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% |