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Table 7 Recommendations for Hirschsprung’s-associated enterocolitis (HAEC)

From: ERNICA guidelines for the management of rectosigmoid Hirschsprung’s disease

HAEC should be clinically suspected in the presence of diarrhoea with explosive, foul-smelling stool, and/or > 4 points from the Pastor et al. HAEC score items (Table 8) [76]
• The definition of HAEC remains imprecise even based on current understanding
• A cut-off of > 10 points has a reported sensitivity of 42% and specificity of 100% for HAEC [75]
• A cut-off of > 4 points has a reported sensitivity of 84% and specificity of 98% for HAEC [75]
Level of evidence III
Strength of recommendation: Strong, for
Level of agreement: 100%
In suspected HAEC, there should be a low threshold for hospital admission
• In mild symptoms with no fluid or electrolyte balance disturbance and normal inflammatory markers, outpatient treatment with oral hydration +/− oral metronidazole and rectal irrigations may be appropriate, but prompt admission is indicated if symptoms do not improve. Recovery should be followed up.
• Admit all other cases for in-patient monitoring and treatment
• Young age (< 1 year) lowers the threshold for admission
Level of evidence III
Strength of recommendation: Strong, for
Level of agreement: 100%
Following admission to hospital, patients with HAEC should be treated with intravenous fluid resuscitation, intravenous broad-spectrum antibiotics and rectal washouts.
• Saline rectal washouts to decompress the bowel should be performed 2–3 times per day until the patient is well enough for discharge
• Antibiotics may be changed to oral metronidazole once sufficient clinical improvement occurs
• Vital functions, fluid and electrolyte balance, including urine output, should be closely monitored.
• Abdominal plain film x-ray should be considered
• Consulting the colorectal surgical team responsible for the patient’s care is recommended
• Consult intensive care unit as appropriate
Level of evidence III
Strength of recommendation: Strong, for
Level of agreement: 100%
Intersphincteric botulinum toxin injections are recommended for patients with recurrent or persistent symptoms of outlet obstruction and/or HAEC
• In reports, 62–89% of HSCR patients with HAEC and/or outlet obstruction improved after the first botulinum toxin injection [82,83,84,85,86]
• Injections may need to be repeated 3–6 monthly
• The tendency to HAEC reduces over time; most episodes usually occur within the first few years after pull through
Principles of botulinum toxin administration
• Botox should be injected under a short general anaesthesia
• The patient is positioned in lateral decubitus or lithotomy position
• Injections are given in the four quadrants at the level of the dentate line into the anal sphincter musculature
• Exposure of the dentate line with retractors, and/or ultrasound guidance can facilitate correct localization of the injections
Level of evidence III
Strength of recommendation: Conditional, for
Level of agreement: 100%
Prophylactic antibiotics may be considered for patients with frequently recurring or persistent HAEC
• Antibiotics may be effective treatment of HAEC in individual patients, but it has not been shown that prophylactic antibiotics prevent recurrent HAEC.
• Recurrent courses of antibiotics interfere with the long-term composition of the gut microbiota, and therefore rationalized use based on severity of symptoms is indicated
Level of evidence III
Strength of recommendation: Conditional, for
Level of agreement: 100%
At present, there is insufficient evidence to support recommending the routine use of probiotics for the prevention of HAEC
• Although intestinal dysbiosis has been shown to be of importance in the aetiology of HAEC, there are only two randomized controlled studies of probiotics and HAEC in the literature, showing conflicting results.
Level of evidence I-III
Strength of recommendation: Conditional, against
Level of agreement: 100%
In children with recurrent HAEC, consultation with a gastroenterologist and endoscopy should be considered
• Patients with HSCR have an increased risk of developing inflammatory bowel disease
• Fecal calprotectin is a non-invasive measure of intestinal inflammation in acute and chronic enterocolitis
Level of evidence III
Strength of recommendation: Conditional, for
Level of agreement: 100%