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Table 9 Recommendations for management of patients with poor functional outcomes

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

Children with normal intellectual development who are not continent of stool by 4 years of age should be considered for further evaluation. This should include:
• A stooling history and stooling pattern to evaluate for tendency to constipation or diarrhoea (for treatment, see below), and involuntary passage of flatus.
• Dietary history and growth
• Examination under anaesthesia +/− anorectal manometry to assess the integrity of the anal canal, sphincter complex and dentate line, and for the presence of rolled muscle cuff, stricture or rectal spur
• Contrast enema to evaluate whether there is colonic dilatation, rectal spur, constipation or a twisted pull-through
• +/− Endorectal ultrasound to assess for sphincter defects
Level of evidence III
Strength of recommendation: Strong, for
Level of agreement: 100%
The management of fecal incontinence should aim for age-appropriate continence in children with normal intellectual development
• Primary prevention of the social consequences of fecal incontinence is a key goal of treatment
• Enabling normal social integration, school attendance and ability to participate in recreational activities from the outset is important for self-esteem, friendships and long-term quality of life
• Deficient fecal continence in a child is also a source of stress for caregivers and psychological support should be available for patients and families
• Cognitive impairment is associated with delays in achieving voluntary bowel control
Level of evidence III
Strength of recommendation: Strong, for
Level of agreement: 100%
Patients with an intact anal canal and appropriate pull-through but fecal incontinence should receive medical management as the first-line treatment
• For patients with a dilated colon and constipation (hypomotility), oral laxatives +/− a short course of enemas to ensure regular and complete colonic emptying
• For patients without colonic dilatation and a tendency to loose stools (hypermotility), a constipating diet +/− loperamide +/− bulking agents (pectin, psyllium)
• Measure fecal calprotectin, consider ileo-colonoscopy and repeat rectal biopsy
• Proceed to bowel management if there is failure to respond, despite adequate dosing and compliance
Level of evidence III
Strength of recommendation: Conditional, for
Level of agreement: 100%
Patients with fecal incontinence and damaged anal canal should receive bowel management
• Maintaining an intact anal canal is a central goal in all standard operations for HSCR, and an indication for performing pull-through surgery in specialist units
• An enterostomy is an option if bowel management fails to control symptoms
Level of evidence III
Strength of recommendation: Conditional, for
Level of agreement: 100%
Children with persistent obstructive symptoms following pull-through surgery should undergo further evaluation and treatment:
• Rectal examination and contrast enema to rule out a mechanical cause and to assess for colonic dilatation
• If no mechanical cause is found, a trial of intersphincteric botulinum toxin injections
• Review the histology of the proximal margins of the originally resected bowel
• Repeat rectal biopsies to ensure normal innervation of the pulled-through bowel
• If repeated botulinum toxin injections are ineffective, histology is normal and there is no mechanical cause, bowel management can be offered
• Consider re-do surgery in patients with a recalcitrant stricture, twisted pull-through, rolled muscle cuff (Soave), rectal spur (Duhamel) or transition zone pull-through
Level of evidence III
Strength of recommendation: Strong, for
Level of agreement: 100%
Bowel management programme should comprise individualized care based on the symptom profile, local recommendations and values/preferences of the patient/carer(s)
• The goal of bowel management is to achieve regular and complete colonic emptying at predictable intervals
• Options include regular retrograde enemas or antegrade colonic irrigation via an antegrade continence enema appendicostomy (ACE) or cecostomy +/− dietary modifications +/− laxatives
• Psychological support can assist patients and families in coping with symptoms
• An enterostomy may be required in isolated cases for intractable symptoms
Level of evidence III
Strength of recommendation: Conditional, for
Level of agreement: 100%