Comparison of Two Different Cut-Off Values of Scoring System for Diagnosis of Hirschsprung-Associated Enterocolitis After Transanal Endorectal Pull-Through

Abstract

Background: Hirschsprung-associated enterocolitis (HAEC) is a major contributor in the mortality of Hirschsprung disease (HSCR) patients that can occur both preoperatively and post-operatively. Several cut-off values of HAEC score have been used, i.e., ≥10 and ≥4. Here, we compared the HAEC frequency after transanal endorectal pull-through (TEPT) using two cut-offs of scoring system and associated them with the risk factors.Methods: Cross-sectional analysis was conducted using medical records of HSCR patients who were aged ≤18 years old and underwent TEPT at our institution, Indonesia between 2009 and 2016. HAEC was determined using the scoring system with cut-off values of ≥10 and ≥4.Results: Seventy subjects were used in the final analysis, consisting of 44 males and 26 females. There was a significant difference in one HAEC finding between the ≥10 and ≥4 cut-off groups; diarrhea with explosive stools (p = 0.002). The HAEC frequency was 5/70 (7.1%) and 49/70 (70%) patients using cut-off values of ≥10 and ≥4 (p < 0.0001), respectively. We found that patients with anemia (i.e., iron deficiency anemia) had a higher risk of HAEC after TEPT than patients with normal hemoglobin level with OR of 3.77 (95% CI = 1.28–11.1; p = 0.027), while no associations were found between other variables, including sex, age at diagnosis, age at definitive therapy, albumin level, and nutritional status and HAEC following TEPT (p = 0.87, 0.15, 0.33, 0.26, and 0.60, respectively). Also, no associations were observed between maternal education level, mother's age at pregnancy and gestational age and HAEC after definitive surgery (p = 0.10, 0.46, and 0.86, respectively).Conclusions: This report is the first study comparing two different cut-off values of scoring system to evaluate the HAEC frequency after TEPT and results suggest further using cut-off of ≥4 to expand the diagnosis of HAEC. Moreover, we also show for the first time that hemoglobin level is a strong risk factor for the HAEC development after TEPT.

Surgical and clinical determinants of postoperative Hirschsprung‐associated enterocolitis: Multivariate analysis in a large cohort

“…A retrospective study reported by Du Juan 36 also indicated that malnutrition is a risk factor for the occurrence of enterocolitis in children with congenital megacolon after surgery. A recent retrospective study 37 identified microcytic hypochromic anemia as a risk factor for postoperative HAEC, but our cohort study did not find a significant association between hemoglobin levels and postoperative HAEC. In this study, other markers such as CRP, PCT, and hemoglobin did not show a significant association in regression analysis, consistent with the conclusions of our study.…”

Bowel Management in Hirschsprung Disease—Pre-, Peri- and Postoperative Care for Primary Pull-Through

“…The definition used in this study for Hirschsprung-associated enterocolitis (HAEC) is the Frykman modification of the Pastor score, with a cut-off value of 4 [1,4]. The HAEC score contains information on patient history, physical and radiological examination, and laboratory results, with a maximum value of 20 [1,4].…”

“…Approximately 30% of HD patients will experience at least one episode of HAEC. Note, however, that there is currently no consent on the exact definition of HAEC [1,4].…”

Prognostic factors for persistent obstructive symptoms in patients with Hirschsprung disease following pull-through

“…These aspects should be considered while interpreting our findings. In addition, we have not included enterocolitis as persistent obstructive symptoms since we have already specifically determined the prognostic factors for enterocolitis in our previous studies [23,24].…”