Esophago-gastric junction findings on high resolution impedance manometry in children with esophageal atresia.

OBJECTIVES: Using high resolution impedance manometry (HRIM), this study characterized the esophago-gastric junction (EGJ) dynamics in children with esophageal atresia (EA). METHOD: Esophageal HRIM was performed in patients with EA aged less than 18 years. Objective motility patterns were analyzed, and EGJ data reported. Controls were pediatric patients without EA undergoing investigations for consideration of fundoplication surgery. RESULTS: Seventy-five patients (M:F = 43:32, median age 1 year 3 months [3 months-17 years 4 months]) completed 133 HRIM studies. The majority (64/75, 85.3%) had EA with distal tracheo-esophageal fistula. Compared with controls, liquid swallows were poorer in patients with EA, as evident by significant differences in distension pressure emptying and bolus flow time (BFT). The integrated relaxation pressure for thin liquid swallows was significantly different between EA types, as well as when comparing patients with EA with and without previous esophageal dilatations. The BFT for solid swallows was significantly different when compared with EA types. CONCLUSIONS: We have utilized HRIM in patients with EA to demonstrate abnormalities in their long-term EGJ function. These abnormalities correlate with poorer esophageal compliance and reduced esophageal peristalsis across the EGJ. Understanding the EGJ function in patients with EA will allow us to tailor long-term management to specific patients.

Mucosal impedance as a diagnostic tool for gastroesophageal reflux disease: an update for clinicians.

Mucosal impedance is a marker of esophageal mucosal integrity and a novel technique for assessing esophageal function and pathology. This article highlights its development and clinical application for gastroesophageal reflux disease (GERD), Barrett's esophagus, and eosinophilic esophagitis. A narrative review of key publications describing the development and use of mucosal impedance in clinical practice was conducted. A low mean nocturnal baseline impedance (MNBI) has been shown to be an independent predictor of response to anti-reflux therapy. MNBI predicts medication-responsive heartburn better than distal esophageal acid exposure time. Patients with equivocal evidence of GERD using conventional methods, with a low MNBI, had an improvement in symptoms following the initiation of PPI therapy compared to those with a normal MNBI. A similar trend was seen in a post fundoplication cohort. Strong clinical utility for the use of mucosal impedance in assessing eosinophilic esophagitis has been repeatedly demonstrated; however, there is minimal direction for application in Barrett's esophagus. The authors conclude that mucosal impedance has potential clinical utility for the assessment and diagnosis of GERD, particularly when conventional investigations have yielded equivocal results.

Pharyngeal tongue base augmentation for dysphagia therapy: A prospective case series in patients post head and neck cancer treatment.

BACKGROUND: Dysphagia post head and neck cancer (HNC) multimodality treatment is attributed to reduced pharyngeal strength. We hypothesized that pharyngeal tongue base augmentation for dysphagia (PAD therapy) would increase pharyngeal pressures during swallowing thereby improving swallow symptoms. METHODS: Adults with moderate-severe dysphagia post-HNC treatment had PAD therapy using a temporary filler (hyaluronic acid [HA]), with follow-up long-lasting lipofilling. Swallowing preprocedure and postprocedure was assessed with the Sydney Swallow Questionnaire (SSQ), High-Resolution Pharyngeal Manometry (HRPM), and Videofluoroscopic Swallowing Study (VFSS). Statistical comparison utilized paired tests. RESULTS: Six participants (all male; median age 64 years [IQR 56, 71]) underwent PAD therapy at a median of 47 [IQR 8, 95] months post-treatment. SSQ scores reduced from baseline (mean 1069 [95%CI 703, 1434]) to post-HA (mean 579 [76, 1081], p > 0.05), and post-lipofilling (491 [95%CI 913, 789], p = 0.003, n = 4). Individual participants demonstrated reduced Swallow Risk Index, Bolus Presence Time, and increased Upper Esophageal Sphincter opening, but mesopharyngeal contractile pressures were unchanged. VFSS measures of aspiration, residue, and severity were unchanged. CONCLUSIONS: Novel PAD therapy is safe and improves dysphagia symptoms. Biomechanical swallowing changes are suggestive of more efficacious bolus propulsion with conservative filler volume, but this was unable to resolve residue or aspiration measures.

