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Introduction Anal sphincter botulinum toxin injections (BTIs) are used in the treatment of children with severe defecation disorders, including Hirschsprung disease (HD) and functional constipation (FC). Our objective was to evaluate the outcomes of BTI in these children. Materials and Methods We performed a prospective cohort study of children undergoing BTI from July 2018 to December 2018. We recorded perceived effect of the BTI, including effectiveness ranging from 0 (not at all effective) to 4 (extremely effective). In addition, we recorded symptoms and the Cleveland Clinic Constipation Score (CCCS). Data were collected at baseline and at 2 weeks, 2 months, and 4 months post-injection. Results Forty-two children (HD = 25, FC = 17) were included in the study (median age 4.3 years, IQR 2.4–7.2, 52% male). Twenty-two (88%) children with HD and eight (47%) children with FC had previously undergone a BTI. BTIs were perceived effective in 16 (76%) and 12 (71%) children with HD and eight (47%) and seven (47%) children with FC at 2-week and 2-month follow-up, respectively. Effectiveness was not rated differently between groups except at the 2-month follow-up, when patients with HD rated the BTI more effective compared to those with FC (median 2 [HD] vs. median 1 [FC], p = 0.022). Over the course of the study, 17/39 (44%) children reported self-limiting adverse effects such as fecal incontinence and pain at the injection site. Conclusion Anal sphincter BTIs can be effective in the treatment of constipation in both HD and FC patients. [ABSTRACT FROM AUTHOR]

Background: Our objective is to describe the prevalence of patients with internal anal sphincter achalasia (IASA) without Hirschsprung disease (HD) among children undergoing anorectal manometry (ARM) and their clinical characteristics. Methods: We performed a retrospective review of high‐resolution ARM studies performed at our institution and identified patients with an absent rectoanal inhibitory reflex (RAIR). Clinical presentation, medical history, treatment outcomes, and results of ARM and other diagnostic tests were collected. We compared data between IASA patients, HD patients, and a matched control group of patients with functional constipation (FC). Key results: We reviewed 1,072 ARMs and identified 109 patients with an absent RAIR, of whom 28 were diagnosed with IASA. Compared to patients with FC, patients with IASA had an earlier onset of symptoms and were more likely to have abnormal contrast enema studies. Compared to patients with HD, patients with IASA were more likely to have had a normal timing of meconium passage, a later onset of symptoms, and were diagnosed at an older age. At the latest follow‐up, the majority of patients diagnosed with IASA (54%) were only using oral laxatives. Over half of patients with IASA had been treated with anal sphincter botulinum toxin injection, and 55% reported a positive response. Conclusions and Inferences: Patients diagnosed with IASA may represent a more severe patient population compared to patients with FC, but have a later onset of symptoms compared to patients with HD. They may require different treatments for their constipation and deserve further study. [ABSTRACT FROM AUTHOR]

Objectives: To investigate the clinical effectiveness and patient experience of transanal irrigation (TAI) in children with constipation or fecal incontinence., Methods: Combined retrospective and cross-sectional study including pediatric patients who used a Navina TAI system. We retrospectively collected baseline characteristics and data on treatment success at 1- and 6-month follow-up (FU). Treatment success was defined as defecating at least 3 times per week and having less than 1 episode of fecal incontinence per week. We cross-sectionally assessed health-related quality of life (HRQoL), treatment adherence, treatment satisfaction (Treatment Satisfaction Questionnaire for Medication [TSQM]), illness perceptions, medication beliefs, and patient empowerment with validated questionnaires., Results: Thirty-four patients were included (median age at start TAI: 11 years old [range, 6-18]), 32 in the retrospective review, and 26 in the cross-sectional survey (median of 3 years after initiation). Most patients were diagnosed with functional constipation (n = 26; 76%) or a neurogenic bowel disorder (n = 6; 18%). Treatment success rates significantly improved at each FU compared with baseline (baseline: 4/25 [16%]; 1-month FU: 12/16 [75%], P = 0.008; 6-month FU: 11/18 [61%], P = 0.016; cross-sectional FU: 13/26 [50%], P = 0.008). HRQoL scores were high (PedsQL median, 73 [IQR, 54-85]). Adherence (defined as Medication Adherence Report Scale [MARS] ≥ 23) was low (36%), whereas TSQM effectiveness scores were high (median, 69 [IQR, 47-86]). The majority of children (61%) reported an increase in independence since TAI treatment. Patient empowerment (GYPES) levels were similar to those reported in children with other chronic conditions., Conclusions: TAI with a Navina system is an effective bowel management system for children with intractable constipation or fecal incontinence., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 The Author(s). Published by Wolters Kluwer on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)