Lack of Agreement on How to Use Antegrade Enemas in Children.

OBJECTIVES: The aim of the present study was to provide an overview of the existing literature regarding the outcomes of the antegrade continence enema (ACE) procedure and to assess the present practices of physicians worldwide regarding the use of the ACE. METHODS: A search of the MEDLINE database was performed using the following criteria: having a clear definition of "successful outcome," published in full manuscript form, sample size >20 patients, age <25 years. We then conducted a survey among 23 pediatric gastroenterologists and surgeons worldwide who were known to use the ACE using an 18-item questionnaire. RESULTS: A total of 21 articles met the inclusion criteria. Successful outcomes were reported in 15% to 100%. Thirteen studies classified the outcome as full continence (success) or incontinence (failure), with a mean successful outcome of 75.6%. The 23 physicians who completed the questionnaire differed in their opinions about indications and mandatory preoperative testing. Constipation with (78%) or without (91%) fecal incontinence, anorectal malformations (96%), and spinal abnormalities (100%) were considered suitable indications for the ACE by the majority. There was less agreement regarding the required preoperative diagnostic workup. Most physicians (70%) start infusions using saline solutions and do not add a stimulant laxative to the cleansing solution. DISCUSSION: There is a wide variation in the reported outcome of the ACE procedure and in the way success is defined. The survey identifies important differences among physicians using the ACE. Consensus on optimal use of the ACE could improve outcome of this treatment option.

The value of fluoroscopic defecography in the diagnostic and therapeutic management of defecation disorders in children.

BACKGROUND: Defecography is a study to assess anorectal function during evacuation. OBJECTIVE: To investigate the value of fluoroscopic defecography in directing diagnostic and therapeutic management in children with defecation disorders. MATERIALS AND METHODS: We reviewed all fluoroscopic defecography studies performed (2003-2009) in children with defecation problems and normal anorectal motility studies. Results were classified into three groups: (1) normal pelvic floor function; (2) pelvic floor dyssynergia, including incomplete relaxation of pelvic musculature, inconsistent change in anorectal angle and incomplete voluntary evacuation; (3) structural abnormality, including excessive pelvic floor descent with an intra-rectal intussusception, rectocele or rectal prolapse. RESULTS: We included 18 patients (13 boys, median age 9.1 years). Indication for fluoroscopic defecography was chronic constipation in 56%, fecal incontinence in 22% and rectal prolapse in 22%. Defecography showed pelvic floor dyssynergia in 9 children (50%), a structural abnormality in 4 (22%) and normal pelvic floor function in 5 (28%). In 12 children (67%) the outcome of fluoroscopic defecography directly influenced therapeutic management. After defecography 4 children (22%) were referred for anorectal biofeedback treatment, 4 children (22%) for surgery, 2 children (11%) for additional MR defecography, and 1 child to the psychology department, and medication was changed in 1 child. In 6 children (33%) the result did not change the management. In 9 children (75%) the change of management was successful. CONCLUSIONS: Fluoroscopic defecography can be a useful tool in understanding the pathophysiology and it may provide information that impacts management of children with refractory defecation disorders.

Adolescent rumination syndrome.

Rumination syndrome is the non-purposeful regurgitation of recently ingested food from the stomach to the mouth, where it is either expelled or reswallowed. Adolescent rumination syndrome (ARS) is a rare condition of which many physicians are unaware. Patients often are misdiagnosed or undergo costly testing, and as a result, diagnosis and treatment are often delayed. While ARS is not life-threatening, it does have medical and emotional effects on the patient and the patient's family. Diagnosis of ARS is based upon the Rome III diagnostic criteria. Antroduodenal manometry, while not required for a diagnosis, can be helpful to confirm the diagnosis. The pathogenesis of this disorder is complex and not well understood. However, because of its behavioral component, treatment of ARS requires a multidisciplinary approach that includes both medical management of symptoms and implementation of strategies that address behavioral, psychological, and general quality-of-life components of the disorder.

Ten-year experience using antegrade enemas in children.

