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.

Antegrade Continence Enema Alone for the Management of Functional Constipation and Segmental Colonic Dysmotility (ACE-FC): A Pediatric Colorectal and Pelvic Learning Consortium Study.

PURPOSE: The purpose of the study was to determine if antegrade continence enema (ACE) alone is an effective treatment for patients with severe functional constipation and segmental colonic dysmotility. METHODS: A retrospective study of patients with functional constipation and segmental colonic dysmotility who underwent ACE as their initial means of management. Data was collected from six participating sites in the Pediatric Colorectal and Pelvic Learning Consortium. Patients who had a colonic resection at the same time as an ACE or previously were excluded from analysis. Only patients who were 21 years old or younger and had at least 1-year follow-up after ACE were included. All patients had segmental colonic dysmotility documented by colonic manometry. Patient characteristics including preoperative colonic and anorectal manometry were summarized, and associations with colonic resection following ACE were evaluated using Fisher's exact test and Wilcoxon rank-sum test. p-Values of less than 0.05 were considered significant. Statistical analyses and summaries were performed using SAS version 9.4 (SAS Institute Inc., Cary, North Carolina, United States). RESULTS: A total of 104 patients from 6 institutions were included in the study with an even gender distribution (males n = 50, 48.1%) and a median age of 9.6 years (interquartile range 7.4, 12.8). At 1-year follow-up, 96 patients (92%) were successfully managed with ACE alone and 8 patients (7%) underwent subsequent colonic resection for persistent symptoms. Behavioral disorder, type of bowel management, and the need for botulinum toxin administered to the anal sphincters was not associated with the need for subsequent colonic resection. On anorectal manometry, lack of pelvic floor dyssynergia was significantly associated with the need for subsequent colonic resection; 3/8, 37.5% without pelvic dyssynergia versus 1/8, 12.5% (p = 0.023) with pelvic dyssynergia underwent subsequent colonic resection. CONCLUSION: In patients with severe functional constipation and documented segmental colonic dysmotility, ACE alone is an effective treatment modality at 1-year follow-up. Patients without pelvic floor dyssynergia on anorectal manometry are more likely to receive colonic resection after ACE. The vast majority of such patients can avoid a colonic resection.

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.

Evaluation and treatment of the post pull-through Hirschsprung patient who is not doing well; Update for 2022.

After operative intervention for Hirschsprung disease (HD) a child should thrive, be fecally continent, and avoid recurrent episodes of abdominal distention and enterocolitis. This is unfortunately not the case for a significant number of patients who struggle following their pull-through procedure. Many clinicians are puzzled by these outcomes as they can occur in patients who they believe have had a technically satisfactory described operation. This review presents an organized approach to the evaluation and treatment of the post HD pull-through patient who is not doing well. Patients with HD who have problems after their initial operation can have: (1) fecal incontinence, (2) obstructive symptoms, and (3) recurrent episodes of enterocolitis (a more severe subset of obstructive symptoms). After employing a systematic diagnostic approach, successful treatments can be implemented in almost every case. Patients may need medical management (behavioral interventions, dietary changes, laxatives, or mechanical emptying of the colon), a reoperation when a specific anatomic or pathologic cause is identified, or botulinum toxin when non-relaxing sphincters are the cause of the obstructive symptoms or recurrent enterocolitis.

Evaluation and Management of Persistent Problems After Surgery for Hirschsprung Disease in a Child.

