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Normal and optimal functioning of the gastrointestinal tract is paramount to ensure optimal nutrition through digestion, absorption and motility function. Disruptions in these functions can lead to adverse physiological symptoms, reduced quality of life and increased nutritional risk. When disruption or dysfunction of neuromuscular function occurs, motility disorders can be classified depending on whether coordination or strength/velocity of peristalsis are predominantly impacted. However, due to their nonspecific presenting symptoms and overlap with sensory disruption, they are frequently misdiagnosed as disorders of the gut-brain interaction. Motility disorders are a prevalent issue in the pediatric population, with management varying from medical therapy to psychological therapy, dietary manipulation, surgical intervention or a multimodal approach. This narrative review aims to discuss the dietary management of common pediatric motility disorders including gastroesophageal reflux, esophageal atresia, achalasia, gastroparesis, constipation, and the less common but most severe motility disorder, pediatric intestinal pseudo-obstruction.

Gastrointestinal dysmotility is a common problem in a subgroup of children with intestinal failure (IF), including short bowel syndrome (SBS) and pediatric intestinal pseudo-obstruction (PIPO). It contributes significantly to the increased morbidity and decreased quality of life in this patient population. Impaired gastrointestinal (GI) motility in IF arises from either loss of GI function due to the primary disorder (e.g., neuropathic or myopathic disorder in the PIPO syndrome) and/or a critical reduction in gut mass. Abnormalities of the anatomy, enteric hormone secretion and neural supply in IF can result in rapid transit, ineffective antegrade peristalsis, delayed gastric emptying or gastroesophageal reflux. Understanding the underlying pathophysiologic mechanism(s) of the enteric dysmotility in IF helps us to plan an appropriate diagnostic workup and apply individually tailored nutritional and pharmacological management, which might ultimately lead to an overall improvement in the quality of life and increase in enteral tolerance. In this review, we have focused on the pathogenesis of GI dysmotility in children with IF, as well as the management and treatment options.

Background: Colonic manometry with intraluminal bisacodyl infusion can be used to assess colonic neuromuscular function in children with treatment-refractory constipation. If bisacodyl does not induce high-amplitude propagating contractions (HAPCs), this can be an indication for surgical intervention. A detailed characterization of the colonic response to intraluminal bisacodyl in children with constipation may help to inform clinical interpretation of colonic manometry studies., Methods: Studies were performed in five pediatric hospitals. Analysis included identification of HAPCs, reporting HAPCs characteristics, and an area under the curve (AUC) analysis. Comparisons were performed between hospitals, catheter type, placement techniques, and site of bisacodyl infusion., Results: One hundred and sixty-five children were included (median age 10, range 1-17 years; n = 96 girls). One thousand eight hundred and ninety-three HAPCs were identified in 154 children (12.3 ± 8.8 HAPCs per child, 0.32 ± 0.21 HAPCs per min; amplitude 113.6 ± 31.5 mm Hg; velocity 8.6 ± 3.8 mm/s, propagation length 368 ± 175 mm). The mean time to first HAPC following bisacodyl was 553 ± 669 s. Prior to the first HAPC, there was no change in AUC when comparing pre- vs post-bisacodyl (Z = -0.53, P = .60). The majority of HAPCs terminated in a synchronous pressurization in the rectosigmoid. Defecation was associated with HAPCs (χ 2 (1)=7.04, P < .01). Site of bisacodyl administration, catheter type, and hospital location did not alter the response., Conclusions and Inferences: Intraluminal bisacodyl induced HAPCs in 93% of children with treatment-refractory constipation. The bisacodyl response is characterized by ≥1 HAPC within 12 minutes of infusion. The majority of HAPCs terminate in a synchronous pressurization in the rectosigmoid. Optimal clinical management based upon colonic manometry findings is yet to be determined., (© 2020 John Wiley & Sons Ltd.)

