High Rate of Venous Thromboembolism in Severe Pediatric Intestinal Failure.

OBJECTIVE: To quantify the rate of venous thromboembolism (VTE) in patients with pediatric intestinal failure and identify associated risk factors. STUDY DESIGN: We performed a retrospective cohort study in pediatric patients (<21 years old) with severe pediatric intestinal failure (≥90 consecutive days of parenteral nutrition) secondary to short bowel syndrome who were treated from 2014 to 2021 at an interdisciplinary intestinal rehabilitation program. The primary outcome was the incidence of VTE. Multivariable regression was performed to identify independent clinical predictors of VTE. RESULTS: A total of 263 patients (59.7% male) met the criteria for inclusion. The cumulative incidence of VTE was 28.1%, with a rate of 0.32 VTEs per 1000 catheter-days. On univariate analysis, the number of catheter days, number of catheters, and history of central line-associated blood stream infection were associated with VTE. On multivariable logistic regression, a higher number of catheters was an independent risk factor for VTE (aOR, 1.17; 95% CI, 1.06-1.29). Additionally, earlier gestational age was a risk factor for VTE such that every week decrease in gestational age conferred a 9% increased risk of VTE (aOR, 1.09; 95% CI, 1.02-1.16). CONCLUSIONS: In this retrospective study, 28.1% of patients with severe pediatric intestinal failure developed VTE; the number of catheters and early gestational age were noted to be independent risk factors for VTE. This high incidence of VTE highlights the need to investigate VTE in pediatric intestinal failure prospectively, including the potential benefit of prophylactic anticoagulation.

Patient-perceived barriers to surgical treatment of cleft lip and palate in Brazil: A multi-region study.

INTRODUCTION: Many patients worldwide are unable to access timely primary repair of cleft lip and palate. The aim of this study was to assess patient-perceived barriers to accessing timely cleft lip and palate repair across Brazil. METHODS: A 29-item questionnaire was applied to patients undergoing surgery for cleft lip and/or palate across five contrasting sites in Brazil from February 2016 to November 2017. Differences in patient timelines, demographics, and patient-reported barriers were compared by region. A multivariate logistic regression was used to determine predictors of delayed care. RESULTS: Of 181 patients, 42% of patients received timely primary surgical repair. The age of the patient at the interview was 82 months (standard deviation [SD] 107) and 52% were male. The majority of delays occurred between diagnosis and primary surgical repair. The mean number of barriers to accessing timely surgical care cited by each patient was 3.77. The most common barrier was perceived "lack of hospitals that provided the surgery in my area" (48% (n = 86)). Univariate logistic regression showed increased odds of receiving late care in the state of Amazonas (odds ratio [OR] 2.91; 95% confidence interval [CI] 1.07-7.96; P = 0.037) or Para (OR 4.46; 95% CI 1.09-19.70; P = 0.037). Multivariate logistic regression determined predictors of delayed care to be female sex (OR = 2.05; 95% CI 1.05-3.99; P = 0.035) and perceived poor availability of care (OR = 0.045; 95% CI 1.02-4.37; P = 0.045). CONCLUSION: The majority of patients in Brazil are not receiving timely primary repair of their clefts. Improvements in the coordination of care, patient education and patient empowerment are required.

The Role of Chronic Conditions in Outcomes following Noncardiac Surgery in Children with Congenital Heart Disease.

