Philip K Frykman1, Sungjin Kim2, Tomas Wester3, Agneta Nordenskjöld3, Akemi Kawaguchi4, Thomas T Hui5, Daniel H Teitelbaum6, Anna L Granström3, Andre Rogatko2. 1. Division of Pediatric Surgery and Departments of Surgery and Biomedical Sciences, Cedars-Sinai Medical Center, Los Angeles, California, USA. Electronic address: Philip.Frykman@cshs.org. 2. Biostatistics and Bioinformatics Research Center at the Cedars-Sinai Samuel Oschin Comprehensive Cancer Institute, Los Angeles, California, USA. 3. Department of Pediatric Surgery, Astrid Lindgren's Children's Hospital, Karolinska University Hospital, Stockholm, Sweden; Department of Women's and Children's Health and Center for Molecular Medicine, Karolinska Institutet, Stockholm, Sweden. 4. Department of Pediatric Surgery, University of Texas Health Science Center Houston, Houston, TX, USA. 5. Division of Pediatric Surgery, University of California San Francisco Benioff Children's Hospital Oakland, Oakland, California, USA. 6. Division of Pediatric Surgery, C.S. Mott Children's Hospital, University of Michigan Medical School, Ann Arbor, MI, USA.
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.
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.
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