Management of the child with refractory constipation.

BACKGROUND: Caring for children with constipation refractory to conventional treatment can be challenging and management practices vary widely. AIMS: To review recent advances in the evaluation and treatment of children with refractory constipation and to propose an algorithm that incorporates the latest evidence and our institutional experience. METHODS: We performed a literature review on diagnostic tests and treatment options for children with refractory constipation. RESULTS: Evaluation of a child with refractory constipation seeks to better understand factors contributing to an individual child's presentation. Anorectal manometry evaluating for a rectal evacuation disorder and colonic manometry evaluating for colonic dysmotility can guide subsequent treatment. For the child who has not responded to conventional treatment, a trial of newer medications like linaclotide can be helpful. Transanal irrigation offers a safe and effective alternative for families able to administer daily rectal treatment. Despite mixed evidence in children, pelvic floor biofeedback therapy can help some children with pelvic floor dyssynergia. For younger children unable to cooperate with pelvic floor therapy, or older children with refractory symptoms, internal anal sphincter botulinum toxin injection can be beneficial. Antegrade continence enema treatment can be effective for children with either normal colonic motility or segmental dysmotility. Sacral nerve stimulation is generally reserved for symptoms that persist despite antegrade continence enemas, particularly if faecal incontinence is prominent. In more severe cases, temporary or permanent colonic diversion and segmental colonic resection may be needed. CONCLUSIONS: Recent advances offer hope for children with refractory constipation.

Current practice in the care of children with functional constipation: What is the hold up?

OBJECTIVES: For children with intractable functional constipation (FC), there are no evidence-based guidelines for subsequent evaluation and treatment. Our objective was to assess the practice patterns of a large, international cohort of pediatric gastroenterologists. METHODS: We administered a survey to physicians who attended the 2nd World Congress of Pediatric Neurogastroenterology and Motility held in Columbus, Ohio (USA) in September 2023. The survey included 29 questions on diagnostic testing, nonpharmacological and pharmacological treatment, and surgical options for children with intractable FC. RESULTS: Ninety physicians from 18 countries completed the survey. For children with intractable FC, anorectal manometry was the most commonly used diagnostic test. North American responders were more likely than Europeans to use stimulant laxatives (97% vs. 77%, p = 0.032), prosecretory medications (69% vs. 8%, p < 0.001), and antegrade continence enemas (ACE; 83% vs. 46%, p = 0.009) for management. Europeans were more likely than North Americans to require colonic transit testing before surgery (85% vs. 30%, p < 0.001). We found major differences in management practices between Americans and the rest of the world, including use of prosecretory drugs (73% vs. 7%, p < 0.001), anal botulinum toxin injections (81% vs. 58%, p = 0.018), ACE (81% vs. 58% p = 0.018), diverting ileostomies (56% vs. 26%, p = 0.006), and colonic resections (42% vs. 16%, p = 0.012). No differences were found when respondents were compared by years of experience. CONCLUSIONS: Practice patterns in the evaluation and treatment of children with intractable FC differ widely among pediatric gastroenterologists from around the world. A clinical guideline regarding diagnostic testing and surgical decision-making is needed.

Anal Botulinum Toxin in Children with Hirschsprung Disease and Functional Constipation: A Prospective Cohort study.

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.

Antegrade continence enemas in children with functional constipation and dyssynergic defecation: Go or no go?

