Identifying Risk Factors for Surgical Site Infection After Stoma Closure in Infants for Targeted Implementation of Surgical Site Infection Reduction Bundle.

Background: To determine risk factors for surgical site infection (SSI) in infants after stoma closure, to identify at-risk patients, plan timing of surgery, and implement SSI-reduction strategies. Patients and Methods: A single center retrospective comparison study of all children less than one year of age who underwent enterostomy closure (2018-2020) with SSI diagnosed through a prospective surveillance program, using criteria from Public Health England (PHE). Demographics and risk factors, types of SSI, systemic sepsis, mortality and length of stay were compared between SSI and non-SSI. Significant factors associated with SSI were analyzed in a multivariate binomial logistic regression model. Results: Eighty-nine stoma closures were performed, most commonly for necrotizing enterocolitis (NEC) and anorectal malformation. Fourteen had SSI (16%): 12 superficial and two deep; three developed systemic sepsis, but no 30-day mortality. Surgical site infection was associated with NEC (12/14 vs. 32/75; p = 0.003), younger age (median 76 vs. 89 days; p = 0.014), lower corrected gestation (cutoff: 39 weeks gestation; 11/14 vs. 27/75; p = 0.004) and lower weight (cutoff: 2.2 kg; 7/14 vs. 16/75; p = 0.032), compared with non-SSI. After correcting for age, gestation, and weight, logistic regression showed NEC was an independent predictor for SSI (odds ratio [OR], 12; 95% confidence interval [CI],1.2-125). The at-risk cohort (n = 56; 63%) had seven-fold increased risk of SSI and four-fold longer hospital stay, which may be the target for SSI-reduction strategies. Conclusions: Necrotizing enterocolitis-related stoma closure is at increased risk for SSI. Considerations for delaying stoma closure until achieving 39 weeks gestation or 2.2 kg in weight may further reduce SSI. Targeting SSI-reduction strategies using these criteria may improve resource-rationalization.

Exomphalos with intestinal fistulation: Case series and systematic review for clinical characterization, management and embryopathogenesis.

INTRODUCTION: Exomphalos with intestinal fistulation (EIF) is a rare variant of exomphalos with intestine opening to the surface of an intact sac, and may result in a diagnostic challenge. We report 3 new cases and conducted a systematic review of the literature, to characterize its association with the type of exomphalos and vitellointestinal duct (VI) as well as to evaluate its management and outcomes. METHODS: A literature search from PubMed using keywords pertaining to exomphalos and fistulation was used to identify all unique cases reported between 1950 and 2020, in addition to the case series reported here, to establish the clinical presentation, histological findings, management and outcomes. RESULTS: We found a total of 28 cases of EIF, of which 25 had been reported in 70 years from 19 reports. There was a male predominance (4-to-1 ratio). The majority presented as an exomphalos minor (n = 23, 82%) with a prolapsing patent VI duct (n = 16, 57%), most had evidence of Meckel's diverticulum (n = 25, 89%). All but one case were managed by fistula excision with or without ileal resection and anastomosis, followed by primary closure of the abdominal wall defect. All patients, except one with Trisomy 13 who received only palliative care, underwent surgery. Post-operative complications occurred in 7 patients (25%). Congenital anomalies were present in 12 (43%) and none had Beckwith-Wiedemann syndrome. Mortality occurred in 4 patients (14%) between 3 and 17 days. One EIF with exomphalos major failed early conservative treatment due to sac disintegration and sepsis, requiring staged closure, but had a good outcome. DISCUSSION: EIF is a rare entity usually associated with exomphalos minor and vitelline duct involvement. EIF presentation is variable but primary surgery is the mainstay of treatment with generally good outcomes. Common features of EIF suggest a different embryopathogenesis to other forms of exomphalos.

Primary low-grade fibromyxoid sarcoma of the kidney in a child with the alternative EWSR1-CREB3L1 gene fusion.

We present the case of a 6-year-old boy with a deceptively bland spindle cell renal neoplasm found to harbor the EWSR1-CREB3L1 gene fusion. This fusion has recently been described as a variant translocation in low-grade fibromyxoid sarcoma (LGFMS), a tumor more typically characterized by a recurrent t(7;16) chromosomal translocation, resulting in the fusion of FUS and CREB3L2 genes. LGFMS is an indolent tumor with late metastatic potential and a propensity for long-term disease recurrence. The tumor is rare in children, with only 33 published cases. In the pediatric population, it has not previously been reported arising in the kidney.