Treatment of Hirschsprung's Disease with Severe Enterocolitis and Abdominal Distension
This protocol covers the management of Hirschsprung's disease in patients who present with severe Hirschsprung's-associated enterocolitis (HAEC) — specifically those with explosive diarrhoea alongside signs of systemic compromise or radiographic abdominal distension.
Patients with explosive diarrhoea and decreased peripheral perfusion, lethargy, and/or dilated loops of bowel on abdominal radiographs meet the threshold for severe HAEC and require hospital admission. Radiographically confirmed abdominal distension is a key indicator of severity in this setting.
Management involves a targeted injection-based procedure directed at the sphincter musculature, performed under anaesthesia. Repeat interventions may be required over time.
Full regimen — including technique details, repeat criteria, and clinical decision points — is available via the full protocol.
References
- Patients with explosive diarrhea and decreased peripheral perfusion, lethargy, and/or dilated loops of bowel on abdominal radiographs have severe HAEC and should be admitted.
- Intersphincteric botulinum toxin injections are recommended for patients with recurrent or persistent symptoms of outlet obstruction and/or HAEC.
- Injections may need to be repeated 3–6 monthly.
- Botox should be injected under a short general anaesthesia.
- Injections are given in the four quadrants at the level of the dentate line into the anal sphincter musculature.
DOI: 10.1186/s13023-020-01362-3
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