Article1 min read·Published Apr 2023Older

Hirschsprung-Associated Enterocolitis at a Referral Institution: A Retrospective Review

Part of Anorectal Malformations and Colorectal Surgery 23 items

pubmed.ncbi.nlm.nih.gov

Nelson G. Rosen

Background: Hirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality amongst patients with Hirschsprung disease (HD); rectal Botulinum toxin (Botox) has been reported a possible prevention strategy. We aimed to evaluate our institution's historic cohort of HD patients, first to determine our incidence of HAEC and second to begin assessing the effect of Botox on HAEC incidence. Methods: Patients with HD seen at our institution between 2005 and 2019 were reviewed. Incidence of HD and frequencies of HAEC and Botox injections were tallied. Associations between initial Botox treatment or transition zone and HAEC incidence were evaluated. Results: We reviewed 221 patients; 200 were included for analysis. One hundred thirteen (56.5%) patients underwent primary pull-through at a median age of 24 days (IQR 91). Eighty-seven (43.5%) patients with initial ostomy had their intestinal continuity reestablished at a median of 318 days (IQR 595). Ninety-four (49.5%) experienced at least one episode of HAEC and 62 (66%) experienced multiple episodes of HAEC. Nineteen (9.6%) patients had total colonic HD and had an increased total incidence of HAEC compared to patients without total colonic HD (89% vs 44%, p < 0.001). Six (2.9%) patients received Botox injections at the time of pull-through or ostomy takedown; one experienced an episode of HAEC (versus 50.7% of the patients who were confirmed to have not received Botox injections at their surgery, p = 0.

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Article · Apr 2023 · 1 min read

In brief

Retrospective study of 200 Hirschsprung disease patients examining enterocolitis incidence and Botulinum toxin as a prevention strategy. Nearly half experienced at least one HAEC episode, with significantly higher rates in total colonic HD patients (89% vs 44%). Preliminary data suggests potential benefit of Botox injections at time of definitive surgery.

Written by the GCMD Library team from the article.

Nelson G. Rosen

Statements in this article

Hirschsprung-associated enterocolitis is the most common cause of morbidity and mortality amongst patients with Hirschsprung disease.
EstablishedBackground
In this cohort of 200 Hirschsprung disease patients, 113 (56.5%) underwent primary pull-through at a median age of 24 days.
ResearchResults
Eighty-seven patients with initial ostomy had their intestinal continuity reestablished at a median of 318 days.
ResearchResults
Ninety-four patients (49.5%) experienced at least one episode of HAEC.
ResearchResults
Sixty-two patients (66% of those with HAEC) experienced multiple episodes of HAEC.
ResearchResults
Nineteen patients (9.6%) had total colonic Hirschsprung disease.
ResearchResults
Patients with total colonic Hirschsprung disease had an increased total incidence of HAEC compared to patients without total colonic disease (89% vs 44%, p < 0.001).
ResearchResults
Six patients (2.9%) received Botox injections at the time of pull-through or ostomy takedown.
ResearchResults
One of six patients who received Botox injections at surgery experienced an episode of HAEC, versus 50.7% of patients confirmed to have not received Botox injections at their surgery (p value incomplete in source).
ResearchResults