Treatment of Hirschsprung's Disease with Severe Enterocolitis and Abdominal Distension

Clinical Scenario

This protocol applies to patients with Hirschsprung's disease who develop severe Hirschsprung's-associated enterocolitis (HAEC) — presenting with explosive diarrhoea alongside systemic compromise such as decreased peripheral perfusion, lethargy, and/or dilated loops of bowel on abdominal radiographs. Abdominal distension is a defining feature of this severe presentation and warrants immediate hospital admission.

Patients with explosive diarrhoea and decreased peripheral perfusion, lethargy, and/or dilated loops of bowel on abdominal radiographs have severe HAEC and should be admitted to hospital.

Treatment Approach

In-hospital management centres on intravenous supportive therapy and bowel decompression, combined with antibiotic treatment. The structured protocol specifies the complete regimen — including decompression frequency, antibiotic selection and escalation, and the criteria for clinical step-down.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1186/s13023-020-01362-3

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.

Patients with severe HAEC should receive rectal washouts, intravenous fluid resuscitation and intravenous broad-spectrum antibiotics in hospital.

Following admission to hospital, patients with HAEC should be treated with intravenous fluid resuscitation, intravenous broad-spectrum antibiotics and rectal washouts.

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