholestasis risk, which was blocked by ABX. However, ABX uniquely elevated metabolic regulatory genes resulting in accumulation of hepatocyte lipids, triglycerides, and elevated tauro-chenoxycholic acid (TCDCA) in serum. Within the gut, PN elevated the relative abundance of Akkermansia, Enterococcus, and Suterella with decreased Anaerostipes and Lactobacillus compared with controls, whereas ABX enriched Proteobacteria. We conclude that short-term PN elevates hepatic inflammatory stress and risk of cholestasis in early life. Although concurrent ABX exposure protects against hepatic immune activation during PN, the dual exposure modulates metabolism and may contribute toward early steatosis phenotype, sometimes observed in infants unable to wean from PN.NEW & NOTEWORTHY This study successfully established a translationally relevant, murine neonatal parenteral nutrition (PN) model. Short-term PN is sufficient to induce hepatitis-associated cholestasis in a neonatal murine model that can be used to understand disease in early life. The administration of antibiotics during PN protects animals from bacterial translocation and proinflammatory responses but induces unique metabolic shifts that may predispose the liver toward early steatosis.

Paneth cell dysfunction in radiation injury and radio-mitigation by human α-defensin 5.

Introduction: The mechanism underlying radiation-induced gut microbiota dysbiosis is undefined. This study examined the effect of radiation on the intestinal Paneth cell α-defensin expression and its impact on microbiota composition and mucosal tissue injury and evaluated the radio-mitigative effect of human α-defensin 5 (HD5). Methods: Adult mice were subjected to total body irradiation, and Paneth cell α-defensin expression was evaluated by measuring α-defensin mRNA by RT-PCR and α-defensin peptide levels by mass spectrometry. Vascular-to-luminal flux of FITC-inulin was measured to evaluate intestinal mucosal permeability and endotoxemia by measuring plasma lipopolysaccharide. HD5 was administered in a liquid diet 24 hours before or after irradiation. Gut microbiota was analyzed by 16S rRNA sequencing. Intestinal epithelial junctions were analyzed by immunofluorescence confocal microscopy and mucosal inflammatory response by cytokine expression. Systemic inflammation was evaluated by measuring plasma cytokine levels. Results: Ionizing radiation reduced the Paneth cell α-defensin expression and depleted α-defensin peptides in the intestinal lumen. α-Defensin down-regulation was associated with the time-dependent alteration of gut microbiota composition, increased gut permeability, and endotoxemia. Administration of human α-defensin 5 (HD5) in the diet 24 hours before irradiation (prophylactic) significantly blocked radiation-induced gut microbiota dysbiosis, disruption of intestinal epithelial tight junction and adherens junction, mucosal barrier dysfunction, and mucosal inflammatory response. HD5, administered 24 hours after irradiation (treatment), reversed radiation-induced microbiota dysbiosis, tight junction and adherens junction disruption, and barrier dysfunction. Furthermore, HD5 treatment also prevents and reverses radiation-induced endotoxemia and systemic inflammation. Conclusion: These data demonstrate that radiation induces Paneth cell dysfunction in the intestine, and HD5 feeding prevents and mitigates radiation-induced intestinal mucosal injury, endotoxemia, and systemic inflammation.

Providers' Perceptions Versus Practices Inform Pediatric Colorectal Enhanced Recovery After Surgery Implementation.

INTRODUCTION: There are many barriers to the implementation of an enhanced recovery after surgery (ERAS) pathway. The aim of this study was to compare surgeon and anesthesia perceptions with current practices prior to the initiation of an ERAS protocol in pediatric colorectal patients and to use that information to inform ERAS implementation. METHODS: This was a mixed method single institution study of barriers to implementation of an ERAS pathway at a free-standing children's hospital. Anesthesiologists and surgeons at a free-standing children's hospital were surveyed regarding current practices of ERAS components. A retrospective chart review was performed of 5- to 18-y-old patients undergoing colorectal procedures between 2013 and 2017, followed by the initiation of an ERAS pathway, and a prospective chart review for 18 mo postimplementation. RESULTS: The response rate was 100% (n = 7) for surgeons and 60% (n = 9) for anesthesiologists. Preoperative nonopioid analgesics and regional anesthesia were rarely used. Intraoperatively, 54.7% of patients had a fluid balance of <10 cc/kg/h and normothermia was achieved in only 38.7%. Mechanical bowel prep was frequently utilized (48%). Median nil per os time was significantly longer than required at 12 h. Postoperatively, 42.9% of surgeons reported that patients could have clears on postoperative day zero, 28.6% on postoperative day one, and 28.6% after flatus. In reality, 53.3% of patients were started on clears after flatus, with a median time of 2 d. Most surgeons (85.7%) expected patients to get out of bed once awake from anesthesia; however, median time that patients were out of bed was postoperative day one. While most surgeons reported frequent use of acetaminophen and/or ketorolac, only 69.3% received any nonopioid analgesic postoperatively, with only 41.3% receiving two or more nonopioid analgesics. Nonopioid analgesia showed the highest rates of improvement from retrospective to prospective: preoperative use of analgesics increased from 5.3% to 41.2% (P < 0.0001), postoperative use of acetaminophen increased by 27.4% (P = 0.5), Toradol by 45.5% (P = 0.11), and gabapentin by 86.7% (P < 0.0001). Postoperative nausea/vomiting prophylaxis with >1 class of antiemetic increased from 8% to 47.1% (P < 0.001). The length of stay was unchanged (5.7 versus 4.4 d, P = 0.14). CONCLUSIONS: For the successful implementation of an ERAS protocol, perceptions versus reality must be assessed to determine current practices and identify barriers to implementation.

