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Critical evaluation of the Hirschsprung-associated enterocolitis (HAEC) score: A multicenter study of 116 children with Hirschsprung disease.

ABSTRACT: OBJECTIVE:To identify the optimal clinical criteria to diagnose Hirschsprung-associated enterocolitis (HAEC) in children with Hirschsprung disease (HSCR). BACKGROUND:HAEC is the most common life-threatening complication in HSCR patients, yet the diagnostic criteria for HAEC remain unclear. The consensus-based HAEC scoring system was not validated using patient data, thereby making its diagnostic accuracy uncertain. METHODS:From 2009 to 2015, consecutive children with HSCR underwent retrospective evaluation of their medical records, and questionnaire-directed parent interviews to identify treatment of suspected HAEC episodes and the 16 clinical criteria in the HAEC score. Logistic regression modeling was employed to identify criteria predicting suspected HAEC episodes. RESULTS:One hundred sixteen HSCR patients met inclusion criteria, 43 patients (37.1%) were treated for at least one suspected HAEC episode. An HAEC score of 4 maximized the sum of sensitivity (83.7%) and specificity (98.6%) while the previously established cut-off score of 10 showed lower sensitivity (41.9%) with perfect specificity. Multivariable analysis identified four criteria utilized to create a new HAEC Risk score with performance characteristics similar to the HAEC score cutoff of 4. CONCLUSION:When using the HAEC score, a cutoff of 4 should be used rather than 10, which under-diagnosed patients with HAEC. Alternatively, the new HAEC Risk score could be employed. LEVEL OF EVIDENCE:Diagnostic Study, Level 3.

SUBMITTER: Frykman PK

PROVIDER: S-EPMC6247908 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature

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Critical evaluation of the Hirschsprung-associated enterocolitis (HAEC) score: A multicenter study of 116 children with Hirschsprung disease.

Frykman Philip K PK Kim Sungjin S Wester Tomas T Nordenskjöld Agneta A Kawaguchi Akemi A Hui Thomas T TT Teitelbaum Daniel H DH Granström Anna L AL Rogatko Andre A

Journal of pediatric surgery 20170714 4

<h4>Objective</h4>To identify the optimal clinical criteria to diagnose Hirschsprung-associated enterocolitis (HAEC) in children with Hirschsprung disease (HSCR).<h4>Background</h4>HAEC is the most common life-threatening complication in HSCR patients, yet the diagnostic criteria for HAEC remain unclear. The consensus-based HAEC scoring system was not validated using patient data, thereby making its diagnostic accuracy uncertain.<h4>Methods</h4>From 2009 to 2015, consecutive children with HSCR u ...[more]

PMID: 28760457

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Project description:BackgroundThe Coronavirus Disease 2019 pandemic provided a natural experiment to study the effect of social distancing on the risk of developing Hirschsprung's Associated Enterocolitis (HAEC).MethodsUsing the Pediatric Health Information System (PHIS), a retrospective cohort study of children (<18 years) with Hirschsprung's Disease (HSCR) across 47 United States children's hospitals was performed. The primary outcome was HAEC admissions per 10,000 patient-days. The exposure (COVID-19) was defined as April 2020-December 2021. The unexposed (historical control) period was April 2018-December 2019. Secondary outcomes included sepsis, bowel perforation, intensive care unit (ICU) admission, mortality, and length of stay.ResultsOverall, we included 5707 patients with HSCR during the study period. There were 984 and 834 HAEC admissions during the pre-pandemic and pandemic periods, respectively (2.6 vs. 1.9 HAEC admissions per 10,000 patient-days, incident rate ratio [95% confidence interval]: 0.74 [0.67, 0.81], p < 0.001). Compared to pre-pandemic, those with HAEC during the pandemic were younger (median [IQR]: 566 [162, 1430] days pandemic vs. 746 [259, 1609] days pre-pandemic, p < 0.001) and more likely to live in the lowest quartile of median household income zip codes (24% pandemic vs. 19% pre-pandemic, p = 0.02). There were no significant differences in rates of sepsis (6.1% pandemic vs. 6.1% pre-pandemic, p > 0.9), bowel perforation (1.3% pandemic vs. 1.2% pre-pandemic, p = 0.8), ICU admissions (9.6% pandemic vs. 12% pre-pandemic, p = 0.2), mortality (0.5% pandemic vs. 0.6% pre-pandemic, p = 0.8), or length of stay (median [interquartile range]: 4 [(Pastor et al., 2009; Gosain and Brinkman, 2015) 2,112,11 days pandemic vs. 5 [(Pastor et al., 2009; Tang et al., 2020) 2,102,10 days pre-pandemic, p = 0.4).ConclusionsThe COVID-19 pandemic was associated with significantly decreased incidence of HAEC admissions across US children's hospitals. Possible etiologies such as social distancing should be explored.Level of evidenceII.

Dataset Information

Critical evaluation of the Hirschsprung-associated enterocolitis (HAEC) score: A multicenter study of 116 children with Hirschsprung disease.

Publications

Critical evaluation of the Hirschsprung-associated enterocolitis (HAEC) score: A multicenter study of 116 children with Hirschsprung disease.

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