StayCurrentMD · Hirschsprung's Associated Enterocolitis (HAEC) Treatment Guideline
Guideline2 min read·Published Aug 2019Older

Hirschsprung's Associated Enterocolitis (HAEC) Treatment Guideline

Guideline · Aug 2019 · 2 min read

In brief

In brief

Clinical practice guideline for the diagnosis, prevention, and management of Hirschsprung's-associated enterocolitis, a serious complication affecting children with Hirschsprung disease before and after surgical correction.

Written by the GCMD Library team from the guideline.

Initial Assessment and Evaluation

Surgical fellow or attending must evaluate suspected HAEC patients within 1 hour and document findings. Rectal exam is mandatory as part of initial assessment, though should be discussed with attending surgeon if patient is within 4 weeks post-surgery. Early recognition and documentation of treatment plan is critical for optimal outcomes.

Rectal Irrigation Protocol

Rectal irrigations must be initiated within 1 hour of arrival and should not be delayed for imaging. Irrigations should be performed by experienced personnel (A4S nurses available for support) at minimum every 8 hours, with consideration for more frequent intervals (Q6H or PRN) in severe cases. This is the primary therapeutic intervention for decompression.

Diagnostic Imaging Requirements

Abdominal x-ray should be obtained on arrival and repeated after initial irrigation to confirm adequate decompression. Subsequent imaging should be performed as clinically indicated to monitor treatment response and guide ongoing management.

Supportive Care Measures

Patients should be made NPO and started on intravenous fluids immediately. Replogle tube placement should be considered for patients with significant abdominal distention to prevent aspiration and further decompression.

Antibiotic Therapy Guidelines

All vomiting patients require IV antibiotics. Outpatients with low suspicion or mild cases may be managed with oral metronidazole, while inpatients without systemic signs can receive PO or IV metronidazole. Patients with systemic signs require IV broad-spectrum antibiotics including metronidazole.

Laboratory Evaluation

Essential laboratory tests include complete blood count, renal panel, and venous blood gas for any patient exhibiting systemic signs. These studies help assess severity of illness and guide fluid resuscitation and antibiotic therapy.

Disposition and Monitoring

Patients without systemic signs should be admitted to the surgical floor with vital signs including blood pressure measured every 4 hours. Patients with systemic signs (fever, lethargy, tachycardia, hypotension, tachypnea, oliguria) require evaluation for potential ICU admission given risk of sepsis and hemodynamic instability.

Rectal Irrigation Technique and Supplies

Irrigation requires silicone Foley catheter (16 Fr for children ≤1 year, 24 Fr for >1 year), 60cc catheter-tip syringe, water-soluble lubricant, saline solution, and non-sterile basins. A standardized orderset and instructional video are available to ensure proper technique and consistency across providers.

Statements in this guideline

  1. A surgical fellow or attending should personally see and evaluate all patients with suspected HAEC within 1 hour and document exam and treatment plan.

    RecommendationTreatment Principles for Suspected HAEC
  2. A rectal exam should be performed as part of initial exam.

    RecommendationTreatment Principles for Suspected HAEC
  3. If patient is less than 4 weeks from surgery, rectal exam or dilations should be discussed with the Attending Surgeon prior to performing exam.

    RecommendationTreatment Principles for Suspected HAEC
  4. Rectal irrigations should be initiated within 1 hour of arrival.

    RecommendationTreatment Principles for Suspected HAEC
  5. Do not delay irrigations to wait for initial abdominal x-ray.

    RecommendationTreatment Principles for Suspected HAEC
  6. Irrigations should be Q8H at minimum, with consideration for more frequent Q6H or PRN if severe enterocolitis.

    RecommendationTreatment Principles for Suspected HAEC
  7. Abdominal x-ray should be obtained on arrival and repeated after the initial irrigation and thereafter as needed to demonstrate adequate decompression.

    RecommendationTreatment Principles for Suspected HAEC
  8. Patient should be NPO and started on IVF.

    RecommendationTreatment Principles for Suspected HAEC
  9. If significant abdominal distention, consider replogle.

    RecommendationTreatment Principles for Suspected HAEC
  10. Any vomiting patients should be on IV antibiotics.

    RecommendationTreatment Principles for Suspected HAEC
  11. Laboratory tests that should be considered include CBC, renal panel, and a venous blood gas for any patient with systemic signs.