OBJECTIVE: To describe a single-center, 10-year experience with the use of antegrade enemas. STUDY DESIGN: Retrospective analysis of 99 patients treated with antegrade enemas at Nationwide Children's Hospital. RESULTS: Study subjects (median age 8 years) were followed for a mean time of 46 months (range 2-125 months) after cecostomy placement. Seventy-one patients had the cecostomy placed percutaneously and 28 by surgery. Thirty-five patients had functional constipation and 64 patients an organic disease (spinal abnormalities, cerebral palsy, imperforate anus, Hirschsprung's disease). While using antegrade enemas, 71% became symptom-free, in 20 subjects symptoms improved, in 2 subjects symptoms did not change, and in 7 subjects symptoms worsened. Poor outcome was associated with surgical placement of the cecostomy (P < .001), younger age (P = .02), shorter duration of symptoms (P = .01), history of Hirschsprung's disease (P = .05), cerebral palsy (P = .03), previous abdominal surgery (P = .001), and abnormal colonic manometry (P = .004). In 88%, successful irrigation solution included use of a stimulant laxative, and subjects who used a stimulant did significantly better (P < .001) than subjects who started without a stimulant. In 13 patients, the cecostomy was removed 49.7 months after placement without recurrence of symptoms. Major complications occurred in 12 patients and minor complications in 47. CONCLUSIONS: Antegrade enemas represent a successful and relatively safe therapeutic option in children with severe defecatory disorders. Prognostic factors are identified.

Gastroesophageal reflux in cystic fibrosis: current understandings of mechanisms and management.

Cystic fibrosis (CF) is an inherited disease that affects both the lungs and the digestive system in children and adults. Thick mucus fills the gut and blocks lumens of the pancreas and hepatobiliary systems, creating insufficient pancreas function and liver disease. Chronic gastrointestinal (GI) complications, including intestinal obstruction, occur in neonates, and poor digestion and gastroesophageal reflux disease (GERD) in children. Although GI symptoms tend to improve with age, CF and associated GERD eventually create respiratory insufficiency; the only available treatment option at this stage is a bilateral lung transplant, which carries considerable morbidity and mortality. While GERD may reoccur as a complication of lung transplantation, GERD symptoms are often reduced following a fundoplication.

Esophageal impedance monitoring for gastroesophageal reflux.

Dual pH-multichannel intraluminal impedance (pH-MII) is a sensitive tool for evaluating overall gastroesophageal reflux disease, and particularly for permitting detection of nonacid reflux events. pH-MII technology is especially useful in the postprandial period or at other times when gastric contents are nonacidic.pH-MII was recently recognized by the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition as being superior to pH monitoring alone for evaluation of the temporal relation between symptoms and gastroesophageal reflux. In children, pH-MII is useful to correlate symptoms with reflux (particularly nonacid reflux), to quantify reflux during tube feedings and the postprandial period, and to assess efficacy of antireflux therapy. This clinical review is simply an evidence-based overview addressing the indications, limitations, and recommended protocol for the clinical use of pH-MII in children.

Tegaserod use in children: a single-center experience.

BACKGROUND: Tegaserod is increasingly prescribed by pediatric gastroenterologists even though there are few published data concerning its use in children. The aim of this study was to describe the authors' experience with tegaserod in children. PATIENTS AND METHODS: Patients treated with tegaserod from 2004 through 2006 were included in this study. Defecation and fecal incontinence frequency and global assessment of relief of symptoms were assessed. RESULTS: Seventy-two patients (44 girls) ranging in age from 1.1 to 18.3 years constitute the patient sample of this report. The median age was 10 years and the median follow-up after initiation of tegaserod treatment was 11.3 months (range 2.3-45.2 months). Indications to prescribe tegaserod were constipation (58%) and a variety of other conditions including functional dyspepsia or inflammatory bowel disease (42%). Defecation frequency increased after tegaserod use (1 vs 7/week, P < 0.001) and presence of fecal incontinence decreased (47% vs 23%, P < 0.001) in the constipation group. Parents rated relief of constipation as moderate or significant in 71% of cases in the constipation group. In the group with other indications to start tegaserod therapy, moderate or significant relief of abdominal pain and bloating was noted in 64% and 68% of patients, respectively. The median dose of tegaserod prescribed was 0.22 mg x kg x day (range 0.05-0.87 mg x kg(-1) x day(-1)). Adverse events were observed in 32% of the patients. The most common side effects were self-limiting diarrhea (20%) and abdominal pain (8%). Only one patient discontinued tegaserod because of side effects; this patient experienced pain at his cecostomy site. CONCLUSIONS: Tegaserod seems to relieve a variety of functional gastrointestinal symptoms in children. Further randomized controlled studies are needed to support the specific pediatric target of prescribing tegaserod.

Constipation: Beyond the Old Paradigms.

Constipation is a common problem in children. Although most children respond to conventional treatment, symptoms persist in a minority. For children with refractory constipation, anorectal and colonic manometry testing can identify a rectal evacuation disorder or colonic motility disorder and guide subsequent management. Novel medications used in adults with constipation are beginning to be used in children, with promising results. Biofeedback therapy and anal sphincter botulinum toxin injection can be considered for children with a rectal evacuation disorder. Surgical management of constipation includes the use of antegrade continence enemas, sacral nerve stimulation, and colonic resection.

