Pediatric retrograde cricopharyngeal dysfunction diagnosed by high-resolution impedance manometry.

OBJECTIVES: The inability to burp, known as retrograde cricopharyngeal dysfunction (R-CPD), was initially described in adults. The proposed clinical diagnostic criteria for R-CPD include belching inability, abdominal bloating and discomfort/nausea, postprandial chest pain, and involuntary noises. Botulinum toxin injection to the cricopharyngeal muscle has been reported to be beneficial. High-resolution esophageal impedance-manometry (HRIM) features in adolescent patients with R-CPD have not been described yet.  The aim of our study was to describe the clinical and HRIM findings of pediatric patients with R-CPD. METHODS: Clinical and manometric features of five pediatric patients diagnosed with R-CPD were reviewed. HRIM study protocol was modified to include the consumption of carbonated drink to provoke symptoms and distinctive manometric features. RESULTS: We report five female patients aged 15-20 years who presented with an inability to burp and involuntary throat sounds. HRIM revealed normal upper esophageal sphincter (UES) relaxation during swallowing, but abnormal UES relaxation with concurrent high esophageal impedance reflecting air entrapment and secondary peristalsis following the carbonated drink challenge. Four patients exhibited esophageal motility disorder. All patients reported improvement or resolution of symptoms after botulinum toxin injection to the cricopharyngeus muscle. CONCLUSIONS: Adolescents with an inability to burp, reflux-like symptoms, bloating, and involuntary throat noises should be assessed for R-CPD by pediatric gastroenterologists with HRIM. The relatively recent recognition of this novel condition is the likely reason for its under- and misdiagnosis in children.

Sacral nerve stimulation effect on colonic motility in pediatric patients.

BACKGROUND: Sacral nerve stimulation (SNS) is a minimally invasive surgical procedure used to treat refractory constipation in children. While its efficacy in improving symptoms has been studied, its effect on colonic motor function remains unclear. This case series explores SNS's impact on colonic motor function in pediatric patients with idiopathic constipation, using high-resolution colonic manometry (HRCM). METHODS: Four pediatric patients with chronic idiopathic constipation underwent SNS placement for intractable symptoms and were subsequently evaluated via HRCM. Clinical characteristics, comorbidities, treatment regimens, and outcomes were reviewed. HRCM was conducted during the SNS-off and SNS-on phases. The motility index (MI) was measured during the SNS-off (fasting and postprandial) and SNS-on phases. RESULTS: Four pediatric patients aged 8 to 21 years met the inclusion criteria. In three patients, SNS-induced high-amplitude propagating contractions (HAPCs) were noted, and in one patient, low-amplitude propagating contractions (LAPCs) were noted. In one patient, propagating contractions were induced only when SNS was turned on. MI changes with SNS-on were variable among different patients with an increase in MI in two patients after turning SNS on and a decrease in the other two compared with baseline. Adverse effects following SNS placement remained minimal across all cases. CONCLUSION: This case series is the first to report SNS effects on colonic motility evaluated by HRCM in pediatrics. We demonstrate that propagating colonic contractions are promptly induced when SNS is turned on. Although the initial effects of SNS on colonic motility were observable, additional investigation is necessary to comprehend the fundamental mechanisms and long-term effectiveness of SNS in pediatric patients.

Diagnostic yield of left-sided colonic manometry in identifying colonic dysmotility in pediatric patients.

BACKGROUND: Colonic manometry (CM) is a diagnostic procedure utilized in the evaluation of intractable constipation and involves endoscopic insertion of a manometry catheter with the tip placed in the cecum. Difficulty in advancing the colonic manometry catheter to the right colon and/or distal displacement of the catheter after appropriate placement can result in partial evaluation of the colon. Our study aimed to assess the value of limited left CM in identifying motility disorders. METHODS: We evaluated CM studies conducted at a tertiary pediatric center (2019-2022). Abnormal CM studies with catheter tips located in the cecum or ascending colon were included. KEY RESULTS: Of 161 CM studied, 68 with abnormal CM studies met inclusion criteria (29 [42.7%] females, median age 10.3 years). Pan-colonic dysmotility was noted in 29 (42.7%) studies and segmental dysmotility in 39 (57.4%) studies. Dysmotility of the descending and/or sigmoid colon was the most common segmental dysmotility (30, 76.9%). Isolated dysmotility of the ascending colon was noted only in patients with a cecostomy (6/13, 46.2%). The diagnostic sensitivity for dysmotility by left CM was 91.2%, which increased to 100% when excluding patients with cecostomy. CONCLUSIONS AND INFERENCES: Left CM is a valuable and sensitive diagnostic tool for identifying abnormal colonic motility in most pediatric patients with constipation without cecostomy. Our study results provide reassurance when the manometry catheter becomes dislodged from the cecum and moves distally. Those with cecostomy have a high prevalence of dysmotility in the ascending colon and need a complete CM to identify it.

