Lewit Ruth A, Veras Laura V, Cowles Robert A, Fowler Kathryn, King Sebastian, Lapidus-Krol Eveline, Langer Jacob C, Park Christine J, Youssef Fouad, Vavilov Sergey, Gosain Ankush
Division of Pediatric Surgery, Le Bonheur Children's Hospital, University of Tennessee Health Science Center, Memphis, Tennessee.
Division of Pediatric Surgery, Department of Surgery, Yale University School of Medicine, New Haven, Connecticut.
J Surg Res. 2021 May;261:253-260. doi: 10.1016/j.jss.2020.12.030. Epub 2021 Jan 15.
Hirschsprung-Associated Enterocolitis (HAEC) is a life-threatening and difficult to diagnose complication of Hirschsprung Disease (HSCR). The goal of this study was to evaluate existing HAEC scoring systems and develop a new scoring system.
Retrospective, multi-institutional data collection was performed. For each patient, all encounters were analyzed. Data included demographics, symptomatology, laboratory and radiographic findings, and treatments received. A "true" diagnosis of HAEC was defined as receipt of treatment with rectal irrigations, antibiotics, and bowel rest. The Pastor and Frykman scoring systems were evaluated for sensitivity/specificity and univariate and multivariate logistic regression performed to create a new scoring system.
Four centers worldwide provided data on 200 patients with 1450 encounters and 369 HAEC episodes. Fifty-seven percent of patients experienced one or more episodes of HAEC. Long-segment colonic disease was associated with a higher risk of HAEC on univariate analysis (OR 1.92, 95% CI 1.43-2.57). Six variables were significantly associated with HAEC on multivariate analysis. Using published diagnostic cutoffs, sensitivity/specificity for existing systems were found to be 38.2%/96% for Pastor's and 56.4%/86.9% for Frykman's score. A new scoring system with a sensitivity/specificity of 67.8%/87.9% was created by stepwise multivariate analysis. The new score outperformed the existing scores by decreasing underdiagnosis in this patient cohort.
Existing scoring systems perform poorly in identifying episodes of HAEC, resulting in significant underdiagnosis. The proposed scoring system may be better at identifying those underdiagnosed in the clinical setting. Head-to-head comparison of HAEC scoring systems using prospective data collection may be beneficial to achieve standardization in the field.
先天性巨结肠相关小肠结肠炎（HAEC）是先天性巨结肠症（HSCR）的一种危及生命且难以诊断的并发症。本研究的目的是评估现有的HAEC评分系统并开发一种新的评分系统。
进行回顾性、多机构数据收集。对每位患者的所有就诊情况进行分析。数据包括人口统计学、症状学、实验室和影像学检查结果以及接受的治疗。HAEC的“确诊”定义为接受直肠灌洗、抗生素治疗和肠道休息。对Pastor和Frykman评分系统进行敏感性/特异性评估，并进行单因素和多因素逻辑回归以创建新的评分系统。
全球四个中心提供了200例患者的1450次就诊情况和369次HAEC发作的数据。57%的患者经历过一次或多次HAEC发作。单因素分析显示长段结肠疾病与HAEC风险较高相关（OR 1.92，95%CI 1.43 - 2.57）。多因素分析发现六个变量与HAEC显著相关。根据已发表的诊断临界值，现有系统的敏感性/特异性在Pastor评分中为38.2%/96%，在Frykman评分中为56.4%/86.9%。通过逐步多因素分析创建了一个敏感性/特异性为67.8%/87.9%的新评分系统。在该患者队列中，新评分通过减少漏诊优于现有评分。
现有评分系统在识别HAEC发作方面表现不佳，导致大量漏诊。所提出的评分系统可能更擅长识别临床环境中漏诊的病例。使用前瞻性数据收集对HAEC评分系统进行直接比较可能有助于该领域实现标准化。
