D. R. Halleran1,2, H. Ahmad1, E. Maloof1, M. M. Paradiso1, H. Lehmkuhl1, J. Wu1, P. C. Minneci2, M. A. Levitt1, R. J. Wood1 1Nationwide Children’s Hospital,Center For Colorectal And Pelvic Reconstruction,Columbus, OH, USA 2Nationwide Children’s Hospital,Division Of Pediatric Surgery,Columbus, OH, USA
Introduction: Hirschsprung associated enterocolitis (HAEC) is a potentially life-threatening condition seen in patients with Hirschsprung disease (HD). Although children with Trisomy 21 (T21) are reported in the literature to experience a higher incidence of HAEC, whether their individual episodes are more severe has not been previously evaluated. The objective of this study was to compare episodes of HAEC between these groups.
Methods: We performed a single institution retrospective cohort study of patients with HD admitted to the hospital with HAEC between 2009 and 2017. Patients were divided into two groups based on an associated diagnosis of T21. HAEC was defined as episodes in which patients presented with fever from a presumed colonic source, abdominal distension, or diarrhea and received antibiotic treatment. Patient characteristics, HAEC score, and clinical outcomes were compared using t-tests for continuous variables and Chi-squared or Fisher exact tests for categorical variables.
Results: Of 86 admissions for enterocolitis over the study period, 12 (14%) were for patients with T21. Patients with and without T21 and enterocolitis were similar in terms of age (3.0 vs. 2.6 years, p=0.72), male gender (58% vs. 77%, p=0.17), proportion with disease proximal to the left colon (9% vs. 12%, p=0.70) history of 1 or more episodes of enterocolitis (67% vs. 53%, p=0.39), and proportion of patients whose HAEC episode occurred after their pull through (92% vs. 86%, p=0.62) (Table 1). At presentation, the presence of fever (17% vs. 12%, p=0.67), abdominal distention (75% vs. 76%, p=0.96), diarrhea (58% vs. 57%, p=0.86), and WBC count (10.0 vs. 12.2, p=0.18) were similar between both groups. The HAEC score was higher in patients with T21 (7.1 vs. 5.6, p=0.05). Patients with T21 were more likely to require ICU admission (58% vs. 12%, p=0.006) and receive a longer course of antibiotics (13 days vs. 7 days, p=0.02). The length of stay was similar between both groups (5.5 vs. 4.8 days, p=0.34). There were no deaths related to HAEC over the study period.
Conclusion: Patients with T21 experience more severe episodes of HAEC. Based on these results, patients with T21 and Hirschsprung disease should receive targeted education and more aggressive care plans to avoid and treat HAEC.
