Eur J Pediatr Surg 2021; 31(03): 207-213
DOI: 10.1055/s-0040-1716876
Original Article

Comparison of Hirschsprung Disease Characteristics between Those with a History of Postoperative Enterocolitis and Those without: Results from the Pediatric Colorectal and Pelvic Learning Consortium

1   Department of Surgery, University of Utah School of Medicine and Primary Children's Hospital, Salt Lake City, Utah, United States
,
Brian T. Bucher
1   Department of Surgery, University of Utah School of Medicine and Primary Children's Hospital, Salt Lake City, Utah, United States
,
Ron W. Reeder
2   Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, Utah, United States
,
Jeffrey R. Avansino
3   Department of Pediatric Surgery, Seattle Children's, Seattle, Washington, United States
,
Megan Durham
4   Division of Pediatric Surgery, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, Georgia, United States
,
Casey M. Calkins
5   Department of Pediatric Surgery, Children's Wisconsin, Milwaukee, Wisconsin, United States
,
Richard J. Wood
6   Department of Pediatric Colorectal and Pelvic Reconstructive Surgery, Nationwide Children's Hospital, Columbus, Ohio, United States
,
Marc A. Levitt
7   Department Colorectal and Pelvic Reconstructive Surgery, Children's National Hospital, District of Columbia, Washington, United States
,
Kaylea Drake
2   Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, Utah, United States
,
Michael D. Rollins
1   Department of Surgery, University of Utah School of Medicine and Primary Children's Hospital, Salt Lake City, Utah, United States
› Author Affiliations

Abstract

Introduction The current understanding of Hirschsprung-associated enterocolitis (HAEC) is based mainly on single-center, retrospective studies. The aims of this study are to determine risk factors for postoperative HAEC using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) database.

Materials and Methods We performed a multicenter, retrospective, case–control study of children with Hirschsprung disease (HD) who had undergone a pull-through procedure and were evaluated at a PCPLC member site between February 2017 and March 2020. The cohort with a history of postoperative HAEC was compared with that without postoperative episodes of HAEC to determine relevant associations with postoperative HAEC.

Results One-hundred forty of 299 (46.8%) patients enrolled had a history of postoperative HAEC. Patients with a rectosigmoid transition zone had a lower association with postoperative HAEC as compared with those with a more proximal transition zone (odds ratio [OR]: 0.46, 95% confidence interval [CI]: 0.26, 0.84, p < 0.01). Private insurance was protective against postoperative HAEC on univariate analysis (OR: 0.62, 95% CI: 0.38, 0.99, p = 0.047), but not on multivariate analysis (OR: 0.62, 95% CI: 0.37, 1.04, p = 0.07). Preoperative HAEC was not associated with the development of postoperative HAEC.

Conclusion Patients with a rectosigmoid transition zone have less postoperative HAEC compared with patients with a more proximal transition zone. Multi-institutional collection of clinical information in patients with HD may allow for the identification of additional risk factors for HAEC and afford the opportunity to improve care.



