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289 Surgical decision-making 14 It has been postulated that development of these abnormalities may make FC particularly difficult to treat and that patients with significant dilation of the distal colon may benefit from surgery. 24 Non-pharmacological and pharmacological treatment The non-pharmacological treatments used by our responders usually consisted of a toilet program (behavioral intervention) and a bowel diary, in compliance with the ESPGHAN- NASPGHAN recommendations. 1 Although use of a reward system has been shown to improve therapy compliance, this measure was only used by a minority of responders. 25 Furthermore, pre-, pro- or synbiotics were used as a non-pharmacological intervention by one-fifth of responders. This is surprising, since there is insufficient evidence to support this practice. 26 The pharmacological management of FC mainly consisted of osmotic laxatives, stimulant laxatives and enemas, in agreement with the ESPGHAN-NASPGHAN guidelines. 1 The regular use of enemas is not recommended in these guidelines, but these guidelines were developed for FC in general, and do not address intractable FC specifically. Prosecretory agents (e.g., lubiprostone and linaclotide) were also reported to be used by a substantial amount of responders despite the fact that neither has been tested and approved for use in children yet. It could be that a large proportion of these children with severe symptoms participate in clinical trials, as lubiprostone is currently being investigated in a multicenter randomized controlled trial, or it could be that these medications are being prescribed off- label to patients who have failed conventional pharmacological management. Anorectal manometry and surgery Anorectal manometry is most commonly used to differentiate between FC and Hirschsprung's disease, but it is also useful in detecting anal achalasia and dyssynergia. 27 In cases of intractable FC with abnormal anal sphincter pressure or function (after Hirschsprung's disease had been ruled out), most responding physicians opted for intra- anal botulinum toxin injections. Several studies, both retrospective and prospective, have shown that injection of botulinum toxin into the internal anal sphincter can be an effective treatment for these patients. 28–31 Although less commonly described, there have also been reports of successful outcomes after injection of botulinum toxin into the external anal sphincter. 32,33 Botulinum toxin causes a temporary chemical paralysis of smooth and striated muscle fibers by blocking the release of acetylcholine from neurons. The effect of botulinum toxin usually lasts for 3 to 6 months and, if necessary, repeated injections can be administered after the initial effect wears off. Other common answers to this case-based question included anal dilation and anal sphincter myectomy. Unlike botulinum toxin

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