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

BackgroundSacral 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.MethodsUsing 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.ResultsTwenty-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.ConclusionsIn 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 evidenceIV

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

Surgical options for the management of severe functional constipation in children

© 2016 Wolters Kluwer Health, Inc. Purpose of review Constipation is a very common problem in pediatrics with both the severity of presentations and treatments varying across a broad spectrum. The majority of children with functional constipation are managed successfully without the need for specialized testing and surgical intervention. Those who present with intractable constipation, with or without fecal soiling, require referrals for motility testing that helps determine both medical and surgical management, and interventions. The literature was reviewed for publications on surgical approaches to children with severe constipation, including assessing the quality and levels of evidence and the use of objective measures to determine outcomes. Recent findings There is very little in the way of recent studies evaluating surgical indications or treatment approaches for functional constipation, apart from one systematic review and one recent expert review. Although the systematic review was published in the last year, most of the studies it analyzes are older. The vast majority of studies comprise level 4 and 5 evidence. Summary The indication for most surgical procedures is \u27failed\u27 medical management, yet no standardized definition for this exists. Many surgical procedures are proposed with little evidence to show outcomes. We recommend that the surgical evaluation and treatment of children with constipation needs to be protocolized and studied in a prospective manner using validated outcomes measures. Our center\u27s current protocol is described

Childhood constipation: Finally something is moving!

© 2015 Taylor & Francis. 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

Update on pediatric gastroparesis: A review of the published literature and recommendations for future research

Background Due to scarcity of scientific literature on pediatric gastroparesis, there is a need to summarize current evidence and identify areas requiring further research. The aim of this study was to provide an evidence‐based review of the available literature on the prevalence, pathogenesis, clinical presentation, diagnosis, treatment, and outcomes of pediatric gastroparesis. Methods A search of the literature was performed using the Preferred Reporting Items for Systematic Reviews and Meta‐analyses guidelines with the following databases: PubMed, EMBASE, Cochrane Database of Systematic Reviews, Database of s of Reviews of Effects, Cochrane Central Register of Controlled Trials, and Web of Science. Two independent reviewers screened s for eligibility. Key Results Our search yielded 1085 original publications, 135 of which met inclusion criteria. Most articles were of retrospective study design. Only 12 randomized controlled trials were identified, all of which were in infants. The prevalence of pediatric gastroparesis is unknown. Gastroparesis may be suspected based on clinical symptoms although these are often non‐specific. The 4‐hour nuclear scintigraphy scan remains gold standard for diagnosis despite lack of pediatric normative comparison data. Therapeutic approaches include dietary modifications, prokinetic drugs, and postpyloric enteral tube feeds. For refractory cases, intrapyloric botulinum toxin and surgical interventions such as gastric electrical stimulation may be warranted. Most interventions still lack rigorous supportive data. Conclusions Diagnosis and treatment of pediatric gastroparesis are challenging due to paucity of published evidence. Larger and more rigorous clinical trials are necessary to improve outcomes. Overview of suggested, step‐wise approach to pediatric gastroparesis

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

Do we need an extra dimension? A pilot study on the use of three-dimensional anorectal manometry in children with functional constipation

Background: Recently, a new high-definition (or three-dimensional “3D”) high-resolution anorectal manometry (3D-ARM) catheter has been introduced. This catheter allows for a more detailed visualization of the anal canal. However, its clinical utility and tolerability in children with constipation are unknown. Our primary objective was to evaluate the agreement between findings from solid-state high-resolution anorectal manometry (HR-ARM) and 3D-ARM. Secondary objectives were to investigate if 3D-ARM has additional value over HR-ARM and to evaluate patient and provider experience. Methods: Prospective pilot study including children (8–18 years of age) with functional constipation scheduled for anorectal manometry. Children underwent HR-ARM and 3D-ARM consecutively. We compared manometry results of both procedures and collected data on patient and provider experience via self-developed questionnaires. Key Results: Data of ten patients were analyzed (60% female, median age 14.9 years). In the majority of patients, ARMs were performed awake (n = 8, 80%). In two patients, the recto-anal inhibitory reflex (RAIR) was visualized during HR-ARM but not during 3D-ARM. Anal canal resting pressures were significantly higher during 3D-ARM compared to HR-ARM (median 77 mmHg [IQR 59–94] vs. 69 mmHg [IQR 51–91], respectively, p = 0.037). No significant anatomical or muscular abnormalities were visualized during the 3D-ARM. The majority of children identified the 3D-ARM as the more unpleasant (5/7 [71%]) and more painful procedure (6/7 [86%]) and therefore preferred the HR-ARM (4/7 [57%]). Conclusions & Inferences: In our patient sample, 3D-ARM was associated with more discomfort without providing more useful information and even resulted in an inconsistent visualization of the RAIR