Pneumatosis Intestinalis in Children With Intestinal Failure: The Result of Intestinal Stress From Enteral Feeding?

OBJECTIVE: The incidence and significance of pneumatosis intestinalis (PI) in children with a diagnosis of intestinal failure is not well understood. The aim of this study was to identify clinical and anatomical factors associated with the imaging findings of PI in patients with intestinal failure. METHODS: We performed a retrospective review of all children with a diagnosis of intestinal failure at Children's Hospital Colorado between January 2019 and April 2022. Patients were stratified and compared based on the incidence of PI on abdominal imaging. Differences were compared using 2-sample Wilcoxon tests, chi-square, or Fisher exact tests. RESULTS: There were 111 patients identified with a diagnosis of intestinal failure and 30.6% (34) developed at least 1 instance of PI. There were no differences in etiology of intestinal failure or anatomy between those who developed PI and those who did not. Patients who developed PI, were less likely to be on total parental nutrition (60.6% vs 98.6%, P < 0.001) and more likely to be receiving any form of enteral feeds (87.9% vs 66.2%, P = 0.035) or tube feeds (75.8% vs 44.2%, P = 0.0045). Of the children with PI, 30.3% (10) were undergoing an enteral feed advancement at time of PI development. Three patients with PI underwent laparotomy for PI treatment, 2 of which were negative laparotomies. CONCLUSIONS: The development of PI in children with intestinal failure is likely a benign finding. It is associated with enteral feeding and may be due to increased intestinal stress.

How we approach pediatric congenital chylous effusions and ascites.

Congenital lymphatic leak may develop in patients with maldeveloped lymphatics and result in life-threatening fluid and electrolyte imbalance, protein deficiency, and immunodeficiency. Rapid diagnosis and therapy are necessary to prevent these complications; however, the field lacks clinical trials to support standardized diagnostic treatment guidelines. We present our current multidisciplinary approach to the diagnosis and management of congenital lymphatic leak including chylous pleural effusions and ascites. Depending on the rate of lymphatic leak, therapy can range from observation with nutritional modifications to surgical and interventional procedures aimed to reduce lymphatic drainage. Modalities to image central and peripheral lymphatics have advanced considerably. Genetic variants and subsequent targets that drive lymphatic maldevelopment have expanded the repertoire of possible pharmacotherapeutic options.

Size and Prevalence of Pediatric Aerodigestive Programs in 2017.

OBJECTIVE: Pediatric aerodigestive programs appear to be rapidly proliferating and provide multidisciplinary, coordinated care to complex, medically fragile children. Pediatric subspecialists are considered essential to these programs. This study evaluated the state of these programs in 2017 by surveying their size, composition, prevalence, and the number of patients that they serve. METHODS: The North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Aerodigestive Special Interest Group leadership distributed an 11-question survey to the Pediatric Gastroenterology International Listserv. The mean time of the programs' existence, number of half-day clinics, number of procedure days, number of patients evaluated, and the lead primary specialty were evaluated. RESULTS: Thirty-four programs responded. Twenty-five were based in academic centers. Thirty-one programs were located across the United States. The average time of program existence was 5.3 years (standard deviation [SD] = 4.3; range 1-17 years). Approximately 64.7% were started in the past 5 years. Twelve programs were based in the division of gastroenterology. The average number of gastroenterologists serving aerodigestive programs was 2 (SD = 1.1). The mean number of half-day clinic sessions and procedure days were 2.8 (SD = 2.9) and 2.6 (SD = 2), respectively. New and follow-up visits per year in each program averaged 184 (SD = 168; range 10-750). CONCLUSIONS: Pediatric aerodigestive programs are prevalent, proliferating, and serve a large number of complex patients across North America and the world. This survey demonstrated that programs are predominantly based in academic settings. The number of patients cared for by aerodigestive centers varies widely depending on size and age of program.

Strokes in children: a systematic review.

CONTEXT: Pediatric strokes lead to significant morbidity and mortality. To date, no systematic review has been available to guide the initial diagnostic approach to pediatric stroke. OBJECTIVE: The objective of this review was to elucidate the current data regarding etiologies of stroke in children and then develop an initial diagnostic evaluation for the pediatric patient presenting to the emergency department with a stroke. DATA SOURCE: Using the PubMed engine, the MEDLINE database was searched using the Preferred Reporting Items in Systematic Reviews and Meta-Analyses guidelines. STUDY SELECTION: The inclusion and exclusion criteria were established a priori. Studies must have extractable data regarding first strokes in pediatric patients with clear diagnostic categories. DATA EXTRACTION: A standardized tool was developed to extract demographic data and stroke etiologies. RESULTS: Twelve studies were found that met the inclusion criteria. From these studies, a total of 1455 children aged between 21 days and 20 years were available to assess the etiologies of stroke. CONCLUSIONS: In pediatric patients, the etiologies of stroke are varied and differ for children with ischemic stroke versus hemorrhagic stroke. With the present systematic review, a guide to the initial evaluation of stroke is presented.

A Novel Approach to Assessment of US Pediatric Trauma System Development.

Importance: Mature trauma systems are critical in building and maintaining national, state, and local resilience against all-hazard disasters. Currently, pediatric state trauma system plans are not standardized and thus are without concrete measures of potential effectiveness. Objective: To develop objective measures of pediatric trauma system capability at the state level, hypothesizing significant variation in capabilities between states, and to provide a contemporary report on the status of national pediatric trauma system planning and development. Design, Setting, and Participants: A national survey was deployed in 2018 to perform a gap analysis of state pediatric trauma system capabilities. Four officials from each state were asked to complete the survey regarding extensive pediatric-related or specific trauma system parameters. Using these parameters, a panel of 14 individuals representing national stakeholder sectors in pediatric trauma care convened to identify the essential components of the ideal pediatric trauma system using Delphi methodology. Data analysis was conducted from March 16, 2019, to February 23, 2020. Main Outcomes and Measures: Based on results from the national survey and consensus panel parameters, each state was given a composite score. The score was validated using US Centers for Disease Control and Prevention Wide-Ranging Online Data for Epidemiologic Research (CDC WONDER) fatal injury database. Results: The national survey had less than 10% missing data. The consensus panel reached agreement on 6 major domains of pediatric trauma systems (disaster, legislation/funding, access to care, injury prevention/recognition, quality improvement, pediatric readiness) and was used to develop the Pediatric Trauma System Assessment Score (PTSAS) based on 100 points. There was substantial variation across states, with state scores ranging from 48.5 to 100. Based on US CDC WONDER data, for every 1-point increase in PTSAS, there was a 0.12 per 100 000 decrease in mortality (95% CI, -0.22 to -0.02; P = .03). Conclusions and Relevance: Results of this cross-sectional study suggest that a more robust pediatric trauma system has a significant association with pediatric injury mortality. This study assessed the national landscape of capability and preparedness to provide pediatric trauma care at the state level. These parameters can tailor the maturation of children's interests within a state trauma system and assist with future state, regional, and national planning.

Case report: Cystic fibrosis with kwashiorkor: A rare presentation in the era of universal newborn screening.

Background: Universal newborn screening changed the way medical providers think about the presentation of cystic fibrosis (CF). Before implementation of universal screening, it was common for children with CF to present with failure to thrive, nutritional deficiencies, and recurrent infections. Now, nearly all cases of CF are diagnosed by newborn screening shortly after birth before significant symptoms develop. Therefore, providers often do not consider this illness in the setting of a normal newborn screen. Newborn screening significantly decreases the risk of complications in early childhood, yet definitive testing should be pursued if a patient with negative newborn screening presents with symptoms consistent with CF, including severe failure to thrive, metabolic alkalosis due to significant salt losses, or recurrent respiratory infections. Case presentation: We present a case of a 6-month-old infant male with kwashiorkor, severe edema, multiple vitamin deficiencies, hematemesis secondary to coagulopathy, and diffuse erythematous rash, all secondary to severe pancreatic insufficiency. His first newborn screen had an immunoreactive trypsinogen (IRT) value below the state cut-off value, so additional testing was not performed, and his growth trajectory appeared reassuring. He was ultimately diagnosed with CF by genetic testing and confirmatory sweat chloride testing, in the setting of his parents being known CF carriers and his severe presentation being clinically consistent with CF. Acutely, management with supplemental albumin, furosemide, potassium, and vitamin K was initiated to correct the presenting hypoalbuminemia, edema, and coagulopathy. Later, pancreatic enzyme supplementation and additional vitamins and minerals were added to manage ongoing deficiencies from pancreatic insufficiency. With appropriate treatment, his vitamin deficiencies and edema resolved, and his growth improved. Conclusion: Due to universal newborn screening, symptomatic presentation of CF is rare and presentation with kwashiorkor is extremely rare in resource-rich communities. The diagnosis of CF was delayed in our patient because of a normal newborn screen and falsely reassuring growth, which after diagnosis was determined to be secondary to severe edematous malnutrition. This case highlights that newborn screening is a useful but imperfect tool. Clinicians should continue to have suspicion for CF in the right clinical context, even in the setting of normal newborn screen results.

Digital Phenotype Generation and Time to Encounter Closure.

This study evaluates whether implementation of a content expert­developed clinical documentation tool can be beneficial to workflow by reducing time from patient arrival to encounter closure among pediatric patients receiving intestinal rehabilitation.

A retrospective review of newborn screening for congenital hypothyroidism and newborn thyroid disease at a major medical center.

Objective. To study the frequency of congenital hypothyroidism (CH)/thyroid disorders at a major, urban medical center. Methods. We conducted a retrospective review of a preexisting database for 2007 to 2011. Infants were classified as having CH, secondary/tertiary hypothyroidism, thyroid-binding globulin deficiency, and other types of newborn thyroid dysfunctions. Results. A total of 353 (50%) abnormal newborn screens were found to be normal and 42% were abnormal on repeat. Of the latter, 14% had true CH, 1% had thyroid-binding globulin deficiency, and 27% had other causes of thyroid dysfunction. The 5-year incidence of CH at NYP Morgan Stanley Children's Hospital was significantly greater than in New York City, New York State, and Upstate New York. Conclusion. The incidence of CH and other thyroid dysfunctions were greater in our population for 2007 to 2010, after which there was an unexplained decline. The study underlines the importance of continued newborn screening for thyroid dysfunction.