nach oben

Erschienen in:

02.02.2017 | Review Article

Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis

verfasst von: Ankush Gosain, Philip K. Frykman, Robert A. Cowles, John Horton, Marc Levitt, David H. Rothstein, Jacob C. Langer, Allan M. Goldstein, On behalf of the American Pediatric Surgical Association Hirschsprung Disease Interest Group

Erschienen in: Pediatric Surgery International | Ausgabe 5/2017

Einloggen, um Zugang zu erhalten

Abstract

Background

Patients with Hirschsprung disease are at risk for Hirschsprung-associated enterocolitis (HAEC), an inflammatory disorder of the bowel that represents the leading cause of serious morbidity and death in these patients. The diagnosis of HAEC is made based on clinical signs and symptoms which are often non-specific, making it difficult to establish a definitive diagnosis in many patients. The purpose of this guideline is to present a rational, expert-based approach to the diagnosis and management of HAEC.

Methods

The American Pediatric Surgical Association Board of Governors established a Hirschsprung Disease Interest Group. Group discussions, literature review, and expert consensus were then used to summarize the current state of knowledge regarding diagnosis, management, and prevention of Hirschsprung-associated enterocolitis (HAEC).

Results

Guidelines for the diagnosis of HAEC and its clinical grade, utilizing clinical history, physical examination findings, and radiographic findings, are presented. Treatment guidelines, including patient disposition, diet, antibiotics, rectal irrigations and surgery, are presented.

Conclusions

Clear, standardized definitions of Hirschsprung-associated enterocolitis and its treatment are lacking in the literature. This guideline serves as a first step toward standardization of diagnosis and management.

Level of evidence

Haricharan RN, Seo J-M, Kelly DR et al (2008) Older age at diagnosis of Hirschsprung disease decreases risk of postoperative enterocolitis, but resection of additional ganglionated bowel does not. J Pediatr Surg 43:1115–1123. doi:10.1016/j.jpedsurg.2008.02.039 CrossRefPubMed

El-Sawaf M, Siddiqui S, Mahmoud M et al (2013) Probiotic prophylaxis after pullthrough for Hirschsprung disease to reduce incidence of enterocolitis: a prospective, randomized, double-blind, placebo-controlled,multicenter trial. J Pediatr Surg 48:111–117. doi:10.1016/j.jpedsurg.2012.10.028 CrossRefPubMed

Ward NL, Pieretti A, Dowd SE et al (2012) Intestinal aganglionosis is associated with early and sustained disruption of the colonic microbiome. Neurogastroenterol Motil 24:874–400. doi:10.1111/j.1365-2982.2012.01937.x CrossRefPubMed

Pierre JF, Barlow-Anacker AJ, Erickson CS et al (2014) Intestinal dysbiosis and bacterial enteroinvasion in a murine model of Hirschsprung’s disease. J Pediatr Surg 49:1242–1251. doi:10.1016/j.jpedsurg.2014.01.060 CrossRefPubMedPubMedCentral

Yan Z, Poroyko V, Gu S et al (2014) Biochemical and biophysical research communications. Biochem Biophys Res Commun 445:269–274. doi:10.1016/j.bbrc.2014.01.104 CrossRefPubMed

Frykman PK, Nordenskjöld A, Kawaguchi A et al (2015) Characterization of bacterial and fungal microbiome in children with Hirschsprung disease with and without a history of enterocolitis: a multicenter study. PLoS One 10:e0124172. doi:10.1371/journal.pone.0124172 CrossRefPubMedPubMedCentral

Thiagarajah JR, Yildiz H, Carlson T et al (2014) Altered goblet cell differentiation and surface mucus properties in Hirschsprung disease. PLoS One 9:e99944. doi:10.1371/journal.pone.0099944.t001 CrossRefPubMedPubMedCentral

Yildiz HM, Carlson TL, Goldstein AM, Carrier RL (2015) Mucus barriers to microparticles and microbes are altered in Hirschsprung’s disease. Macromol Biosci 15:712–718. doi:10.1002/mabi.201400473 CrossRefPubMedPubMedCentral

Gosain A, Barlow-Anacker AJ, Erickson CS et al (2015) Impaired cellular immunity in the murine neural crest conditional deletion of endothelin receptor-B model of Hirschsprung’s disease. PLoS One 10:e0128822. doi:10.1371/journal.pone.0128822 CrossRefPubMedPubMedCentral

10.

Frykman PK, Cheng Z, Wang X, Dhall D (2015) Enterocolitis causes profound lymphoid depletion in endothelin receptor B- and endothelin 3-null mouse models of Hirschsprung-associated enterocolitis. Eur J Immunol 45:807–817. doi:10.1002/eji.201444737 CrossRefPubMedPubMedCentral

11.

Cheng Z, Dhall D, Zhao L et al (2010) Murine model of Hirschsprung-associated enterocolitis. I: phenotypic characterization with development of ahistopathologic grading system. J Pediatr Surg 45:475–482. doi:10.1016/j.jpedsurg.2009.06.009 CrossRefPubMedPubMedCentral

12.

Pastor AC, Osman F, Teitelbaum DH et al (2009) Development of a standardized definition forHirschsprung’s-associated enterocolitis:a Delphi analysis. J Pediatr Surg 44:251–256. doi:10.1016/j.jpedsurg.2008.10.052 CrossRefPubMed

13.

Bell MJ, Ternberg JL, Feigin RD et al (1978) Neonatal necrotizing enterocolitis. Therapeutic decisions based upon clinical staging. Ann Surg 187:1–7CrossRefPubMedPubMedCentral

14.

Frykman PK, Short SS (2012) Hirschsprung-associated enterocolitis: prevention and therapy. YSPSU 21:328–335. doi:10.1053/j.sempedsurg.2012.07.007

15.

Wang X, Li Z, Xu Z et al (2015) Probiotics prevent Hirschsprung’s disease-associated enterocolitis: a prospective multicenter randomized controlled trial. Int J Colorectal Dis 30:105–110. doi:10.1007/s00384-014-2054-0 CrossRefPubMed

16.

Patrus B, Nasr A, Langer JC, Gerstle JT (2011) Intrasphincteric botulinum toxin decreases the rate of hospitalization for postoperative obstructive symptoms in children with Hirschsprung disease. J Pediatr Surg 46:184–187. doi:10.1016/j.jpedsurg.2010.09.089 CrossRefPubMed

Titel: Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis
verfasst von: Ankush Gosain
Philip K. Frykman
Robert A. Cowles
John Horton
Marc Levitt
David H. Rothstein
Jacob C. Langer
Allan M. Goldstein
On behalf of the American Pediatric Surgical Association Hirschsprung Disease Interest Group
Publikationsdatum: 02.02.2017
Verlag: Springer Berlin Heidelberg
Erschienen in: Pediatric Surgery International / Ausgabe 5/2017
Print ISSN: 0179-0358
Elektronische ISSN: 1437-9813
DOI: https://doi.org/10.1007/s00383-017-4065-8

Update Pädiatrie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Springer Medizin

Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis