Abstract
BackgroundHirschsprung-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.MethodsMedical records of HSCR children who were admitted to our hospital from January 2009 to December 2015 were retrospectively analyzed.ResultsNinety-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.ConclusionsThe 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.
Highlights
Hirschsprung disease (HSCR) is a complex genetic disorder that is caused by the absence of intramural ganglion cells in the Auerbach and Meissner plexus, resulting in a functional obstruction of the colon [1]
Based on the Delphi method, 14/96 (14.6%) of the children had Hirschsprung-associated enterocolitis (HAEC) before the surgery with a mean score of 10.79 ± 1.85. Among those diagnosed as HAEC with the Delphi method, eleven were male and three were female while only one of them had long type aganglionosis (Table 1)
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%, 97.6%, 50%, 87%, and 85.4%, respectively (Table 3)
Summary
Hirschsprung disease (HSCR) is a complex genetic disorder that is caused by the absence of intramural ganglion cells in the Auerbach and Meissner plexus, resulting in a functional obstruction of the colon [1]. The classical diagnostic criteria were formerly used to diagnose HAEC [3], which are based on the clinical presentations that are most often seen in the clinical setting, such as abdominal distension (83%), explosive diarrhea (69%), vomiting (51%), fever (34%), lethargy (27%) rectal bleeding (5%) and colonic perforation (2.5%) [3]. A child is diagnosed with HAEC when there is abdominal distension, explosive diarrhea and cut-off appearance in the radiological analysis [3]. Hirschsprung-associated enterocolitis (HAEC) is the most common complication of Hirschsprung disease (HSCR) that may happen pre-operatively. 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. The accuracy of the classical criteria is considered relatively moderate for diagnosis of pre-operative HAEC
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