Interpretation of Impedance Data on High-resolution Impedance Manometry Studies-A Worldwide Survey.

Background/Aims: Esophageal manometry is the gold standard for esophageal motility evaluation. High-resolution esophageal manometry with impedance (HRIM) allows concurrent assessment of bolus transit and manometry. Inconsistencies between concomitant impedance and manometry data pose a clinical dilemma and has not yet been addressed. We aim to assess interpretation trends of HRIM data among gastroenterologists worldwide. Methods: A cross-sectional study using an anonymous survey was conducted among gastroenterologists worldwide. Statistical analysis was performed to compare responses between providers. Results: We received responses from 107 gastroenterologists (26 countries). Most were adult providers (69, 64.5%), and most (77, 72.0%) had > 5 years of experience. Impedance was found to be helpful by 83 (77.6%) participants, but over 30% reported inconsistencies between impedance and manometry data. With incomplete bolus clearance and normal manometry 41 (38.7%) recommended observation, 41 (38.7%) recommended 24-hours pH-impedance, and 16 (15.1%) recommended prokinetics. With abnormal manometry and complete bolus clearance, 60 (57.1%) recommended observation while 18 (17.1%) recommended 24-hours pH impedance and 15 (14.3%) recommended prokinetics. A significant difference was found between providers from different continents in treating cases with discrepancy between impedance and manometry findings (P < 0.001). No significant differences were seen in responses between adult versus pediatric providers and between providers with different years of experience. Conclusions: There is no consensus on interpreting HRIM data. Providers' approaches to studies with inconsistencies between manometry and impedance data vary. There is an unmet need for guidelines on interpreting impedance data in HRIM studies.

High amplitude propagated contractions with Glycerin versus Bisacodyl: A within-subject comparison in children undergoing colonic manometry.

BACKGROUND: The presence of high amplitude propagated contractions (HAPCs) measured by colonic manometry (CM) reflect an intact neuromuscular function of the colon. Bisacodyl and Glycerin are colonic stimulants that induce HAPCs and are used for the treatment of constipation. HAPCs characteristics with each drug have not been compared before. We aimed to compare the HAPC characteristics with Bisacodyl and Glycerin in children undergoing CM for constipation. METHODS: This is a prospective single-center cross-over study of children aged 2-18 years undergoing CM. All patients received both Glycerin and Bisacodyl during CM. They were randomized to group A with Bisacodyl first (n = 22) and group B with Glycerin first (n = 23), with 1.5 hours in between each dose. Differences in patient and HAPC characteristics between groups were summarized using descriptive statistics and compared using Chi-square test or Wilcoxon rank sum test as appropriate. KEY RESULTS: A total of 45 patients were included. HAPCs post Bisacodyl had a longer duration of action (median of 40 vs 21.5 min, p < 0.0001), longer propagation (median of 70 vs 60 cm, p = 0.02), and more HAPCs (median of 10 vs 5, p < 0.0001) compared Glycerin. No differences were found in the HAPC amplitude and onset of action between both medications.

Evaluation of Chronic Constipation in Children With Autism Spectrum Disorder.

OBJECTIVES: Chronic constipation occurs frequently in children with autism spectrum disorder (ASD). The primary objective was to determine whether chronic constipation is associated with a higher rate of abnormal colonic motor activity in ASD children than in non-ASD children. A secondary goal was to determine if clinical variables could identify children with ASD at risk for possessing abnormal colonic motility. METHODS: A retrospective, propensity-matched, case-control study compared colonic manometry (CM) of an ASD cohort and non-ASD controls with chronic constipation. Clinical variables were evaluated as potential predictors for abnormal colonic motility. RESULTS: Fifty-six patients with ASD and 123 controls without the diagnosis of ASD who underwent CM were included. Propensity score resulted in 35 matched cohorts of ASD and controls. The rate of abnormal CM findings between ASD and matched controls (24% vs 20%, P = 0.78) did not differ significantly. A prediction model of abnormal CM that included ASD diagnosis, duration of constipation, and soiling achieved a sensitivity of 0.94 and specificity of 0.65. The risk for abnormal colonic motility increased 11% for every 1-year increase in duration of constipation. Odds for abnormal motility were 30 times higher in ASD children with soiling than controls with soiling (P < 0.0001). CONCLUSIONS: Chronic constipation does not appear to be associated with a higher rate of abnormal colonic motility in children with ASD. Clinical information of disease duration and presence of soiling due to constipation show promise in identifying patients with ASD at a greater risk for abnormal colonic motility.

