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.
Highlights
Hirschsprung-associated enterocolitis (HAEC) is a major contributor of mortality for patients with Hirschsprung disease (HSCR) [1, 2]
Several papers confirmed that the HAEC scoring system with cut-off of 4 increase the sensitivity, which is better for screening purposes [15, 17]
We found that patients with anemia had a higher risk of HAEC after pull-through than patients with normal hemoglobin level with OR of 3.77 when we used cut-off value of ≥4 (Table 3)
Summary
Hirschsprung-associated enterocolitis (HAEC) is a major contributor of mortality for patients with Hirschsprung disease (HSCR) [1, 2]. Frykman et al [16] performed a critical univariate and multivariate application on those scoring criteria, clustered some of the criteria and concluded that a score ≥4 optimized sensitivity and specificity They proposed a score ≥4 as diagnostic of HAEC [16]. We compared the HAEC frequency after TEPT using two cut-offs of scoring system and associated them with the risk factors, including sex, age at HSCR diagnosis and TEPT performed, nutritional status, hemoglobin and albumin level, gestational age, mother’s age during pregnancy, and maternal education level. 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
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