OBJECTIVE: To assess outcomes of children with functional constipation after antegrade continence enemas (ACEs) and determine if pre operative anorectal manometry (AMAN) findings, including dyssynergic defecation, are associated with outcomes. METHODS: A retrospective review of pediatric patients with functional constipation who received a Malone appendicostomy or cecostomy after failed medical management was conducted. Patients were included if they had AMAN data prior to their operation. Patients that underwent colonic resection were excluded. Demographics, clinical characteristics, manometry results, and post ACE outcomes were obtained. Descriptive statistics were performed. RESULTS: Thirty-nine patients were identified with median age at ACE of 8.9 years (IQR: 7.2-12.6) and median follow-up of 2.5 years (IQR: 1.8-3.2 years). Twenty patients (51%) were female and most (35, 92%) were White. All patients had severe constipation prior to ACE and 59% had fecal incontinence. Thirty-four patients (87%) received a Malone and 5 (13%) received a cecostomy. Post ACE, 35 (90%) were clean with daily flushes and 6 (15%) eventually successfully transitioned to laxatives only. Awake AMAN and balloon expulsion test were performed in 15 patients, with 14 (93%) displaying evidence of dyssynergic defecation. Twelve of 14 of patients (86%) with dyssynergia were clean with ACE at follow-up. because of the majority of patients being clean post ACE, there was limited power to detect predictors of poor outcomes. CONCLUSIONS: ACEs are successful treatment options for patients with severe constipation and fecal incontinence, including those with dyssynergic defecation. Larger studies are needed to identify factors predictive of poor outcomes. LEVEL OF EVIDENCE: III.

The Use of Linaclotide in Children with Functional Constipation or Irritable Bowel Syndrome: A Retrospective Chart Review.

BACKGROUND: Linaclotide is a well-tolerated and effective agent for adults with functional constipation (FC) or irritable bowel syndrome with constipation (IBS-C). However, data in children are lacking. The aim of this study is to examine the efficacy and safety of linaclotide in children. METHODS: We performed a retrospective review of children < 18 years old who started linaclotide at our institution (Nationwide Children's Hospital, Columbus, Ohio). We excluded children already using linaclotide or whom had an organic cause of constipation or abdominal pain. We recorded information on patient characteristics, medical and surgical history, symptoms, clinical response, course of treatment, and adverse events at baseline, first follow-up, and after 1 year of linaclotide use. A positive clinical response was based on the physician's global assessment of symptoms at the time of the visit as documented. RESULTS: We included 93 children treated with linaclotide for FC (n = 60) or IBS-C (n = 33); 60% were female; median age was 14.7 years (IQR 13.2-16.6). Forty-five percent of patients with FC and 42% with IBS-C had a positive clinical response at first follow-up a median of 2.5 and 2.4 months after starting linaclotide, respectively. Approximately a third of patients experienced adverse events and eventually 27% stopped using linaclotide due to adverse events. The most common adverse events were diarrhea, abdominal pain, nausea, and bloating. CONCLUSION: Nearly half of children with FC or IBS-C benefited from linaclotide, but adverse events were relatively common. Further prospective, controlled studies are needed to confirm these findings and to identify which patients are most likely to benefit from linaclotide.

Comparison of antegrade continence enema treatment and sacral nerve stimulation for children with severe functional constipation and fecal incontinence.

BACKGROUND: To compare antegrade continence enema (ACE) treatment and sacral nerve stimulation (SNS) in children with intractable functional constipation (FC) and fecal incontinence (FI). METHODS: We performed a retrospective review of children 6-18 years old with FC and FI treated with either ACE or SNS at our institution. We recorded symptoms at baseline, 6 months, 12 months, 24 months, and their most recent visit after starting treatment. We compared improvement in FI, bowel movement (BM) frequency, abdominal pain, laxative use, and complications. Patients were contacted to evaluate perceived benefit using the Glasgow Children's Benefit Inventory. KEY RESULTS: We included 23 patients treated with ACE (52% female, median age 10 years) and 19 patients treated with SNS (74% female, median age 10 years). Improvement in FI was greater with SNS than ACE at 12 months (92.9% vs 57.1%, P = .03) and 24 months (100% vs 57.1%, P = .02). Improvement in BM frequency was greater with ACE, and children were more likely to discontinue laxatives at all follow-up time points (all P < .05). Improvement in abdominal pain was greater with ACE at the most recent visit (P < .05). Rate of complications requiring surgery was similar between groups (26.3% vs 21.7%). Benefit was reported in 83.3% and 100% of ACE and SNS groups, respectively (NS). CONCLUSIONS AND INFERENCES: Although both ACE and SNS can lead to durable improvement in children with FC and FI, SNS appears more effective for FI and ACE more effective in improving BM frequency and abdominal pain and in discontinuation of laxatives.

