This protocol covers patients with Hirschsprung's disease who develop severe Hirschsprung's-associated enterocolitis (HAEC). The presentation is defined by explosive diarrhoea together with decreased peripheral perfusion, lethargy, and/or dilated loops of bowel on abdominal radiographs — findings that together indicate severe disease.
Explosive diarrhoea in combination with decreased peripheral perfusion, lethargy, and abdominal distension — including radiographic evidence of dilated bowel loops — places these patients in the severe HAEC category. Inpatient management is required.
Management includes an antibiotic-based strategy. How it is applied depends on the severity of the patient's presentation. The complete regimen, clinical decision points, and sequencing are available in the full protocol.