    RecommendationTreatment Principles for Suspected HAEC
  12. Patients without systemic signs should be admitted to the surgical floor.

    RecommendationTreatment Principles for Suspected HAEC
  13. Patients with systemic signs should be evaluated for potential ICU admission.

    RecommendationTreatment Principles for Suspected HAEC
  14. All HAEC patients on the surgical floor should have vitals with blood pressure measurement every 4 hours.

    RecommendationTreatment Principles for Suspected HAEC
  15. PO metronidazole is sufficient for low suspicion or mild outpatient cases.

    RecommendationSuspected Hirschsprung's Associated Enterocolitis (HAEC) Treatment Guidelines
  16. Inpatients without systemic signs may be managed with PO or IV metronidazole, with PO sufficient for low suspicion or mild cases.

    RecommendationSuspected Hirschsprung's Associated Enterocolitis (HAEC) Treatment Guidelines
  17. Inpatients with systemic signs require IV broad-spectrum antibiotics, including metronidazole.

    RecommendationSuspected Hirschsprung's Associated Enterocolitis (HAEC) Treatment Guidelines
  18. GI complaints may include abdominal distention, vomiting, no or minimal stooling, foul-smelling stool, and explosive diarrhea.

    EstablishedSuspected Hirschsprung's Associated Enterocolitis (HAEC) Treatment Guidelines
  19. Systemic signs include fever, lethargy, age-adjusted tachycardia, hypotension, tachypnea, and oliguria.

    EstablishedSuspected Hirschsprung's Associated Enterocolitis (HAEC) Treatment Guidelines
  20. Use 16 fr silicone foley catheter for children 1 year or younger and 24 fr for children older than 1 year for rectal irrigations.

    RecommendationRectal Irrigation Supplies
Full text

Treatment Principles for Suspected HAEC1). A surgical fellow or attending should personally see and evaluate all patients with suspected HAEC at the earliest possible opportunity (within 1 hour) and document exam and treatment plan. 2). A rectal exam should be performed as part of initial exam. If patient is <4 weeks from surgery, rectal exam or dilations should be discussed with the Attending Surgeon prior to performing exam. 3). Rectal irrigations should be initiated at earliest opportunity (within 1 hour) of arrival. Do not delay irrigations to wait for initial abdominal x-ray. Rectal irrigations should be performed by the person most versed in the process; A4S nurses are available to provide support and assistance. Irrigations should be Q8H at minimum (consider more frequent –Q6H or PRN –if severe enterocolitis). 4). Abdominal x-ray should be obtained on arrival and repeated after the initial irrigation and thereafter as needed to demonstrate adequate decompression. 5). Patient should be NPO and started on IVF . If significant abdominal distention, consider replogle. 6). Antibiotics: Any vomiting patients should be on IV antibiotics. 7). Laboratory tests that should be considered include: CBC, renal panel, and a venous blood gas (for any patient with systemic signs). 8). Disposition:•Patients withoutsystemic signs**: Admit to the surgical floor. •Patient withsystemic signs**: Should be evaluated for potential ICU admission. 9). All HAEC patients on the surgical floor should have vitalswith blood pressure measurement every 4 hours. Suspected Hirschsprung’s Associated Enterocolitis (HAEC) Treatment Guidelines Scenario: Patient with known/suspected Hirschsprung’s Disease (HD) presents to the ED with GI complaints* and/or fever . v2 Updated 4/2019 Outpatient:PO metronidazole is sufficient for low suspicion or mild cases Inpatient: WITHsystemic signs**WITHOUT systemic signs** IV broad-spectrum antibiotics, including metronidazole PO or IV metronidazole (low suspicion or mild cases may be managed with PO) *GI complaints may include:Abdominal distention, vomiting, no/minimal stooling, foul-smelling stool, and/or explosive diarrhea Rectal Irrigation Supplies:•Silicone foleycatheter (16 frfor children ≤1 year; 24 frfor children >1 year)•60 cc catheter tip syringe•Lubricant (water soluble)•Saline solution•2 non-sterile basins (e.g. emesis basin)Rectal Irrigation Orderset:Use “Hirschsprung Disease Rectal Irrigation” ordersetto order subsequent irrigations. Rectal Irrigation Video:https://cchmcstream.cchmc.org/MediasiteEX/Play/545154a603a844e8988ef74cd5b4c1c11d **Systemic signs include:Fever, lethargy, age-adjusted tachycardia, hypotension, tachypnea, oliguria

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