Pediatric Eosinophilic Esophagitis is Associated With Low Baseline Impedance.

BACKGROUND AND AIMS: Eosinophilic esophagitis (EoE) is a chronic inflammatory disorder that requires repeat endoscopic evaluation(s) to assess response to treatment. This results in high health care costs and a procedural burden in affected children. Noninvasive alternate modalities to reassess disease activity have not been established. Low baseline impedance measured by multichannel pH impedance (pH-MII) is seen in adults with EoE, in keeping with poor mucosal integrity. We aimed to investigate the relationship between esophageal eosinophilia (or severity of eosinophilic infiltration) and baseline impedance in children with EoE. METHODS: We retrospectively identified 15 children diagnosed with EoE at our institution who had undergone pH-MII within 30 days of 3-level esophageal biopsy. This group were not concurrently prescribed proton pump inhibitors and had negligible reflux parameters on pH-MII. Average impedance baseline was calculated upper, mid, and lower esophageal segments via baseline impedance automated analysis (RIAA) and mean nocturnal baseline impedance (MNBI) methods. Eosinophil count data for upper, mid, and lower esophageal segments in the EoE group was collated. RESULTS: A significantly lower baseline impedance was seen across the esophageal length in children with EoE, compared with 30 controls who had no differences in age or reflux burden on nonparametric testing. A relationship between baseline impedance and eosinophil number at corresponding esophageal segments was not established. CONCLUSIONS: Baseline impedance may be an important, less invasive adjunct in clinical practice to monitor treatment response in children with EoE. Larger prospective cohort studies should delineate optimally predictive baseline impedance thresholds for active and inactive disease.

High-resolution impedance manometry characterizes the functional role of distal colonic motility in gas transit.

BACKGROUND: The colonic motor patterns associated with gas transit are poorly understood. This study describes the application of high-resolution impedance manometry (HRiM) in the human colon in vivo to characterize distal colonic motility and gas transit; (a) after a meal and (b) after intraluminal gas insufflation into the sigmoid colon. METHODS: HRiM recordings were performed in 19 healthy volunteers, with sensors positioned from the distal descending colon to the proximal rectum. Protocol 1 (n = 10) compared pressure and impedance prior to and after a meal. Protocol 2 (n = 9) compared pressure and impedance before and after gas insufflation into the sigmoid colon (60 mL total volume). KEY RESULTS: Both the meal and gas insufflation resulted in an increase in the prevalence of the 2-8/minute "cyclic motor pattern" (meal: (t(9) = -6.42, P<0.001); gas insufflation (t(8) = -3.13, P = 0.01)), and an increase in the number of antegrade and retrograde propagating impedance events (meal: Z = -2.80, P = 0.005; gas insufflation Z = -2.67, P = 0.008). Propagating impedance events temporally preceded antegrade and retrograde propagating contractions, representing a column of luminal gas being displaced ahead of a propagating contraction. Three participants reported an urge to pass flatus and/or flatus during the studies. CONCLUSIONS AND INFERENCES: Initiation of the 2-8/minute cyclic motor pattern in the distal colon occurs both following a meal and/or as a localized sensorimotor response to gas. The near-absence of a flatal urge and the temporal association between propagating contractions and gas transit supports the hypothesis that the 2-8/minute cyclic motor pattern acts as a physiological "brake" modulating rectal filling.

Swallowing biomechanics before and following multi-level upper airway surgery for obstructive sleep apnea.