Artifact Rejection Methodology Enables Continuous, Noninvasive Measurement of Gastric Myoelectric Activity in Ambulatory Subjects.

The increasing prevalence of functional and motility gastrointestinal (GI) disorders is at odds with bottlenecks in their diagnosis, treatment, and follow-up. Lack of noninvasive approaches means that only specialized centers can perform objective assessment procedures. Abnormal GI muscular activity, which is coordinated by electrical slow-waves, may play a key role in symptoms. As such, the electrogastrogram (EGG), a noninvasive means to continuously monitor gastric electrical activity, can be used to inform diagnoses over broader populations. However, it is seldom used due to technical issues: inconsistent results from single-channel measurements and signal artifacts that make interpretation difficult and limit prolonged monitoring. Here, we overcome these limitations with a wearable multi-channel system and artifact removal signal processing methods. Our approach yields an increase of 0.56 in the mean correlation coefficient between EGG and the clinical "gold standard", gastric manometry, across 11 subjects (p < 0.001). We also demonstrate this system's usage for ambulatory monitoring, which reveals myoelectric dynamics in response to meals akin to gastric emptying patterns and circadian-related oscillations. Our approach is noninvasive, easy to administer, and has promise to widen the scope of populations with GI disorders for which clinicians can screen patients, diagnose disorders, and refine treatments objectively.

Atomoxetine-induced hepatitis in a child.

OBJECTIVE: To report a case of hepatitis associated with atomoxetine hydrochloride use and to describe the previously-unpublished severe cases of this syndrome. CASE SUMMARY: An eight-year-old female with attention deficient hyperactive disorder (ADHD) was treated with atomoxetine hydrochloride. She complained of increased abdominal pain and occasional emesis; her transaminases and bilirubin were markedly elevated. She was admitted to a tertiary-care pediatric hospital and treated for drug-induced hepatitis. Atomoxetine was discontinued and supportive care was instituted. A liver biopsy showed hepatitis with moderate piecemeal necrosis. Clinical status and liver function tests improved over 13 days of hospitalization. DISCUSSION: To our knowledge this is the first published severe case of atomoxetine-induced hepatitis. The International Organization of Medical Science Diagnostic Scale and the Adverse Drug Reaction Probability Scale by Naranjo et al. were applied to assess causality. Both scales indicated the association of atomoxetine and hepatitis as "probable;" a positive rechallenge would have made this association "definitive." This potential serious adverse reaction should be considered in children receiving atomoxetine therapy.

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.

Sacral nerve stimulation: a promising therapy for fecal and urinary incontinence and constipation in children.

PURPOSE: This study describes our series of children with bowel and bladder dysfunction (BDD) treated with sacral nerve stimulation in order to begin to identify characteristics associated with better outcomes and guide future therapies. METHODS: Between May 2012 and February 2014, 29 patients were evaluated before and after sacral nerve stimulator (SNS) placement. A prospective data registry was developed that contains clinical information and patient-reported measures: Fecal Incontinence Qualify of Life Scale, Fecal Incontinence Severity Scale, PedsQL Gastrointestinal Symptom Scale, and Vancouver DES Symptom Scale. RESULTS: The median age of patients was 12.1 (interquartile range: 9.4, 14.3) years and the median follow-up period was 17.7 (12.9, 36.4) weeks. 93% had GI complaints and 65.5% had urinary symptoms while 7% had urologic symptoms only. The most common etiologies of BBD were idiopathic (66%) and imperforate anus (27%). Five patients required reoperation due to a complication with battery placement. Six of 11 patients (55%) with a pre-SNS cecostomy tube no longer require an antegrade bowel regimen as they now have voluntary bowel movements. Ten of eleven patients (91%) no longer require anticholinergic medications for bladder overactivity after receiving SNS. Significant improvements have been demonstrated in all four patient-reported instruments for the overall cohort. CONCLUSIONS: Early results have demonstrated improvements in both GI and urinary function after SNS placement in pediatric patients with bowel and bladder dysfunction.

Efficacy of permanent gastric electrical stimulation for the treatment of gastroparesis and functional dyspepsia in children and adolescents.