PURPOSE OF REVIEW: Ideally, after operative intervention, a child born with Hirschsprung disease (HD) should thrive, achieve fecal continence, and avoid recurrent episodes of abdominal distention and enterocolitis. However, a significant number of patients continue to struggle following their pull-through procedure. The purpose of this review is to present an organized and practical approach to the evaluation and management of the symptomatic patient post pull-through operation for HD. RECENT FINDINGS: Children diagnosed with HD who are not doing well after their initial operation can be categorized in three distinct groups: (1) those that have fecal incontinence, (2) those with obstructive symptoms, and (3) those with recurrent episodes of enterocolitis. It is important to have a systematic diagnostic approach for these patients based on a comprehensive protocol. All three of these patient groups can be treated with a combination of either medical management, reoperation when a specific anatomic or pathologic etiology is identified, or botulinum toxin for non-relaxing sphincters contributing to the obstructive symptoms or recurrent enterocolitis. For patients not doing well after their initial pull-through, a systematic workup should be employed to determine the etiology. Once identified, a multidisciplinary and organized approach to management of the symptomatic patients can alleviate most post pull-through symptoms.

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.

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.

High Cost and Low Yield: The Diagnostic Evaluation of Rumination Syndrome in Pediatrics.

OBJECTIVE: To document the use of diagnostic testing in adolescents who ultimately were diagnosed with rumination syndrome, a functional gastrointestinal disorder. We examined the diagnostic yield of each test as well as the associated costs, and we determined if any demographic or illness-related variables impacted the magnitude of the work-up. STUDY DESIGN: A retrospective chart review was conducted for 68 patients with rumination syndrome admitted to our inpatient treatment program. The cost and findings of patients' diagnostic investigations were gathered, as well as demographic and illness-related variables to determine factors that may be related to evaluation size. RESULTS: The most commonly used tests in the evaluation of rumination syndrome included esophagogastroduodenoscopy, gastric emptying, antroduodenal manometry, upper gastrointestinal series, and abdominal ultrasound scan. Each patient underwent an average of 8.8 tests, with the average cost for each patient's diagnostic work-up being US $19 795. Few tests were found to be beneficial in the diagnosis of rumination syndrome, and few demographic or illness variables were found to be related to the overall extent of the investigation. CONCLUSIONS: Extensive testing for rumination syndrome in adolescents is common in clinical practice, and comes at a high financial cost with low yield, likely delaying diagnosis and treatment. Symptom-based criteria should be used to make the diagnosis of rumination syndrome.

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.

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.

Childhood constipation: finally something is moving!

Recent developments in the evaluation and treatment of childhood constipation are likely to influence the way we deal with pediatric defecation disorders in the near future. Innovations in both colonic and anorectal manometry are leading to novel insights into functional defecation disorders in children. Promising results have been achieved with innovative therapies such as electrical stimulation and new drugs with targets that differ from conventional pharmacological treatments. Also, new surgical approaches, guided by manometric findings, have led to improvement in patient outcome. Finally, utilization of non-pharmacological interventions such as fiber and probiotics has been a field of particular interest in recent years. The aim of this article is to provide an update on these and other novel diagnostic and therapeutic tools related to childhood constipation.

Dexmedetomidine-ketamine sedation during upper gastrointestinal endoscopy and biopsy in a patient with Duchenne muscular dystrophy and egg allergy.

Sedation during invasive procedures provides appropriate humanitarian care as well as facilitating the completion of procedure. Although generally safe and effective, adverse effects may occur especially in patients with co-morbid diseases. In many cases, given its rapid onset and offset, propofol is chosen to provide sedation during various invasive procedures. We present a nine-year-old, 45 kg child with Duchenne muscular dystrophy (DMD) who presented for esophagogastroduodenoscopy (EGD). Given the egg allergy, which was a relative contraindication to the use of propofol, and the potential risk of malignant hyperthermia due to DMD, a combination of dexmedetomidine and ketamine was used for procedural sedation. Dexmedetomidine was administered as a loading dose of 1 µg/kg along with a single bolus dose of ketamine (1 mg/kg). This was followed by a dexmedetomidine infusion at 0.5 µg/kg/hour. The patient tolerated the procedure well and was discharged to home. Previous reports regarding the use of dexmedetomidine and ketamine for procedural sedation are reviewed and the potential efficacy of this combination is discussed.