Publication History

Received: 06 May 2020

Accepted: 16 August 2020

Article published online:
18 September 2020

© 2020. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 
  • References

  • 1 Bjørnland K, Pakarinen MP, Stenstrøm P. Nordic Pediatric Surgery Study Consortium. et al. A Nordic multicenter survey of long-term bowel function after transanal endorectal pull-through in 200 patients with rectosigmoid Hirschsprung disease. J Pediatr Surg 2017; 52 (09) 1458-1464
  • 2 Langer JC, Rollins MD, Levitt M. American Pediatric Surgical Association Hirschsprung Disease Interest Group. et al. Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease. Pediatr Surg Int 2017; 33 (05) 523-526
  • 3 Soh HJ, Nataraja RM, Pacilli M. Prevention and management of recurrent postoperative Hirschsprung's disease obstructive symptoms and enterocolitis: systematic review and meta-analysis. J Pediatr Surg 2018; 53 (12) 2423-2429
  • 4 Pastor AC, Osman F, Teitelbaum DH, Caty MG, Langer JC. Development of a standardized definition for Hirschsprung's-associated enterocolitis: a Delphi analysis. J Pediatr Surg 2009; 44 (01) 251-256
  • 5 Frykman PK, Kim S, Wester T. HAEC Collaborative Research Group (HCRG). et al. Critical evaluation of the Hirschsprung-associated enterocolitis (HAEC) score: a multicenter study of 116 children with Hirschsprung disease. J Pediatr Surg 2018; 53 (04) 708-717
  • 6 Dore M, Vilanova Sanchez A, Triana Junco P. et al. Reliability of the Hirschsprung-associated enterocolitis score in clinical practice. Eur J Pediatr Surg 2019; 29 (01) 132-137
  • 7 Le-Nguyen A, Righini-Grunder F, Piché N, Faure C, Aspirot A. Factors influencing the incidence of Hirschsprung associated enterocolitis (HAEC). J Pediatr Surg 2019; 54 (05) 959-963
  • 8 Parahita IG, Makhmudi A. Gunadi Comparison of Hirschsprung-associated enterocolitis following Soave and Duhamel procedures. J Pediatr Surg 2018; 53 (07) 1351-1354
  • 9 Chung PHY, Yu MON, Wong KKY, Tam PKH. Risk factors for the development of post-operative enterocolitis in short segment Hirschsprung's disease. Pediatr Surg Int 2019; 35 (02) 187-191
  • 10 Pini Prato A, Gentilino V, Giunta C. et al. Hirschsprung disease: do risk factors of poor surgical outcome exist?. J Pediatr Surg 2008; 43 (04) 612-619
  • 11 Cheng S, Wang J, Pan W. et al. Pathologically assessed grade of Hirschsprung-associated enterocolitis in resected colon in children with Hirschsprung's disease predicts postoperative bowel function. J Pediatr Surg 2017; 52 (11) 1776-1781
  • 12 Lawal TA, Chatoorgoon K, Collins MH, Coe A, Peña A, Levitt MA. Redo pull-through in Hirschsprung's [corrected] disease for obstructive symptoms due to residual aganglionosis and transition zone bowel. J Pediatr Surg 2011; 46 (02) 342-347
  • 13 Friedmacher F, Puri P. Residual aganglionosis after pull-through operation for Hirschsprung's disease: a systematic review and meta-analysis. Pediatr Surg Int 2011; 27 (10) 1053-1057
  • 14 Dickie BH, Webb KM, Eradi B, Levitt MA. The problematic Soave cuff in Hirschsprung disease: manifestations and treatment. J Pediatr Surg 2014; 49 (01) 77-80
  • 15 Kwendakwema N, Al-Dulaimi R, Presson AP. et al. Enterocolitis and bowel function in children with Hirschsprung disease and trisomy 21. J Pediatr Surg 2016; 51 (12) 2001-2004
  • 16 Reeder RW, Wood RJ, Avansino JR. Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). et al. The Pediatric Colorectal and Pelvic Learning Consortium (PCPLC): rationale, infrastructure, and initial steps. Tech Coloproctol 2018; 22 (05) 395-399
  • 17 Huang WK, Li XL, Zhang J, Zhang SC. Prevalence, risk factors, and prognosis of postoperative complications after surgery for Hirschsprung disease. J Gastrointest Surg 2018; 22 (02) 335-343
  • 18 Reding R, de Ville de Goyet J, Gosseye S. et al. Hirschsprung's disease: a 20-year experience. J Pediatr Surg 1997; 32 (08) 1221-1225
  • 19 Elhalaby EA, Teitelbaum DH, Coran AG, Heidelberger KP. Enterocolitis associated with Hirschsprung's disease: a clinical histopathological correlative study. J Pediatr Surg 1995; 30 (07) 1023-1026
  • 20 Menezes M, Puri P. Long-term outcome of patients with enterocolitis complicating Hirschsprung's disease. Pediatr Surg Int 2006; 22 (04) 316-318
  • 21 Austin KM. The pathogenesis of Hirschsprung's disease-associated enterocolitis. Semin Pediatr Surg 2012; 21 (04) 319-327
  • 22 Frykman PK, Nordenskjöld A, Kawaguchi A. HAEC Collaborative Research Group (HCRG). et al. Characterization of bacterial and fungal microbiome in children with Hirschsprung disease with and without a history of enterocolitis: a multicenter study. PLoS One 2015; 10 (04) e0124172
  • 23 Pierre JF, Barlow-Anacker AJ, Erickson CS. et al. Intestinal dysbiosis and bacterial enteroinvasion in a murine model of Hirschsprung's disease. J Pediatr Surg 2014; 49 (08) 1242-1251
  • 24 Gosain A. Established and emerging concepts in Hirschsprung's-associated enterocolitis. Pediatr Surg Int 2016; 32 (04) 313-320
  • 25 Neuvonen MI, Kyrklund K, Rintala RJ, Pakarinen MP. Bowel function and quality of life after transanal endorectal pull-through for Hirschsprung disease: controlled outcomes up to adulthood. Ann Surg 2017; 265 (03) 622-629
  • 26 Fujimoto T, Puri P. Persistence of enterocolitis following diversion of faecal stream in Hirschsprung's disease - a study of mucosal defence mechanisms. Pediatr Surg Int 1988; 3 (2–3): 141-146
  • 27 Imamura A, Puri P, O'Briain DS, Reen DJ. Mucosal immune defence mechanisms in enterocolitis complicating Hirschsprung's disease. Gut 1992; 33 (06) 801-806
  • 28 Gosain A, Frykman PK, Cowles RA. American Pediatric Surgical Association Hirschsprung Disease Interest Group. et al. Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis. Pediatr Surg Int 2017; 33 (05) 517-521
  • 29 Yang W, Chen SC, Lai JY, Ming YC, Chen JC, Chen PL. Distinctive genetic variation of long-segment Hirschsprung's disease in Taiwan. Neurogastroenterol Motil 2019; 31 (11) e13665
  • 30 Veras LV, Chotai PN, Tumen AZ, Gosain A. Impaired growth outcomes in children with congenital colorectal diseases. J Surg Res 2018; 229: 102-107
  • 31 Stone ML, LaPar DJ, Mulloy DP. et al. Primary payer status is significantly associated with postoperative mortality, morbidity, and hospital resource utilization in pediatric surgical patients within the United States. J Pediatr Surg 2013; 48 (01) 81-87