Gastrocolonic Response.

PURPOSE OF REVIEW: The gastrocolonic response (GCR), is a physiologic increase in motor activity of the colon, which usually occurs within minutes following meal ingestion. Over the years several triggers that provoke GCR were recognized including gastric dilation, caloric intake, and fat component of the meal. The response is mediated by the vagal nerve and neurohumoral mechanisms, and it can be modified by several pharmacological factors. Assessment of GCR is part of high-resolution colonic manometry studies, performed in patients with suspected colonic dysmotility. This review highlights the physiologic basis of GCR as well as its clinical and diagnostic features and implementation in variable pathological conditions and clinical practice. RECENT FINDINGS: GCR has a role in patients with constipation, diabetes, dumping syndrome, bowel hyper and hypomotility and irritable bowel syndrome. Novel sensory triggers for GCR were recently recognized. GCR is a physiological response which is evaluated during colonic manometry studies. Abnormal GCR is a marker of an underlying pathology, which can provide a potential target for treatment.

Tachygastria in Preterm Infants: A Longitudinal Cohort Study.

OBJECTIVES: Tachygastria is a gastric dysrhythmia (>4 to ≤9 cycles per minute, cpm) associated with gastric hypomotility and gastrointestinal disorders. Healthy preterm infants spend more time in tachygastria than adults; however, normative values are not defined. We sought to determine the percent of time preterm infants spend in tachygastria. METHODS: We conducted a longitudinal, prospective cohort study with weekly electrogastrography (EGG) recordings in 51 preterm <34 weeks' gestation and 5 term (reference) infants. We calculated percentage recording time in tachygastria (% tachygastria) and determined the mean ± standard deviation (SD) across EGG sessions. Mixed effects model was performed to test weekly variance in % tachygastria and gestational age effect. Successive pre- and post-prandial measurements were obtained to assess reproducibility of % tachygastria. We compared time to achieve full feeds between subjects with % tachygastria within 1 SD from the mean versus % tachygastria >1 SD from mean. RESULTS: Three hundred seventy-six EGG sessions were completed (N = 56). Mean % tachygastria was 40% with SD ±5%. We demonstrated no change in % tachygastria across 9 postnatal weeks (P = 0.70) and no gestational age effect. No difference was demonstrated between successive pre- (P = 0.91) and post-prandial (P = 0.96) % tachygastria. Infants with 35%-45% tachygastria (within 1 SD from mean) had higher gestational age and less time to achieve full feeds than infants with <35% or >45% tachygastria. CONCLUSIONS: EGG is a reproducible tool to assess % tachygastria in preterm infants. Clinical significance of increased or decreased % tachygastria needs further investigation to validate if 35%-45% tachygastria is safe for feeding.

Pediatric gastrointestinal neuromodulation: A review.

Neuromodulation, also known as bioelectric neuromodulation or neurostimulation, is the therapeutic use of electrical stimulation of nerves or brain centers. Neuromodulation has been trialed in an increasing range of human diseases as well as gastrointestinal disorders. The application of neuromodulation to treat pediatric motility and functional disorders is an exciting recent development. This review aims to briefly discuss the use of neuromodulation for the treatment of pediatric gastroparesis, constipation, and visceral hyperalgesia.

Anal Sphincter Defect and Fecal Incontinence.

Anal sphincter defects can lead to fecal incontinence. The relationship between the extent of defect and continence is controversial. Magnetic resonance imaging (MRI) of the pelvis can assess anal sphincter defects. Transrectal ultrasonography (TRUS) is used to assess sphincter integrity in adults. We present a 17-year-old male with history of sexual abuse, rectal prolapse, and fecal incontinence. MRI showed a small defect that did not explain his clinical presentation. TRUS identified more extensive defects which were not picked up by MRI. The patient had rectopexy, and his rectal prolapse and fecal incontinence resolved. TRUS was superior in identifying sphincter defects compared with MRI. Our case also highlights that continence is possible despite large sphincter defects in pediatric patients. This may reflect the compensatory mechanism of residual sphincter in the absence of the aggravating factors like rectal prolapse.