Reliability of the Hirschsprung-Associated Enterocolitis Score in Clinical Practice

Abstract

Introduction There is a lack of an agreed Hirschsprung-associated enterocolitis (HAEC) definition. In 2009, a HAEC score was proposed for the diagnosis of HAEC episodes. Our aim was to apply the HAEC score on HAEC episodes to determine its diagnostic efficiency and whether it correlated to its severity.

Methods Retrospective study of patients with HAEC admitted between 2000 and 2016. Episodes of HAEC were identified and the HAEC score was calculated. A cut-off of ≥ 10 according to Pastor et al and ≥ 4 according to Frykman et al were used. A Pearson's correlation coefficient was performed for outcome variable: length-of-stay (LOS).

Results Note that 21/93 (22.6%) patients with Hirschsprung's disease presented 50 HAEC episodes with a median of 2 (1–5) episodes during an 8.3-year (2–15.6) follow-up. The most common symptoms were foul-smelling (86% [43/50]) and explosive (60% [30/50]) diarrhea. Physical findings showed a distended abdomen (76% [38/50]) and fever (60% [30/50]) with dilated bowel (82% [41/50]) and rectosigmoid cut-off (80% [40/50]) identified on X-rays. Only 34% (17/50) showed abnormal laboratory findings. Patients were admitted with a median LOS of 7 days (1–28). A HAEC score of 9 (2–16) was found, and only 50% (25/50) of patients met the initial criteria (score of 10 points). However, the use of a 4-point cut-off would have allowed the diagnosis of 98% (49/50) of the patients. A positive linear correlation was found for LOS and HAEC score (r = 0.3, p = 0.014).

Conclusion There is no standardized definition for HAEC. The initial HAEC score cut-off is restrictive and might fail to identify milder episodes. The positive correlation with LOS and thus HAEC severity might aid in patient information and anticipation of treatment.

Keywords

Hirschsprung's disease - Hirschsprung-associated enterocolitis - Hirschsprung's complications - Hirschsprung-associated enterocolitis severity - Hirschsprung-associated enterocolitis score

• References

• 1 Frykman PK, Short SS. Hirschsprung-associated enterocolitis: prevention and therapy. Semin Pediatr Surg 2012; 21 (04) 328-335
• 2 Austin KM. The pathogenesis of Hirschsprung's disease-associated enterocolitis. Semin Pediatr Surg 2012; 21 (04) 319-327
• 3 Demehri FR, Halaweish IF, Coran AG, Teitelbaum DH. Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention. Pediatr Surg Int 2013; 29 (09) 873-881
• 4 Frykman PK, Nordenskjöld A, Kawaguchi A. , et al; HAEC Collaborative Research Group (HCRG). 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
• 5 Gosain A. Established and emerging concepts in Hirschsprung's-associated enterocolitis. Pediatr Surg Int 2016; 32 (04) 313-320
• 6 Gosain A, Barlow-Anacker AJ, Erickson CS. , et al. Impaired cellular immunity in the murine neural crest conditional deletion of endothelin receptor-B model of Hirschsprung's disease. PLoS One 2015; 10 (06) e0128822
• 7 Travassos D, van Herwaarden-Lindeboom M, van der Zee DC. Hirschsprung's disease in children with Down syndrome: a comparative study. Eur J Pediatr Surg 2011; 21 (04) 220-223
• 8 Menezes M, Puri P. Long-term clinical outcome in patients with Hirschsprung's disease and associated Down's syndrome. J Pediatr Surg 2005; 40 (05) 810-812
• 9 Ieiri S, Higashi M, Teshiba R. , et al. Clinical features of Hirschsprung's disease associated with Down syndrome: a 30-year retrospective nationwide survey in Japan. J Pediatr Surg 2009; 44 (12) 2347-2351
• 10 Friedmacher F, Puri P. Hirschsprung's disease associated with Down syndrome: a meta-analysis of incidence, functional outcomes and mortality. Pediatr Surg Int 2013; 29 (09) 937-946
• 11 Lee C-C, Lien R, Chiang M-C. , et al. Clinical impacts of delayed diagnosis of Hirschsprung's disease in newborn infants. Pediatr Neonatol 2012; 53 (02) 133-137
• 12 Gosain A, Frykman PK, Cowles RA. , et al; American Pediatric Surgical Association Hirschsprung Disease Interest Group. Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis. Pediatr Surg Int 2017; 33 (05) 517-521
• 13 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
• 14 Frykman PK, Kim S, Wester T. , et al; HAEC Collaborative Research Group (HCRG). 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