OBJECTIVES: To compare outcomes in children with congenital heart disease (CHD) undergoing noncardiac surgery by presence of chronic conditions and identify associated risk factors. STUDY DESIGN: Retrospective analysis of 14 031 children with CHD who underwent noncardiac surgery in the 2016 Healthcare Cost and Utilization Project Kid's Inpatient Database. Multivariable regression was used to assess patient and hospital factors associated with in-hospital mortality and length of stay (LOS). RESULTS: Overall, 94% had at least 1 chronic condition. The in-hospital mortality rate was 5.6%. Neonates with CHD only had the highest mortality (15.6%); otherwise, children with CHD and at least 1 chronic condition had higher mortality than patients with CHD only (infant 3.93%, child 1.22%, adolescent 1.04% vs 2.34%, 0%, and 0%). Neonates (OR, 15.5; 95% CI, 7.1-34.1 vs adolescent), number of chronic conditions (OR, 1.34; 95% CI, 1.27-1.42), chronic conditions type (circulatory system; OR 2.46; 95% CI, 2.04-2.98), and low socioeconomic status (OR, 1.36; 95% CI, 1.05-1.77) were associated with increased mortality. The median LOS was 20 days (IQR, 5-66). Those with CHD and at least 1 chronic condition had a greater LOS (21 days; IQR, 5-68) than those with CHD only (9 days; IQR, 3-46). Neonates (adjusted coefficient, 44.3; 95% CI, 40.3-48.3 vs adolescent), Black race (adjusted coefficient, 4.78; 95% CI, 2.27-7.3), chronic condition indicator number (adjusted coefficient, 5.17; 95% CI, 4.56-5.78), and subtype (adjusted coefficient, 23.6; 95% CI, 20.4-26.7) were associated with a prolonged LOS. CONCLUSIONS: Most children with CHD who undergo noncardiac surgery have at least 1 chronic condition. Age, chronic conditions type and number, low socioeconomic status, and Black race impart increased risks of in-hospital mortality and prolonged LOS. Further research is needed to evaluate the impact of specific chronic conditions and determine barriers to equitable care.

Feeding and Growth Outcomes in Infants with Type C Esophageal Atresia Who Undergo Early Primary Repair.

OBJECTIVES: To describe growth and feeding outcomes in patients with type C esophageal atresia who underwent early primary repair and to identify predictors for poor growth. STUDY DESIGN: This single-center, retrospective, cohort study included all patients with type C esophageal atresia who underwent early primary repair from 2013 to 2019. Weight-for-age z score (WAZ) was calculated at birth, and every 6 months until 3 years postoperatively. Longitudinal median regression was used to evaluate WAZ over time. A multivariable logistic regression model explored predictors of growth outcomes. RESULTS: Of 46 infants who met the inclusion criteria, 72% were term. The median age at repair was 1.5 days of life (IQR, 1-2 days of life) and the hospital length of stay was 20 days (IQR-14, 30 days). Two patients had esophageal leak (4.3%). The median WAZ at birth was below average (-0.72; IQR, -1.37 to -0.40), but improved to reach average by 3 years (-0.025; IQR, -0.85 to 0.97, P < .001). At discharge, 72% of patients were receiving full oral nutrition, which improved to 95% by 3 years. The only independent predictor of poor growth at 1 year (WAZ < -1 [33%]) was WAZ at discharge (P = .02). CONCLUSIONS: Infants with esophageal atresia who undergo early primary repair are capable of achieving standard growth curves by 3 years of age. However, poor discharge WAZ score was predictive of poor WAZ score at 1 year. Efforts to identify at-risk patients and institute targeted inpatient and outpatient nutrition interventions are needed to improve their growth trajectory.

Longitudinal Analysis of Ventilation Perfusion Mismatch in Congenital Diaphragmatic Hernia Survivors.

OBJECTIVE: To determine the natural history of pulmonary function for survivors of congenital diaphragmatic hernia (CDH). STUDY DESIGN: This was a retrospective cohort study of survivors of CDH born during 1991-2016 and followed at our institution. A generalized linear model was fitted to assess the longitudinal trends of ventilation (V), perfusion (Q), and V/Q mismatch. The association between V/Q ratio and body mass index percentile as well as functional status was also assessed with a generalized linear model. RESULTS: During the study period, 212 patients had at least one V/Q study. The average ipsilateral V/Q of the cohort increased over time (P < .01), an effect driven by progressive reduction in relative perfusion (P = .012). A higher V/Q ratio was correlated with lower body mass index percentile (P < .001) and higher probability of poor functional status (New York Heart Association class III or IV) (P = .045). CONCLUSIONS: In this cohort of survivors of CDH with more severe disease characteristics, V/Q mismatch worsens over time, primarily because of progressive perfusion deficit of the ipsilateral side. V/Q scans may be useful in identifying patients with CDH who are at risk for poor growth and functional status.