Risk factors for enterocolitis in patients with Hirschsprung disease: A retrospective observational study

Daniëlle Roorda,Jaap Oosterlaan,Ernest Van Heurn,Joep P.M Derikx

doi:10.1016/j.jpedsurg.2021.04.020

Abstract

IntroductionHirschsprung-associated enterocolitis (HAEC) accounts for substantial morbidity and mortality in patients with Hirschsprung disease (HD). The aim of this study was to identify incidence of pre- and postoperative HAEC in our consecutive cohort and to identify patient and clinical characteristics that are associated with developing postoperative HAEC and HAEC-free interval. Material and methodsA retrospective cohort study was performed with all 146 HD patients treated between 2000 and 2017. Data were retrieved from the medical records. HAEC was defined as presence of clinical signs of bowel inflammation, that required treatment with intravenous antibiotics and admittance to the hospital during at least two days. To identify risk factor for HAEC, patients with and without a history of postoperative HAEC were compared. Kaplan-Meier and Cox-regression were used to assess HAEC free intervals before and after surgery. ResultsOut of 146 patients, 12 patients had pre-operative HAEC (8%) and 31 patients had postoperative HAEC (21%). Median preoperative HAEC free interval was 112 days (IQR 182 days). Length of hospital stay due to readmissions was longer for patients with a history of postoperative HAEC compared to patients without a history of postoperative HAEC (9.5 vs 16 days, U = 1872.5, p = 0.047). Median postoperative HAEC free interval was 226 days. Of the patients who had postoperative HAEC, 66% had their first episode within the first year after surgery and that the incidence of HAEC declined over follow-up. ConclusionsHAEC incidence was relatively low in our population. No patient or clinical characteristics were associated with the risk of postoperative HAEC.

Highlights

Hirschsprung-associated enterocolitis (HAEC) accounts for substantial morbidity and mortality in patients with Hirschsprung disease (HD)
Two patients deceased before corrective surgery was performed and two patients were treated conservatively: one patient with short segment disease had no symptoms of constipation and had spontaneous bowel movements with laxatives; the other patient was diagnosed late, at the age of five years, and had satisfactory bowel movements with laxatives and rectal irrigation
Our findings suggested that a history of pre-operative HAEC, length of aganglionosis, type of corrective surgery, presence of a temporary ostomy, and postoperative complications within 30 days after surgery were not associated with higher hazards on developing postoperative enterocolitis

Summary

Introduction

Hirschsprung-associated enterocolitis (HAEC) accounts for substantial morbidity and mortality in patients with Hirschsprung disease (HD). The aim of this study was to identify incidence of preand postoperative HAEC in our consecutive cohort and to identify patient and clinical characteristics that are associated with developing postoperative HAEC and HAEC-free interval. Median postoperative HAEC free interval was 226 days. Of the patients who had postoperative HAEC, 66% had their first episode within the first year after surgery and that the incidence of HAEC declined over follow-up. No patient or clinical characteristics were associated with the risk of postoperative HAEC. Hirschsprung-associated enterocolitis (HAEC) is a severe complication of Hirschsprung disease (HD) and is one of the significant causes of morbidity and mortality in patients with HD, with an estimated incidence of postoperative HAEC in 10% of patients [1,2,3]. The estimated incidence of preoperative HAEC is 15–18% [5, 6]

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