Anal sphincter botulinum toxin injection in children with functional anorectal and colonic disorders: A large institutional study and review of the literature focusing on complications.

BACKGROUND AND AIM: Botulinum toxin (botox) is a commonly used treatment for functional anorectal and colonic disorders. Although generally regarded as safe, complications associated with botox injection into the anal sphincters in children with severe defecation disorders are not well described. We aimed to review our institutional experience and the existing literature to better understand the safety of this practice. METHODS: We performed a retrospective review of pediatric patients undergoing botox administration into the anal sphincter for treatment of a variety of defecation disorders between 2014 and 2018. Additionally, we performed a review of all published literature reporting complications from botox injection in this patient population. RESULTS: 881 patients ranging from 5 weeks to 19.7 years underwent a total of 1332 botox injections including our institution (332 patients/526 injections) and the reviewed series (549 patients/806 injections). Overall, complications were seen after 9 (0.7%) injections and included urinary incontinence (n = 5), pelvic muscle paresis (n = 2), perianal abscess (n = 1), pruritis ani (n = 1), and rectal prolapse (n = 1). Patient age, weight, and diagnosis were not associated with an increased rate of complication in our institutional experience. All complications were self-limited and did not require intervention. There were no episodes of systemic botulinum toxicity. CONCLUSION: Botox injection into the anal sphincters is accepted practice in children with Hirschsprung disease, severe functional constipation, and internal anal sphincter achalasia and appears to be safe from this review. The precise dosing and age at which complications are more likely to arise could not be ascertained and require further study. LEVEL OF EVIDENCE: IV TYPE OF STUDY: Retrospective cohort study.

A comparison of Malone appendicostomy and cecostomy for antegrade access as adjuncts to a bowel management program for patients with functional constipation or fecal incontinence.

BACKGROUND: Appendicostomy and cecostomy are two approaches for antegrade enema access for children with severe constipation or fecal incontinence as adjuncts to a mechanical bowel management program. Each technique is associated with a unique set of complications. The purpose of our study was to report the rates of various complications associated with antegrade enema access techniques to help guide which option a clinician offers to their patients. METHODS: We reviewed all patients in our Center who received an appendicostomy or cecostomy from 2014 to 2017 who were participants in our bowel management program. RESULTS: 204 patients underwent an antegrade access procedure (150 appendicostomies and 54 cecostomies). Skin-level leakage (3% vs. 22%) and wound infections (7% vs. 28%) occurred less frequently in patients with appendicostomy compared to cecostomy. Nineteen (13%) appendicostomies required revision for stenosis, 4 (3%) for mucosal prolapse, and 1 (1%) for leakage. The rates of stenosis (33 vs. 12%) and wound infection (13 vs. 6%) were higher in patients who received a neoappendicostomy compared to an in situ appendicostomy. Intervention was needed in 19 (35%) cecostomy patients, 15 (28%) for an inability to flush or a dislodged tube, and 5 for major complications including intraperitoneal spillage in 4 (7%) and 1 (2%) for a tube misplaced in the ileum, all occurring in patients with a percutaneously placed cecostomy. One appendicostomy (1%) patient required laparoscopic revision after the appendicostomy detached from the skin. CONCLUSION: Patients had a lower rate of minor and major complications after appendicostomy compared to cecostomy. The unique complication profile of each technique should be considered for patients needing these procedures as an adjunct to their care for constipation or fecal incontinence. TYPE OF STUDY: Retrospective comparative study. LEVEL OF EVIDENCE: Level III.