Readability of patient and family education materials on pediatric surgical association websites.

PURPOSE: Globally, pediatric surgical association websites present patient/family education materials on an extensive list of conditions, including descriptions of the condition, signs and symptoms, diagnostic modalities, and treatment options. The purpose of this project was to assess the readability of pediatric surgical association websites' patient/family education materials. METHODS: With IRB approval, we accessed all patient/family education materials on pediatric surgical association websites from around the globe and used multiple grade-level assessments and readability assessments to determine the reading level at which the information is presented. RESULTS: The American Pediatric Surgical Association (APSA) website and the British Association of Paediatric Surgeons (BAPS) present publicly accessible patient/family education materials. Seventy-four (74) conditions on APSA's website were analyzed. Three grade-level assessments and the Flesch Reading Ease assessment indicated that the articles are written at high school reading levels. No articles were available in languages other than English. BAPS presented 6 conditions, most of which were more readable than their APSA counterparts. CONCLUSIONS: Our analysis indicates that the patient/family education materials available on pediatric surgical association websites may not be written at a level that is comprehensible by the general population. Potential solutions include re-writing the materials with an emphasis on readability and presenting materials in languages other than English. LEVEL OF EVIDENCE: V.

Impact of institutional prophylaxis guidelines on rates of pediatric venous thromboembolism following trauma-A multicenter study from the pediatric trauma society research committee.

BACKGROUND: A paucity of data exists with regard to the incidence, management, and outcomes of venous thromboembolism (VTE) in injured children. We sought to determine the impact of institutional chemoprophylaxis guidelines on VTE rates in a pediatric trauma population. METHODS: A retrospective review of injured children (≤15 years) admitted between 2009 and 2018 at 10 pediatric trauma centers was performed. Data were gathered from institutional trauma registries and dedicated chart review. The institutions were surveyed as to whether they had chemoprophylaxis guidelines in place for high-risk pediatric trauma patients, and outcomes were compared based on the presence of guidelines using χ 2 analysis ( p < 0.05). RESULTS: There were 45,202 patients evaluated during the study period. Three institutions (28,359 patients, 63%) had established chemoprophylaxis policies during the study period ("Guidelines"); the other seven centers (16,843 patients, 37%) had no such guidelines ("Standard"). There were significantly lower rates of VTE in the Guidelines group, but these patients also had significantly fewer risk factors. Among critically injured children with similar clinical presentations, there was no difference in VTE rate. Specifically within the Guidelines group, 30 children developed VTE. The majority (17/30) were actually not indicated for chemoprophylaxis based on institutional guidelines. Still, despite protocols only one VTE patient in the guidelines group who was indicated for intervention ended up receiving chemoprophylaxis prior to diagnosis. No consistent ultrasound screening protocol was in place at any institution during the study. CONCLUSION: The presence of an institutional policy to guide chemoprophylaxis for injured children is associated with a decreased overall frequency of VTE, but this disappears when controlling for patient factors. However, the overall efficacy is impacted by a combination of deficits in guideline compliance and structure. Further prospective data are needed to help determine the ideal role for chemoprophylaxis and protocols in pediatric trauma. LEVEL OF EVIDENCE: Therapeutic/Care Management; Level IV.

Upper Gastrointestinal Series in the Workup for Pediatric Gastrostomy Placement: Does it Delay Care?

INTRODUCTION: Upper gastrointestinal (UGI) series is often part of the workup prior to the placement of gastrostomy tubes in children. Prior studies have suggested UGI to be limited in utility and an extra financial burden. The goal of this study was to investigate the utility and cost of UGI studies. METHODS: A retrospective, case control study of patients aged < 18 y receiving gastrostomy tubes at a free-standing children's hospital between 2012 and 2017. Total costs were obtained from the Pediatric Health Information System. RESULTS: Six hundred eighty five patients underwent gastrostomy placement during the study period. UGI was obtained in 90.8% of patients; 23.6% of studies were abnormal. The most common abnormal findings were reflux (13.8%) and abnormal anatomy (5.8%). The median time to obtain a UGI was 0.82 d (interquartile range 0.22-1.05). Obtaining a UGI was associated with delayed care in 104 patients (15.2%). If a delay was encountered, median time was 2.47 d (interquartile range 1.86-2.99). Ladd's procedures were performed in 12 patients (1.7%) found to have malrotation on UGI. None of the 63 patients who did not undergo UGI required a Ladd's procedure. Patients that had a UGI did not experience an increase in overall length of stay (14.3 versus 15.6 d, excluding intensive care unit patients), operative time (34 versus 39 min), or a change in rate of operative complications (11.5% versus 14.3%). In addition, UGI did not have a significant impact on total adjusted costs ($49,844 versus $83,438 without UGI, P = 0.12) but did slightly increase total adjusted costs per day ($2212 versus $1999 without UGI, P = 0.01). CONCLUSIONS: UGI prior to gastrostomy placement in children rarely identified abnormal findings that changed the operative plan, was associated with delayed care in 15% of patients, and was associated with slightly increased costs per day. Further analyses to identify subsets of children that may benefit from routine UGI are warranted.