STUDY OBJECTIVES: The effect of contemporary multi-level upper airway surgery for obstructive sleep apnea on swallowing is unclear. This study assessed the biomechanical swallowing function in participants with obstructive sleep apnea pre- and post-modified uvulopalatopharyngoplasty and coblation channeling of the tongue. METHODS: In this prospective, longitudinal study, adults diagnosed with moderate-severe obstructive sleep apnea who underwent modified uvulopalatopharyngoplasty and coblation channeling of the tongue surgery had swallowing biomechanics assessed using high-resolution pharyngeal manometry and analyzed with swallowgateway.com. Symptomatic swallowing difficulty was evaluated using the Sydney Swallow Questionnaire (≥ 234). General linear mixed-model analysis was conducted to evaluate the difference pre- and post-modified uvulopalatopharyngoplasty and coblation channeling of the tongue. Data are presented as mean [95% confidence intervals]. RESULTS: High-resolution pharyngeal manometry assessments were conducted in 10 participants (7 men; median age 50 [interquartile range 36-65]) preoperatively and repeated postoperatively at 9 months [interquartile range 6-13]. Self-reported dysphagia was unchanged following surgery (Sydney Swallow Questionnaire =149 [53, 447] to 168 [54, 247]; P = .093). High-resolution pharyngeal manometry outcomes indicated reduced mesopharyngeal pressures (148 [135, 161] to 124 [112, 137] mm Hg s cm; P = .011), reduced hypopharyngeal pressures (113 [101, 125] to 93 [84, 102] mm Hg s cm; P = 0.011), and reduced upper esophageal sphincter relaxation pressure (5 [4, 6] to 2 [1,3] mm Hg; P = 0.001) but no change to velopharyngeal pressures (135 [123, 147] to 137 [117, 157] mm Hg s cm; P = .850) postsurgery. CONCLUSIONS: Modified uvulopalatopharyngoplasty may have less implications on the swallow mechanism than previously suspected. In contrast, the reduction in mesopharyngeal contractile pressures associated with coblation channeling of the tongue, although within normal limits, may affect bolus propulsion. Biomechanical alterations were insufficient to worsen self-reported swallowing function. CITATION: Schar MS, Omari TI, Woods CW, et al. Swallowing biomechanics before and following multi-level upper airway surgery for obstructive sleep apnea. J Clin Sleep Med. 2022;18(4):1167-1176.

Radiation burden in patients with esophageal atresia: a systematic review.

Esophageal atresia (EA) is the most common congenital esophageal disorder. Radiological imaging facilitates diagnosis, surgical interventions, and follow-up. Despite this, standardized monitoring guidelines are lacking. We aimed to: (1) review the literature regarding radiation burden in children with EA; (2) establish the presence of guidelines for diagnosis and follow-up in children with EA. The systematic review was performed according to PRISMA protocol. Two investigators conducted independent searches (PubMed, Ovid, Cochrane Review) and data extraction. Analysis focused on pre- and post-operative imaging type and frequency to determine the radiation burden. Seven studies met the inclusion criteria (337 patients). All authors agreed upon the need to minimize radiation burden, recommending symptoms-guided management, use of dosimeters, and non-radiating imaging. One study identified a median 130-fold increase in cumulative lifetime cancer risk in children with EA compared with other babies in the special care unit. The most common investigations were X-ray and CT (pre-operatively), and X-ray and contrast swallow (post-operatively). Standardized guidelines focused upon the frequency and type of radiological imaging for children with EA are lacking. Children with EA are subjected to more radiation exposure than the general population. Implementation of non-radiating imaging (ultrasonography, manometry) is recommended.

Esophageal morbidity in patients following repair of esophageal atresia: A systematic review.

