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

Abstract

<div class="section"> <a class="named-anchor" id="S1"> <!-- named anchor --> </a> <h5 class="section-title" id="d3006285e209">Objective:</h5> <p id="P1">To identify the optimal clinical criteria to diagnose Hirschsprung-associated enterocolitis (HAEC) in children with Hirschsprung disease (HSCR). </p> </div><div class="section"> <a class="named-anchor" id="S2"> <!-- named anchor --> </a> <h5 class="section-title" id="d3006285e214">Background:</h5> <p id="P2">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. </p> </div><div class="section"> <a class="named-anchor" id="S3"> <!-- named anchor --> </a> <h5 class="section-title" id="d3006285e219">Methods:</h5> <p id="P3">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. </p> </div><div class="section"> <a class="named-anchor" id="S4"> <!-- named anchor --> </a> <h5 class="section-title" id="d3006285e224">Results:</h5> <p id="P4">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 spec-ificity. Multivariable analysis identified four criteria utilized to create a new HAEC Risk score with performance characteristics similar to the HAEC score cutoff of 4. </p> </div><div class="section"> <a class="named-anchor" id="S5"> <!-- named anchor --> </a> <h5 class="section-title" id="d3006285e229">Conclusion:</h5> <p id="P5">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. </p> </div><div class="section"> <a class="named-anchor" id="S6"> <!-- named anchor --> </a> <h5 class="section-title" id="d3006285e234">Level of Evidence:</h5> <p id="P6">Diagnostic Study, Level 3.</p> </div>