Alterations in colonic motility are implicated in the pathophysiology of bowel disorders, but high-resolution manometry of human colonic motor function has revealed that our knowledge of normal motor patterns is limited. Furthermore, various terminologies and definitions have been used to describe colonic motor patterns in children, adults and animals. An example is the distinction between the high-amplitude propagating contractions in humans and giant contractions in animals. Harmonized terminology and definitions are required that are applicable to the study of colonic motility performed by basic scientists and clinicians, as well as adult and paediatric gastroenterologists. As clinical studies increasingly require adequate animal models to develop and test new therapies, there is a need for rational use of terminology to describe those motor patterns that are equivalent between animals and humans. This Consensus Statement provides the first harmonized interpretation of commonly used terminology to describe colonic motor function and delineates possible similarities between motor patterns observed in animal models and humans in vitro (ex vivo) and in vivo. The consolidated terminology can be an impetus for new research that will considerably improve our understanding of colonic motor function and will facilitate the development and testing of new therapies for colonic motility disorders.

Pediatric gastrointestinal motility disorders represent a range of severe developmental or acquired conditions that disrupt enteric neuromuscular function. Current medical and surgical therapeutic options are very limited but recent advances have highlighted the possibility of improved or curative stem cell-based treatments. Not only has the ability to harvest, propagate and transplant human-derived enteric neural stem cells (ENSCs) been demonstrated but recent in vivo transplantation studies have confirmed that ENSCs are capable of engraftment within recipient intestine of animal models of enteric neuropathy and effecting functional rescue. Pluripotent stem cell-derived cells and pharmacological modulation of both endogenous and transplanted neural stem cells have further enhanced the exciting prospect of clinical application of such stem cell-based therapies in the near future., (Copyright © 2018. Published by Elsevier Ltd.)

The bewildering complexity of the enteric nervous system makes it susceptible to develop a wide array of motility disorders, collectively called enteric neuropathies. These gastrointestinal conditions are among the most challenging to manage, mainly given poor characterization of their etiopathophysiology and outcomes. Not surprisingly, therefore, targeted or curative therapies for enteric neuropathies are lacking and management is largely symptomatic. Nonetheless, recent advances in neurogastroenterology have witnessed improvements in established strategies, such as intestinal transplantation and the emergence of new treatments including novel drugs, electrical pacing, and manipulation of fecal microbiota, as well as stem cell and gene therapy., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background/Objectives: Gastric emptying (GE) requires precise antropyloroduodenal coordination for effective transpyloric flow, the mechanisms of which are still unclear. We aimed to correlate gastric antral function assessed by antroduodenal manometry (ADM) with GE scintigraphy (GES) for liquid feeds in children with suspected gastrointestinal dysmotility. Methods: Children who underwent both ADM and GES over a five‐year period were reviewed. ADM tracings were re‐analyzed to assess antral frequency, amplitude, and motility index (MI) pre‐prandially and postprandially. Transpyloric propagation (TPP) was defined as antegrade propagated antral activity preceding duodenal phase III of the migrating motor complex (MMC). TPP was defined as "poor" if occurring in <50% of all presented duodenal phases III. For GES, regions of interest over the whole stomach, fundus, and antrum were drawn to calculate GE half‐time (GE‐T1/2) and retention rate (RR) in each region at 1 and 2 h. Results: Forty‐seven children (median age: 7.0 years) were included. Twenty‐two had PIPO, 14 functional GI disorders, and 11 gastroparesis. Children with poor TPP had longer GE‐T1/2 (113.0 vs 66.5 min, p = 0.028), higher RR of the whole stomach and fundus at 1 h (79.5% vs 63.5%, p = 0.038; 60.0% vs 41.0%, p = 0.022, respectively) and 2 h (51.0% vs 10.5%, p = 0.005; 36.0% vs 6.5%, p = 0.004, respectively). The pre‐prandial antral amplitude of contractions inversely correlated with GE‐T1/2, RR of the whole stomach, and fundus at 2 h. Conclusions: TPP during phase III of the MMC correlated with gastric emptying of liquid and its assessment on ADM might predict abnormalities in postprandial gastric function. [ABSTRACT FROM AUTHOR]