BACKGROUND: Esophageal atresia (EA) is a life-threatening congenital condition, affecting one in 2600 newborns. Morbidity remains high, with many patients experiencing complications, including anastomotic leak/stricture, and gastro-esophageal reflux disease (GERD). Increased understanding of esophageal motility patterns may help explain the etiology of these complications. AIMS: We aimed to review knowledge regarding esophageal motility and related complications in children with EA, evaluate patients' symptomatology and relate this to esophageal motility. METHODS: We performed a systematic review (PROSPERO: CRD42018092277), according to the PRISMA protocol. Two investigators independently conducted search strategies (OvidMEDLINE, PubMed, Cochrane Review, BMJ BestPractice), identifying complications in patients following EA repair. Rates of esophageal dysmotility, GERD, dysphagia, anastomotic leak, anastomotic stricture, recurrent fistula formation, and esophagitis were sought. RESULTS: A total of 65 publications met selection criteria (n = 4882). Rates of morbidity were high: esophageal dysmotility (78%), GERD (43%), dysphagia (44%), anastomotic leak (19%), anastomotic stricture (26%), recurrent fistula formation (7%), and esophagitis (47%). No correlation appeared to exist with severity of symptoms. CONCLUSIONS: This systematic review identified high rates of complications in children with EA, with esophageal dysmotility present in the majority of patients. Increasing survival, with resultant longer timeframes to develop morbidities, makes standardized follow-up regimens crucial. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level 3.

Biomechanical correlates of sequential drinking behavior in aging.

BACKGROUND: The timed water swallow test (TWST) is a test of sequential swallowing where a measured volume is ingested as quickly as comfortably possible. We undertook a study of the biomechanics underpinning the TWST in healthy young and older participants. METHODS: Thirty healthy volunteers underwent high-resolution impedance manometry (MMS; Unisensor, 2.7 mm diameter, 32 pressure sensors, 16 impedance segments). Participants were asked to drink 150 mL, 0.9% normal saline solution rapidly. Swallowing biomechanics and bolus flow characteristics were assessed using pressure-flow analysis and compared using t test and Fisher's exact test with significance as P < .05. KEY RESULTS: Older participants (n = 18; 76 ± 11 years) took longer to complete the TWST (21.2 ± 2.5 vs 9.2 ± 1.0 seconds; P < .001) and displayed reduced volume per swallow (16.6 ± 1.3 vs 27.8 ± 2.9 mL; P < .001) compared to younger participants (n = 12; 29 ± 5 years). Two distinctive pharyngeal swallowing patterns were observed: (a) a single rapid sequence of swallows with or without a clearing swallow (Pattern I) or (b) multiple, shorter sequences interrupted and/or interspersed with single swallows or breaks (Pattern II). Some older participants showed biomechanical evidence of upper esophageal sphincter restriction (n = 7) or impaired deglutitive inhibition (n = 7), associated with the more prolonged Pattern II (TWST duration 30.1 ± 1.5 vs Pattern I 11.9 ± 1.5 seconds; P < .001). CONCLUSIONS AND INFERENCES: Healthy older participants had an increased duration of TWST, suggesting a need to adapt normative values for this population. Rapid sequential swallowing was associated with evidence of UES restriction and impaired deglutitive inhibition in some older participants.

High-resolution esophageal manometry in pediatrics: Effect of esophageal length on diagnostic measures.

BACKGROUND: High-resolution esophageal manometry (HREM), derived esophageal pressure topography metrics (EPT), integrated relaxation pressure (IRP), and distal latency (DL) are influenced by age and size. Combined pressure and intraluminal impedance also allow derivation of metrics that define distension pressure and bolus flow timing. We prospectively investigated the effects of esophageal length on these metrics to determine whether adjustment strategies are required for children. METHODS: Fifty-five children (12.3 ± 4.5 years) referred for HREM, and 30 healthy adult volunteers (46.9 ± 3.8 years) were included. Studies were performed using the MMS system and a standardized protocol including 10 × 5 mL thin liquid bolus swallows (SBM kit, Trisco Foods) and analyzed via Swallow Gateway (www.swallowgateway.com). Esophageal distension pressures and swallow latencies were determined in addition to EGJ resting pressure and standard EPT metrics. Effects of esophageal length were examined using partial correlation, correcting for age. Adult-derived upper limits were adjusted for length using the slopes of the identified linear equations. KEY RESULTS: Mean esophageal length in children was 16.8 ± 2.8 cm and correlated significantly with age (r = 0.787, P = .000). Shorter length correlated with higher EGJ resting pressure and 4-s integrated relaxation pressures (IRP), distension pressures, and shorter contraction latencies. Ten patients had an IRP above the adult upper limit. Adjustment for esophageal length reduced the number of patients with elevated IRP to three. CONCLUSIONS & INFERENCES: We prospectively confirmed that certain EPT metrics, as well as potential useful adjunct pressure-impedance measures such as distension pressure, are substantially influenced by esophageal length and require adjusted diagnostic thresholds specifically for children.

