What is the management protocol for IEC (Intestinal) HS?

Management Protocol for Intestinal Hirschsprung-Associated Enterocolitis (IEC HS)

The management of Intestinal Hirschsprung-Associated Enterocolitis (IEC HS) requires immediate intervention with colonic decompression, fluid resuscitation, and broad-spectrum antibiotics as first-line treatment. 1

Initial Assessment and Acute Management

• Recognize early signs of HAEC including abdominal distension, explosive diarrhea, emesis, fever, lethargy, and potential progression to shock 1
• Perform timely fluid resuscitation to correct dehydration and electrolyte imbalances 1
• Implement colonic decompression through rectal washouts to relieve obstruction 1
• Administer broad-spectrum antibiotics immediately to treat bacterial overgrowth 1

Medical Management

• Offer oral tetracyclines such as doxycycline or lymecycline for at least 12 weeks as first-line therapy, with treatment breaks to assess ongoing need and limit antimicrobial resistance 2
• Consider combination treatment with oral clindamycin 300 mg twice daily and rifampicin 300 mg twice daily for 10-12 weeks in patients unresponsive to oral tetracyclines 2
• Consider acitretin 0.3-0.5 mg/kg/day in males and non-fertile females who are unresponsive to antibiotic therapies 2
• Consider dapsone in patients unresponsive to antibiotic therapies 2

Surgical Interventions

• Recurrent nodules and tunnels should be treated with deroofing or excision 2
• Incision and drainage should only be performed for acute abscesses to relieve pain 2
• Wide local excision (using scalpel, CO2 laser, or electrosurgery) with or without reconstruction is appropriate for extensive chronic lesions 2
• Evaluate for mechanical obstruction or residual aganglionosis in cases of recurrent or persistent HAEC, which may require posterior myotomy/myectomy or redo pull-through 1

Preventative Strategies

• Implement regular rectal washouts following pull-through procedures to prevent HAEC recurrence 1
• Screen for associated comorbidities including depression, anxiety, and cardiovascular risk factors (diabetes, hypertension, hyperlipidemia, and central obesity) 2
• Refer patients to smoking-cessation services where relevant 2
• Refer patients to weight-management services where relevant 2

Monitoring and Follow-up

• Measure treatment response using recognized instruments for pain and quality of life, including inflammatory lesion count for those on adalimumab therapy 2
• Monitor for fistulating gastrointestinal disease, inflammatory arthritis, genital lymphoedema, cutaneous squamous cell carcinoma, and anemia in patients with long-standing, moderate-to-severe disease 2
• Schedule repeat endoscopic evaluation every 2-4 weeks until complete resolution 2

Special Patient Populations

Pediatric Patients

• Perform complete physical examination of typical sites and look for signs of metabolic syndrome and precocious puberty 2
• Aim to minimize scarring, progression, and need for surgery in pediatric patients 2
• Consider early procedural interventions which have potential for cure in some pediatric patients 2

Pregnant Patients

• Prioritize topical treatments, procedural treatments, and lifestyle modifications as first-line treatment 2
• Consider systemic agents as second-line treatment only when necessary 2
• Avoid retinoids and hormonal therapies which are contraindicated during pregnancy 2

Complications and Pitfalls

• Delayed recognition of HAEC can lead to rapid deterioration and poor outcomes; maintain high clinical suspicion 1
• Failure to evaluate for mechanical obstruction or residual aganglionosis in recurrent cases may lead to persistent symptoms 1
• Total intestinal aganglionosis is a rare, uniformly fatal condition that may present atypically with passage of meconium on the first day of life and no obvious intestinal distention or transition zone at laparotomy 3
• Genetic factors play a significant role in Hirschsprung disease, with mutations in the RET gene responsible for approximately half of familial cases 4, 5