Sacral nerve stimulation allows for decreased antegrade continence enema use in children with severe constipation.

BACKGROUND: Sacral nerve stimulation (SNS) can be beneficial for children with constipation, but no studies have focused on children with constipation severe enough to require antegrade continence enemas (ACEs). Our objective was to evaluate the efficacy of SNS in children with constipation treated with ACE. METHODS: Using a prospective patient registry, we identified patients <21years old who were receiving ACE prior to SNS placement. We compared ACE/laxative usage, PedsQL Gastrointestinal Symptom Scale (GSS), Fecal Incontinence Quality of Life Scale (FIQL), Fecal Incontinence Severity Index (FISI), and Vancouver Dysfunctional Elimination Syndrome Score (DES) at baseline and progressive follow-up time intervals. RESULTS: Twenty-two patients (55% male, median 12years) were included. Median ACE frequency decreased from 7 per week at baseline to 1 per week at 12months (p<0.0001). Ten children (45%) had their cecostomy/appendicostomy closed. Laxative use, GSS, FIQL, and DES did not change. FISI improved over the first 12months with statistical significance reached only at 6months (p=0.02). Six (27%) children experienced complications after SNS that required further surgery. CONCLUSIONS: In children with severe constipation dependent on ACE, SNS led to a steady decrease in ACE usage with nearly half of patients receiving cecostomy/appendicostomy closure within 2years. LEVEL OF EVIDENCE: IV.

Neurostimulation of the gastrointestinal tract in children: is it time to shock the gut?

PURPOSE OF REVIEW: The use of neurostimulation for treatment of gastrointestinal disorders has been growing over the past two to three decades. Our objective is to review current applications of neurostimulation in the treatment of gastrointestinal disorders with an emphasis on the use of these treatment modalities in children. RECENT FINDINGS: Gastric electrical stimulation can lead to symptomatic improvement in children with chronic nausea and vomiting refractory to conventional treatment, and a recent report of long-term outcomes is encouraging. Sacral nerve stimulation can be effective in the treatment of children with constipation and fecal incontinence refractory to conventional treatment, and patient satisfaction with treatment remains high despite the risk of complications requiring further surgery. Abdominal transcutaneous electrical stimulation and posterior tibial nerve stimulation are noninvasive neurostimulation techniques that may be effective in the treatment of children with constipation and fecal incontinence. SUMMARY: Although neurostimulation-based treatments appear promising and offer advantages compared to more invasive surgical treatment options, evidence for their benefit in children remains limited. High-quality studies demonstrating safety and efficacy and a better understanding of the mechanism of each modality are needed before there is more widespread acceptance of neurostimulation in the treatment of children with gastrointestinal disorders.

Surgical decision-making in the management of children with intractable functional constipation: What are we doing and are we doing it right?

BACKGROUND: Children with intractable functional constipation (FC) may eventually require surgery, often guided by motility testing. However, there are no evidence-based guidelines for the surgical management of intractable FC in children. AIM: To assess the diagnostic and surgical approach of pediatric surgeons and pediatric gastroenterologists towards children with intractable FC. METHODS: A survey was administered to physicians attending an international conference held simultaneously in Columbus (Ohio, USA) and Nijmegen (the Netherlands). The survey included 4 questions based on cases with anorectal and colonic manometry results. RESULTS: 74 physicians completed the questionnaire. Anorectal manometry was used by 70%; 52% of them would consider anal sphincter botulinum toxin injections for anal achalasia and 21% would use this to treat dyssynergia. Colonic manometry was used by 38%; 57% of them reported to use this to guide surgical decision-making. The surgical approach varied considerably among responders answering the case questions based on motility test results; the most commonly chosen treatments were antegrade continence enemas and anal botulinum injections. CONCLUSION: Surgical decision-making for children with intractable FC differs among physicians. There is a need for clinical guidelines regarding the role of anorectal and colonic manometry in surgical decision-making in children with intractable FC.