Quality of life assessment in esophageal atresia patients: a systematic review focusing on long-gap esophageal atresia.

BACKGROUND: Children born with esophageal atresia (EA) have inherent abnormalities in esophageal motility which may impact upon patient and family Quality of Life (QoL). Currently, paucity of data exists for long-term outcomes of long-gap EA. We aimed to: (1) summarize QoL tools reported in the literature, focusing upon studies involving long-gap EA patients, and (2) compare QoL for long-gap versus non-long-gap EA patients. METHOD: We performed a systematic review of Cochrane Register of Controlled Trials, PubMed, EMBASE, and Ovid databases (January 1980-May 2018) in accordance with the PRISMA protocol. RESULT: Six studies were identified (536 patients total), and 419/536 (78%) patients completed QoL assessment. Response rates ranged from 29% to 100%. Median study size was 86 (range 8-159). Esophageal atresia type was described in 477 patients, and 74/477 (16%) were long-gap. Common assessment tools were Gastrointestinal Quality of Life Index and 36-Item Short-Form Health Survey. Compared with healthy individuals, long-gap EA patients suffered more gastrointestinal symptoms. There were no significant differences in QoL outcomes between long-gap and non-long-gap EA patients. CONCLUSION: Current literature suggests no significant difference in QoL outcomes between long-gap and non-long-gap EA patients. However, due to questionnaire variability and range of response rates, the data should be interpreted with care. LEVEL OF EVIDENCE: Level II.

Oesophageal atresia.

Oesophageal atresia (EA) is a congenital abnormality of the oesophagus that is caused by incomplete embryonic compartmentalization of the foregut. EA commonly occurs with a tracheo-oesophageal fistula (TEF). Associated birth defects or anomalies, such as VACTERL association, trisomy 18 or 21 and CHARGE syndrome, occur in the majority of patients born with EA. Although several studies have revealed signalling pathways and genes potentially involved in the development of EA, our understanding of the pathophysiology of EA lags behind the improvements in surgical and clinical care of patients born with this anomaly. EA is treated surgically to restore the oesophageal interruption and, if present, ligate and divide the TEF. Survival is now ~90% in those born with EA with severe associated anomalies and even higher in those born with EA alone. Despite these achievements, long-term gastrointestinal and respiratory complications and comorbidities in patients born with EA are common and lead to decreased quality of life. Oesophageal motility disorders are probably ubiquitous in patients after undergoing EA repair and often underlie these complications and comorbidities. The implementation of several new diagnostic and screening tools in clinical care, including high-resolution impedance manometry, pH-multichannel intraluminal impedance testing and disease-specific quality of life questionnaires now provide better insight into these problems and may contribute to better long-term outcomes in the future.

Clinical Management of Pediatric Achalasia: A Survey of Current Practice.