PURPOSE: Permanent gastric electrical stimulation (GES) has been performed in adults as a treatment for gastroparesis and refractory nausea and vomiting in patients who have failed medical therapy. We assessed the feasibility and clinical outcomes of permanent GES in children. METHODS: Permanent GES was performed in 16 children (10 females/6 males), median age 15 years (range 4-19 years). All patients had chronic nausea and vomiting refractory to medical therapy and met ROME III criteria for functional dyspepsia. Symptoms, route for nutrition, and satisfaction with procedure were recorded before and after permanent GES. Statistical analysis was performed using paired Student's t test. RESULTS: After permanent GES, there was significant improvement in severity of vomiting (p=0.0001), frequency of vomiting (p=0.0003), frequency of nausea (p<0.0001), and severity of nausea (p<0.0001). At the time of follow-up, 13/16 were on oral feeds exclusively, two patients on oral plus G-tube feedings, and one patient on oral plus G-tube plus intermittent TPN. CONCLUSIONS: 1). Permanent GES improved health in children with functional dyspepsia and gastroparesis who fail medical therapy. 2). No serious adverse effects of permanent GES were noted. 3). Long-term efficacy and safety of GES therapy in children need to be established.

Novel and alternative therapies for childhood constipation.

Constipation is a worldwide problem that affects many children. Treatment of constipation is largely based on clinical experience rather than on evidence-based controlled clinical trials. Stool softeners and cathartic agents in combination with behavioral interventions constitute the programs most commonly used to facilitate painless and frequent defecation. Long-term treatment is needed for most patients, and approximately 30% of children beyond puberty continue to struggle with symptoms of constipation, such as infrequent, painful evacuation of stools and fecal incontinence. Not surprisingly, chronicity of these bowel complaints may cause significant interference with the child's emotional growth and development. Development of new therapeutic strategies is necessary in order to treat these challenging patients more effectively. This review provides an overview of novel and alternative therapies, such as new drugs, surgery, and probiotics, that are being proposed for the treatment of childhood chronic constipation.

Cecostomy in children with defecation disorders.

Administration of antegrade enemas through a cecostomy is a therapeutic option for children with severe defecation disorders. The purpose of this study is to report our 4-year experience with the cecostomy procedure in 31 children with functional constipation (n = 9), Hirschsprung's disease (n = 2), imperforate anus (n = 5), spinal abnormalities (n = 8), and imperforate anus in combination with tethered spinal cord (n = 7). Data regarding complications, antegrade enemas used, symptoms, and quality of life were retrospectively obtained. Placement of cecostomy tubes was successful in 30 of 31 patients. Soiling episodes decreased significantly in children with functional constipation (P = 0.01), imperforate anus (P < 0.01), and spinal abnormalities (P = 0.04). Quality of life improved in patients with functional constipation and imperforate anus. No difference in complications was found between percutaneous and surgical placement. Use of antegrade enemas via cecostomy improved symptoms and quality of life in children with a variety of defecation disorders.

Barostat testing in children with functional gastrointestinal disorders.

Children and adolescents with chronic defecation disorders and chronic abdominal pain without obvious organic etiology form a challenging group of patients for pediatric health-care professionals. The pathophysiologic mechanisms underlying such functional gastroenterology disorders are poorly understood. Research studies on the use of the barostat have been aimed to increase our knowledge in this area. Barostat testing allows defining visceral hyper- or hyposensitivity, contractility, and compliance of the gut. This review focuses on rectal barostat studies performed in children with abdominal pain, constipation, and fecal incontinence.

Colonic manometry as predictor of cecostomy success in children with defecation disorders.

PURPOSE: The aim of this study was to define the predictive value of colonic manometry and contrast enema before cecostomy placement in children with defecation disorders. METHODS: Medical records, contrast enema, and colonic manometry studies were reviewed for 32 children with defecation disorders who underwent cecostomy placement between 1999 and 2004. Diagnoses included idiopathic constipation (n = 13), Hirschsprung's disease (n = 2), cerebral palsy (n = 1), imperforate anus (n = 6), spinal abnormality (n = 6), and anal with spinal abnormality (n = 4). Contrast enemas were evaluated for the presence of anatomic abnormalities and the degree of colonic dilatation. Colonic manometry was considered normal when high-amplitude propagating contractions (HAPC) occurred from proximal to distal colon. Clinical success was defined as normal defecation frequency with no or occasional fecal incontinence. RESULTS: Colonic manometry was done on 32 and contrast enema on 24 patients before cecostomy. At follow-up, 25 patients (78%) fulfilled the success criteria. Absence of HAPC throughout the colon was related to unsuccessful outcome (P = .03). Colonic response with normal HAPC after bisacodyl administration was predictive of success (P = .03). Presence of colonic dilatation was not associated with colonic dysmotility. CONCLUSION: Colonic manometry is helpful in predicting the outcome after cecostomy. Patients with generalized colonic dysmotility are less likely to benefit from use of antegrade enemas via cecostomy. Normal colonic response to bisacodyl predicts favorable outcome.