Background: Antroduodenal manometry (ADM) and histopathology are currently employed to aid the diagnosis of pediatric intestinal pseudo‐obstruction (PIPO). Limited data are available on the reliability of ADM analysis and its correlation with histopathology. We aimed to develop a protocol for enhanced analysis of ADM contractile patterns, including a scoring system, and explore whether this provided better correlation with histopathology. Methods: Children referred with suspected PIPO between April 2012‐December 2019 who underwent both ADM and full‐thickness biopsies were included. ADM tracings were analyzed using both standard (conventional ADM) and novel (enhanced ADM) motility parameters. A novel ADM score (GLASS score) was generated based on the enhanced ADM analysis. Conventional and enhanced ADM analyses were then correlated with histopathology. Results: Forty patients were included. Using conventional clinical criteria, 29 of these were diagnosed with PIPO and the other 11 with non‐PIPO diagnoses. Twenty‐three of the PIPO patients had abnormal histopathology: 6 myopathy, 4 neuropathy, 3 neuro‐myopathy, and 10 non‐specific changes. No agreement in diagnosis was found between conventional ADM analysis and histopathology (ϰ = 0.068; p = 0.197), whereas the latter significantly correlated with enhanced ADM analysis (ϰ = 0.191; p = 0.003). The enhanced ADM score was significantly higher in PIPO versus non‐PIPO (16.0 vs. 8.0; p < 0.001). Conclusions: As opposed to conventional analysis protocols, the newly developed enhanced ADM analysis and associated score is not only able to discriminate between PIPO and non‐PIPO patients, but also between distinct histopathological pathologies. Further studies are required to assess the utility of enhanced ADM analysis in larger populations. [ABSTRACT FROM AUTHOR]

OBJECTIVES:Food allergy is thought to trigger functional constipation in children but the underlying mechanisms are still unknown. Mast cells (MCs) and their relationship with nerve fibers (NFs) in the rectal mucosa, as well as anorectal motility, were studied in children with refractory chronic constipation before and after an elimination diet for cow's milk, egg, and soy proteins.METHODS:Thirty-three children (range: 1–10.8 years) underwent anorectal manometry and suction rectal biopsy before and after 8 weeks of oligoantigenic diet. MCs and NFs were identified immunohistochemically. Quantification of MCs (%MC/area) and MCs within 10 μm of NFs (%MC-NF/area) was performed by computer-assisted analysis.RESULTS:Eighteen children responded to the diet (R-group) and fifteen did not (the NR-group). At baseline there was a significant difference in anal resting pressure (ARP; mm Hg), percentage of relaxation (%R), and residual pressure (RP; mm Hg) of anal canal during rectal distension between the R-group (66±4.1, 84.3±2.8, 10.4±2.3, respectively) and the NR-group (49±5, 92.2±1.7, 4.8±1.7, respectively; P<0.05). After the diet, significant changes in ARP, RP, and %R were observed only in the R-group (44±3.7, 93.7±1.5, 3.8±1.2, respectively; P<0.05). At baseline, the R-group showed an increase in %MC/area (8.3±0.7) and %MC-NF/area (5.2±2.6) with respect to the NR-group (5.1±0.5 and 2.3±0.4, respectively; P<0.05). After the diet, only the R-group showed a significant reduction of %MC/area and %MC-NF/area (4.4±0.5 and 2.2±0.4, respectively; P<0.001). Both ARP and RP significantly correlated with %MC/area and %MC-NF/area; %R showed a significant inverse correlation with both %MC/area and %MC-NF/area.CONCLUSIONS:In children with food allergy-related chronic constipation, an increase in both rectal MC density and spatial interactions between MCs and NFs correlates with anal motor abnormalities. These variables are significantly affected by the diet.Am J Gastroenterol 2009; 104:454–463; doi:10.1038/ajg.2008.109; published online 20 January 2009 [ABSTRACT FROM AUTHOR]