OBJECTIVES: Pediatric achalasia is a rare neurodegenerative disorder of the esophagus that requires treatment. Different diagnostic and treatment modalities are available, but there are no data that show how children can best be diagnosed and treated. We aimed to identify current practices regarding the diagnostic and therapeutic approach toward children with achalasia. METHODS: Information on the current practice regarding the management of pediatric achalasia was collected by an online-based survey sent to members of the European and North American Societies for Pediatric Gastroenterology Hepatology and Nutrition involved in pediatric achalasia care. RESULTS: The survey was completed by 38 centers from 24 countries. Within these centers, 108 children were diagnosed with achalasia in the last year (median 2, range 0-15). Achalasia was primarily managed by a pediatric gastroenterologist (76%) and involved a multidisciplinary team in 84% of centers, also including a surgeon (87%), radiologist (61%), dietician (37%), speech pathologist (8%), and psychologist (5%). Medical history taking and physical examination were considered most important to establish the diagnosis (50%), followed by (a combination of) manometry (45%) or contrast swallow (21%). Treatment of first choice was Heller myotomy (58%), followed by pneumatic dilation (46%) and peroral endoscopic myotomy (29%). CONCLUSION: This study shows a great heterogeneity in the management of pediatric achalasia amongst different centers worldwide. These findings stress the need for well-designed intervention trials in children with achalasia. Given the rarity of this disease, we recommend that achalasia care should be managed in centers with access to appropriate diagnostic and treatment modalities.

High-resolution impedance manometry parameters in the evaluation of esophageal function of non-obstructive dysphagia patients.

BACKGROUND: High-resolution impedance manometry (HRIM) allows evaluation of esophageal bolus retention, flow, and pressurization. We explored novel HRIM measures and assessed their temporal relationship to dysphagia symptoms for boluses of different volume and consistency in non-obstructive dysphagia (NOD) patients. METHODS: Thirty-three NOD patients (n = 19 minor or no disorder of peristalsis ("Normal") and n = 14 esophagogastric junction outflow obstruction ("EGJOO")) were evaluated with HRIM. Patients were administered 5 and 10 mL liquid, semisolid, and 2 and 4 cm solid boluses and indicated bolus perception during individual swallows using a 5-point Likert scale. HRIM was analyzed to assess Chicago Classification and pressure flow metrics, esophageal impedance integral (EII) ratio, and bolus flow time (BFT). KEY RESULTS: Overall, bolus perception increased with increasing bolus consistency (P < 0.001), but did not differ significantly between EGJOO and Normal patients. EGJOO patients had higher IRP4, higher levels of bolus residual (ie, EII ratio and IR), and restricted esophageal emptying. The results for linking semisolid bolus perception to semisolid-derived measures revealed more biomechanically plausible and consistent patterns when compared to those derived for liquid boluses. In Normal patients, perception of boluses of heavier viscosity was related to higher bolus flow resistance during transport, whilst in EGJOO, perception was related to restriction of esophageal emptying. CONCLUSION & INFERENCES: These novel pressure-impedance measures may aid in the evaluation of NOD patients by revealing abnormal motor patterns, which may explain symptom generation. Future studies are needed to evaluate which of these measures are worthy of calculation and to establish protocol settings that allow for their meaningful interpretation.

Clinical management of pediatric achalasia.

INTRODUCTION: Achalasia is a rare esophageal motility disorder. Much of the literature is based on the adult population. In adults, guidance of therapeutic approach by manometric findings has led to improvement in patient outcome. Promising results have been achieved with novel therapies such as PerOral Endoscopic Myotomy (POEM). Areas covered: In this review, we provide an overview of the novel diagnostic and therapeutic tools for achalasia management and in what way they will relate to the future management of pediatric achalasia. We performed a PubMed and EMBASE search of English literature on achalasia using the keywords 'children', 'achalasia', 'pneumatic dilation', 'myotomy' and 'POEM'. Cohort studies < 10 cases and studies describing patients ≥ 20 years were excluded. Data regarding patient characteristics, treatment outcome and adverse events were extracted and presented descriptively, or pooled when possible. Expert commentary: Available data report that pneumatic dilation and laparoscopic Heller's myotomy are effective in children, with certain studies suggesting lower success rates in pneumatic dilation. POEM is increasingly used in the pediatric setting with promising short-term results. Gastro-esophageal reflux disease (GERD) may occur post-achalasia intervention due to disruption of the LES and therefore requires diligent follow-up, especially in children treated with POEM.

