Abstract
Purpose
This study aims to characterize risk factors for Hirschsprung-associated enterocolitis
(HAEC). We hypothesize that earlier pull-through surgery is associated with lower
risks of developing postoperative HAEC.
Methods
A comparative study of 171 Hirschsprung patients treated from 1990 to 2017 was performed.
Patients without HAEC were compared to patients with preoperative and/or postoperative
HAEC. Results are presented as median [IQR] or frequency (%). Pearson's χ2 test and Wilcoxon rank sum test were performed with a significance level at p < 0.05.
Multivariable logistic regression analysis was used to adjust for potential confounders.
A subanalysis was done to evaluate laparoscopic, laparotomy, and transanal surgeries.
Results
Risk of developing preoperative HAEC was significantly associated with congenital
malformations (OR 2.63 [1.11, 6.24]; p = 0.02). Birth weight was lower in patients
with preoperative HAEC (OR 0.48 [95% CI 0.25, 0.93]; p = 0.03). On regression analysis,
intestinal obstruction after surgery was significantly associated with postoperative
HAEC (OR 8.2 [3.18, 21.13]; p < 0.0001). Patients with earlier pull-through surgery
did not have a lower risk of developing postoperative HAEC.
Conclusions
Timing of surgery does not seem to be associated with a higher risk of developing
pre- and postoperative HAEC. Predisposing factors for preoperative HAEC included associated
malformations and lower birth weight, whereas intestinal obstruction was found to
be associated with postoperative HAEC.
Type of study
Treatment study.
Level of evidence
Level III.
Abbreviations:
HSCR (Hirschsprung disease), HAEC (Hirschsprung-associated enterocolitis), IQR (interquartile range), N/A (not available), AIC (Akaike information criterion)Key words
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References
- Hirschsprung disease, associated syndromes, and genetics: a review.J Med Genet. 2001; 38: 729-739
- Development of a standardized definition for Hirschsprung's-associated enterocolitis: a Delphi analysis.J Pediatr Surg. 2009; 44: 251-256
- Enterocolitis complicating Hirschsprung's disease.Semin Pediatr Surg. 2004; 13: 263-272
- Hirschsprung-associated enterocolitis: prevention and therapy.Semin Pediatr Surg. 2012; 21: 328-335
- Mortality in Swedish patients with Hirschsprung disease.Pediatr Surg Int. 2017; 33: 1177-1181
- Hirschsprung's disease: what about mortality?.Pediatr Surg Int. 2011; 27: 473-478
- Hirschsprung disease: do risk factors of poor surgical outcome exist?.J Pediatr Surg. 2008; 43: 612-619
- Long-term outcome of patients with enterocolitis complicating Hirschsprung's disease.Pediatr Surg Int. 2006; 22: 316-318
- Established and emerging concepts in Hirschsprung's-associated enterocolitis.Pediatr Surg Int. 2016; 32: 313-320
- Enterocolitis after the surgical treatment of Hirschsprung's disease: risk factors and financial impact.J Pediatr Surg. 1998; 33: 830-833
- The pathogenesis of Hirschsprung's disease-associated enterocolitis.Semin Pediatr Surg. 2012; 21: 319-327
- Novel insights into the pathogenesis of Hirschsprung's-associated enterocolitis.Chin Med J (Engl). 2016; 129: 1491-1497
- New insights into the pathogenesis of Hirschsprung's associated enterocolitis.Pediatr Surg Int. 2005; 21: 773-779
- Older age at diagnosis of Hirschsprung disease decreases risk of postoperative enterocolitis, but resection of additional ganglionated bowel does not.J Pediatr Surg. 2008; 43: 1115-1123
- Clinical impacts of delayed diagnosis of Hirschsprung's disease in newborn infants.Pediatr Neonatol. 2012; 53: 133-137
- Enterocolitis causes profound lymphoid depletion in endothelin receptor B- and endothelin 3-null mouse models of Hirschsprung-associated enterocolitis.Eur J Immunol. 2015; 45: 807-817
- Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention.Pediatr Surg Int. 2013; 29: 873-881
- Enterocolitis in Hirschsprung's disease.Pediatr Surg Int. 1992; 7: 356-360
- Hirschsprung's disease. Identification of risk factors for enterocolitis.Ann Surg. 1988; 207: 240-244
- Enterocolitis and bowel function in children with Hirschsprung disease and trisomy 21.J Pediatr Surg. 2016; 51: 2001-2004
- The pathophysiology of Hirschsprung's-associated enterocolitis: importance of histologic correlates.J Pediatr Surg. 1989; 24: 1271-1277
- Persistent obstructive symptoms after surgery for Hirschsprung's disease: development of a diagnostic and therapeutic algorithm.J Pediatr Surg. 2004; 39: 1458-1462
- Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis.Pediatr Surg Int. 2017; 33: 517-521
Article info
Publication history
Published online: February 24, 2019
Accepted:
January 27,
2019
Received:
January 16,
2019
Footnotes
☆Declarations of interest: None.
☆☆This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Identification
Copyright
© 2019 Elsevier Inc. All rights reserved.
