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Hirschsprung associated enterocolitis HAEC

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  • See also

    IV fluids
    Constipation

    Key Points

    1. HAEC (Hirschsprung associated enterocolitis) is the leading cause of significant morbidity and mortality in HD (Hirschsprung Disease)
    2. Mild HAEC can mimic simple gastroenteritis or constipation however requires specialised treatment
    3. Prompt treatment and surgical referral are necessary

    Background

    • HAEC is an inflammatory disorder of the colon in children with HD, commonly associated with complete or partial obstruction
    • All children with HD are at risk of developing HAEC. It is the most common cause of significant morbidity and mortality in children with HD
    • HAEC may occur before and/or after pull-through surgery for HD
    • HAEC is more common in children <2 years of age, those with undiagnosed HD, Trisomy 21, children with long segment HD, and children with previous episodes of HAEC
    • Diagnosis is based on clinical symptoms and signs, which may be non- specific. Mild HAEC may present similarly to gastroenteritis

    Assessment

    History

    • Fever
    • Lethargy
    • Anorexia
    • Reduced oral intake
    • Vomiting
    • Stool (time since last stool, diarrhoea - particularly if explosive in quality, constipation, malodorous)
      • Most patients with HAEC will have reduced stool output but some may have diarrhoea  
    • Rectal bleeding
    • Recent viral infections (respiratory, gastrointestinal)
    • History of recurrent episodes of HAEC
    • Medications
    • Extent of HD
      • Short segment (rectosigmoid)
      • Long segment (proximal colon)
      • Total colonic
    • Current management for HD (rectal bowel washout type and frequency)
    • Type of definitive procedure
      • Swenson - removal of the aganglionated and transition zone bowel, with the ganglionated bowel brought down and attached just above the anus
      • Soave - removal of the aganglionated and transition zone bowel, with the ganglionated bowel brought down through a muscle cuff of aganglionated bowel and attached just above the anus
      • Duhamel - the ganglionated bowel bought down behind the rectum, leaving a limited section of aganglionated rectum on the front wall to create a slowing mechanism for the stool

    Examination

    • Signs of haemodynamic instability
    • Fever
    • Hydration status
    • Abdominal distension
    • Abdominal tenderness
    • Signs of peritonitis (may be subtle)
    • Explosive gas/stool on direct rectal examination (to be performed by senior clinician/surgical team)

    Management

    Investigations

    • Abdominal x-ray (dilated bowel loops, pneumatosis, air fluid levels, ‘cut off sign’ in rectosigmoid)
      • Cut off sign: gaseous distension of the proximal colon followed by abrupt absence of gas in distal colon
    • Bloods
      • Blood gas
      • FBE (leucocytosis, left shift)
      • UEC
      • Blood culture
    • Stool (send for MCS, C. difficile, and rotavirus PCR)
    • Consider respiratory PCR

    Treatment

    • Resuscitation for signs of shock or hypovolaemia (see Resuscitation: Care of the seriously unwell child)
    • Nil by mouth
    • Intravenous fluids
    • Surgical review
    • IV antibiotics (after discussion with surgical team)
      • Cefazolin 50 mg/kg (max 2 g) IV every 8 hours
      • Metronidazole 7.5 mg/kg (max 500 mg) IV 3 times per day
      • Typically 5 days duration
      • See local guidelines
    • Antiemetic
      • Ondansetron (6 months – 18 years) 0.15 mg/kg (max 8 mg) IV every 8 hours
    • Rectal bowel washout (should only performed by surgical team)
      • 20 mL/kg warm sodium chloride 0.9%, up to 3 times per day

    Consider consultation with local paediatric team

    • For all children with HD and/or suspected HAEC
    • All patients should also be discussed with paediatric surgical team (local or referral hospital)

    Consider transfer when

    • Child requiring care above the level of care provided at local hospital or treating medical team
    • Children with HAEC are often transferred to a tertiary centre

    For emergency advice and paediatric or neonatal ICU transfers, see Retrieval Services

    Consider discharge

    Only after discussion with the paediatric surgical team

    Parent information

    Hirschsprung Associated Enterocolitis - information for families and health professionals
    Hirschsprung Disease
    HAEC Medical Alert Card

    Additional notes

    Bowel washout rectal

    Last updated April 2023

  • Reference List

    1. Demehri FR, Halaweish IF, Coran AG, Teitelbaum DH. Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention. Pediatric surgery international. 2013. 29(9):873-81
    2. Gosain A, Frykman PK, Cowles RA, Horton J, Levitt M, Rothstein DH, Langer JC, Goldstein AM. Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis. Pediatric surgery international. 2017. 33(5):517-21
    3. Pastor AC, Osman F, Teitelbaum DH, Caty MG, Langer JC. Development of a standardized definition for Hirschsprung's-associated enterocolitis: a Delphi analysis. Journal of pediatric surgery. 2009. 44(1):251-6