Modulation of Upper Esophageal Sphincter (UES) Relaxation and Opening During Volume Swallowing.

UES opening occurs following cricopharyngeus deactivation and submental muscle contraction causing hyolaryngeal elevation and UES distraction. During impedance manometry, the inverse of impedance (admittance) can be used to measure bolus presence and infer UES opening. We hypothesized that the temporal relationship between UES relaxation, opening and hyolaryngeal elevation would change with increasing bolus volume. Simultaneous intramuscular cricopharyngeal (CP) electromyography (EMG), surface submental EMG (SM-EMG), and high-resolution impedance manometry were recorded in eight (aged 27 ± 7 years, 5 M) healthy volunteers, while swallowing 0.9 % saline boluses of 2, 5, 10, and 20 ml. Data were exported and analyzed via Matlab. Statistical analysis comprised repeated measures one-way ANOVA and Pearson correlation. A P value of <0.05 was considered significant. Duration of CP deactivation increased at 20 ml volume (P < 0.001). UES relaxation and opening increased with increasing bolus volume (P < 0.001); however, overall duration of SM activation did not change. As UES opening occurs progressively earlier with increasing volumes, peak SM-EMG activity occurs relatively later (P < 0.001) and shifts from occurring before to following peak UES distention. During healthy swallowing, there is sensory modulation of cricopharyngeal and submental muscle activity. Intrabolus pressures, transmitted from the tongue base and pharynx, play a progressively more important role in sphincter opening with increasing volume. The findings may explain why some healthy elderly and patients with oropharyngeal dysphagia have difficulty swallowing larger while tolerating smaller bolus volumes.

Upper gastrointestinal motility: prenatal development and problems in infancy.

Deglutition, or swallowing, refers to the process of propulsion of a food bolus from the mouth into the stomach and involves the highly coordinated interplay of swallowing and breathing. At 34 weeks gestational age most neonates are capable of successful oral feeding if born at this time; however, the maturation of respiration is still in progress at this stage. Infants can experience congenital and developmental pharyngeal and/or gastrointestinal motility disorders, which might manifest clinically as gastro-oesophageal reflux (GER) symptoms, feeding difficulties and/or refusal, choking episodes and airway changes secondary to micro or overt aspiration. These problems might lead to impaired nutritional intake and failure to thrive. These gastrointestinal motility disorders are mostly classified according to the phase of swallowing in which they occur, that is, the oral preparatory, oral, pharyngeal and oesophageal phases. GER is a common phenomenon in infancy and is referred to as GERD when it causes troublesome complications. GER is predominantly caused by transient relaxation of the lower oesophageal sphincter. In oesophageal atresia, oesophageal motility disorders develop in almost all patients after surgery; however, a congenital origin of disordered motility has also been proposed. This Review highlights the prenatal development of upper gastrointestinal motility and describes the most common motility disorders that occur in early infancy.

Outcomes of endoscopy and novel pH-impedance parameters in children: is there a correlation?

OBJECTIVES: Discordance exists between outcomes of endoscopy, multichannel intraluminal impedance monitoring (pH-MII), MII baselines, and gastroesophageal reflux symptoms. The aim of the present study was to determine the association between endoscopy, pH-MII and MII baselines, in children with gastroesophageal reflux symptoms. METHODS: Endoscopies were graded for reflux esophagitis (RE). Biopsies of the distal esophagus were assessed for signs suggestive of esophagitis. Reflux index (RI), symptom association probability (SAP), number of reflux episodes, and mean baseline values were calculated. pH-MII was considered positive in children when RI was ≥ 3% and/or SAP was ≥ 95% and for infants when RI was ≥ 10% and/or SAP was ≥ 95%. Baselines were manually calculated and compared with an automated analysis. For MII baselines, patients were divided in 3 groups: normal endoscopy and negative overall pH-MII; normal endoscopy and an overall positive pH-MII; and RE. RESULTS: A total of 26 children and 14 infants were included, median age: 26.5 months (2 months-16.2 years). Thirteen (32.5%) had RE. A significant negative association was found for RI and MII baselines (P = 0.009) and between SAP and RE (P = 0.039, odds ratio 1.018). MII baseline values were predictive for neither conventional pH-MII parameters nor RE. Manual analysis and automated calculation of MII baselines showed a perfect correlation. Distal MII baselines were significantly lower in children with a positive overall pH-MII outcome compared with the proximal esophagus (P = 0.049). No significant changes were found in baselines among the different groups 1 to 3. CONCLUSIONS: Acid-related parameters are significantly related to MII baselines. A perfect correlation between manual- and automated analysis of MII baselines was found. Large prospective studies are needed to confirm the exact role of endoscopy and MII baselines.

