Suggested categorization and management of patients with suspected or established Hirschsprung-associated enterocolitis

APSA categorization	Typical presenting symptoms	Typical radiographic features	Recommended treatment (do all of the following)	Possible additional measures
Possible HAEC (grade I)	Anorexia, diarrhea, mild abdominal distention	Normal radiograph or mild signs of ileus	Oral hydration Oral metronidazole	Rectal irrigations
Definite HAEC (grade II)	One or more of the following*: Explosive diarrhea Fever, tachycardia, or lethargy Moderate abdominal distention and/or tenderness Explosive gas/stool on rectal examination	May include: Signs of ileus, including air-fluid levels and dilated loops of bowel Distension of the proximal colon, with rectosigmoid cutoff^¶	Clear liquids or hold feeds IV hydration Metronidazole (oral or IV) Broad-spectrum antibiotic coverage^Δ (in addition to metronidazole) Rectal irrigations	Nasogastric decompression^◊
Severe HAEC (grade III)	Symptoms of grade II (above), PLUS: Obstipation Poor perfusion Hypotension Altered consciousness/mentation Marked abdominal distension Signs of peritonitis	Signs of grade II (above), PLUS possible: Pneumatosis intestinalis Pneumoperitoneum (rare)	Hold feeds Metronidazole (IV), AND Broad-spectrum IV antibiotics^Δ IV hydration Rectal irrigations	Nasogastric decompression^◊ Possible surgical intervention^§

HAEC: Hirschsprung-associated enterocolitis; APSA: American Pediatric Surgical Association; IV: intravenous.
* A particularly high suspicion for HAEC is appropriate for patients with a prior history of this disorder.
¶ On plain radiograph, HAEC is specifically suggested by distension of the proximal colon and absence of air in the distal rectosigmoid colon, with an abrupt cutoff at the level of the pelvic brim.
Δ Broad-spectrum antibiotic coverage might consist of IV ampicillin with gentamicin, or IV piperacillin/tazobactam.
◊ Patients with marked abdominal distension should be treated with nasogastric decompression as well as holding of feeds.
§ Patients who develop pneumoperitoneum may require surgical exploration. Patients with severe HAEC who fail to improve with maximal medical management may require surgery with proximal bowel diversion.

Adapted from Gosain A, Frykman PK, Cowles RA, et al. Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis. Pediatr Surg Int 2017; 33:517.

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Suggested categorization and management of patients with suspected or established Hirschsprung-associated enterocolitis