Comparison of pre-operative Hirschsprung-associated enterocolitis using classical criteria and Delphi method: A diagnostic study

Background: Hirschsprung-associated enterocolitis (HAEC) is the most common complication of Hirschsprung
disease (HSCR) that may happen pre-operatively. Several methods have been reported to determine HAEC.
Because the diagnosis of pre-operative HAEC might change the surgical plan, we aimed to determine the accuracy
of the classical criteria for diagnosis of pre-operative HAEC and using the Delphi method as a gold
standard.
Methods: Medical records of HSCR children who were admitted to our hospital from January 2009 to December
2015 were retrospectively analyzed.
Results: Ninety-six subjects were involved in this study, consisting of 74 males and 22 females. The most
common findings of the Delphi score were abdominal distension (100%) and dilated loops of bowel (100%),
followed by leucocytosis (78.6%), lethargy (71.4%), cutoff sign in rectosigmoid with absence of distal air
(71.4%), and shift to left (71.4%). The frequency of pre-operative HAEC was 4.2% and 14.6% using the classical
criteria and Delphi method, respectively (p = 0.016). The sensitivity, specificity, positive predictive value,
negative predictive value, and accuracy rates of the classical criteria for diagnosis of pre-operative HAEC were
14.3% (95% CI: 1.8–42.8%), 97.6% (95% CI: 91.5–99.7%), 50% (95% CI: 13.3–86.7%), 87% (95% CI:
84.3–89.2), and 85.4% (95% CI: 76.7–91.8%), respectively.
Conclusions: The frequency of pre-operative HAEC is low in our hospital. The accuracy of the classical criteria is
considered relatively moderate for diagnosis of pre-operative HAEC.