Gastroesophageal reflux, esophageal function, gastric emptying, and the relationship to dysphagia before and after antireflux surgery in children.

OBJECTIVES: To assess gastroesophageal reflux (GER), esophageal motility, and gastric emptying in children before and after laparoscopic fundoplication and to identify functional measures associated with postoperative dysphagia. STUDY DESIGN: Combined impedance-manometry, 24-hour pH-impedance, and gastric-emptying breath tests were performed before and after laparoscopic anterior partial fundoplication. Impedance-manometry studies were analyzed with the use of conventional analysis methods and a novel automated impedance manometry (AIM) analysis. RESULTS: Children with therapy resistent GER disease (n = 25) were assessed before fundoplication, of whom 10 (median age 6.4 years; range, 1.1-17.1 years; 7 male; 4 with neurologic impairment) underwent fundoplication. GER episodes reduced from 97 (69-172) to 66 (18-87)/24 hours (P = .012). Peristaltic contractions were unaltered. Complete lower esophageal sphincter relaxations decreased after fundoplication (92% [76%-100%] vs 65% [29%-91%], P = .038). Four (40%) patients developed postoperative dysphagia, which was transient in 2. In those patients, preoperative gastric emptying was delayed compared with patients without postoperative dysphagia, 96 minutes (71-104 minutes) versus 48 minutes (26-68 minutes), P = .032, and AIM analysis derived dysphagia risk index was greater (56 [15-105] vs 2 [2-6] P = .016). Two patients underwent a repeat fundoplication. DISCUSSION: Fundoplication in children reduced GER without altering esophageal motility. Four patients who developed dysphagia demonstrated slower gastric emptying and greater dysplasia risk index preoperatively. AIM analysis may allow detection of subtle esophageal abnormalities potentially leading to postoperative dysphagia.

Relation between pancreatic lipase activity and gastric emptying rate in children with cystic fibrosis.

OBJECTIVE: To determine whether abnormal gastric emptying is responsible for the inability of pancreatic enzyme replacement therapy (PERT) to normalize fat digestion in patients with cystic fibrosis (CF) who are pancreatic-insufficient. Study design Gastric emptying of a solid meal and pancreatic lipase function were assessed in 10 children with CF and 12 healthy control subjects with noninvasive breath tests using (13)C-octanoic acid and (13)C-mixed triglyceride, respectively. Lipase function was assessed in the subjects with CF with and without PERT. RESULTS: Without PERT, the lipase activity for the patients was less than that for the control subjects (P<.001); however, with PERT, 40% of the patients had a normalized lipase function. There were no differences between the mean gastric emptying rates of the patients with CF and the control subjects (P>.05), but there was a negative correlation between gastric half emptying time and percentage improvement in (13)C-mixed triglyceride results of the patients with CF with pancreatic enzymes compared with placebo (P<.05), with patients with slow gastric emptying having less improvement with PERT. CONCLUSIONS: The success of PERT in improving pancreatic lipase activity is reduced in patients with slow gastric emptying, which could explain the variations in improvement of fat digestion with enzyme supplementation.