Hirschsprung's disease associated enterocolitis: A comprehensive review

Table 1 Hirschsprung's disease associated enterocolitis severity score and management[17]

APSA category	Symptom	Radiography	Treatment	Additional measures
Grade 1 (Possible HAEC)	Anorexia, diarrhea, mild abdominal distension	Normal, or mild signs of ileus	Oral hydration; Oral metronidazole	Rectal irrigations
Grade 2 (Definite HAEC)	One or more of the following: Explosive diarrhea; Fever, tachycardia, or lethargy; Moderate abdominal distension and/or tenderness; Explosive gas/stool on rectal examination;	May include: Signs of ileus (air fluid levels, dilated bowel loops); Distension of the proximal colon w/rectosigmoid cutoff	Clear liquids or NPO; IV hydration; Metronidazole (PO or IV); Broad spectrum antibiotic coverage; Rectal irrigations	NGT decompression
Grade 3 (Severe HAEC)	Grade II symptoms plus: Obstipation; Poor perfusion; Hypotension; Altered mental status; Marked abdominal distension; Signs of peritonitis	Signs of Grade II PLUS possible: Pneumatosis intestinalis; Pneumoperitoneum (rare)	NPO; Metronidazole (IV) + broad spectrum antibiotics (IV ampicillin + gentamicin, IV piperacillin/tazobactam); Rectal irrigations	NGT decompression; Possible surgical intervention

APSA: American Pediatric Surgery Association; HAEC: Hirschsprung’s disease associated enterocolitis; IV: Intravenous; NGT: Nasogastric tube; NPO: Nothing by mouth; PO: Per oral.