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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., (© 2024 European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objectives: The aim of the study was to characterize esophageal motility and esophagogastric junction (EGJ) function in infants who underwent repair of an isolated congenital diaphragmatic hernia (iCDH)., Methods: High-resolution manometry with impedance was used to investigate esophageal motility and EGJ function after diaphragmatic repair in 12 infants with iCDH (11 left-sided; 9 patch repair). They had esophageal motility studies during neonatal admission (n = 12), at 6 months (n = 10) and at 12 months of life (n = 7). Swallows were analyzed using conventional esophageal pressure topography and pressure flow analysis and were compared with 11 healthy preterm born infants at near-term age., Results: Esophageal peristaltic motor patterns in patients with iCDH were comparable to controls. EGJ end-expiratory pressure was higher in patients with patch repair compared with controls (P = 0.050) and those without patch (P = 0.009). The difference between inspiratory and expiratory pressures at the EGJ was lower in patients with iCDH with patch (P = 0.045) compared to patients without. Patients with iCDH with patch showed increased Pressure Flow Index, resistance of bolus flow at the EGJ, compared with controls (P = 0.043)., Conclusions: Normal esophageal wave patterns are present in the investigated patients with iCDH. EGJ end-expiratory pressure seems lower in patients with iCDH without patch suggesting a decreased EGJ barrier function hence increased vulnerability to gastroesophageal reflux. Patch repair appears to increase end-expiratory pressure at the EGJ above that of controls suggesting that patch surgery tightens the EGJ, thereby increasing flow resistance. This is in line with the increased Pressure Flow Index. In infants with a patch, the inspiration-expiration pressure difference is lower, reflecting diminished activity of the crural diaphragm.

Objective: The clinical relevance of esophageal baseline impedance (BI) remains to be determined. In the present study, we explored the impact of gastroesophageal reflux disease (GERD) and esophageal dysmotility on BI., Methods: A total of 18 children with esophageal atresia, 26 children with GERD, and 17 controls prospectively underwent esophagogastroduodenoscopy and pH-impedance monitoring. BI was measured in both proximal and distal esophagus. Gastroesophageal reflux (GER) and bolus transit indicators were defined according to published criteria., Results: Patients with esophageal atresia showed significantly lower proximal and distal BI values (952 [716-1811] Ω; 895 [284-1189] Ω; respectively) compared with those with GERD (3015 [2368-3975] Ω; 2231 [1770-3032] Ω, P < 0.001 and <0.001, respectively) and controls (3699 [3194-4358] Ω; 3522 [2927-3994] Ω, P < 0.001 and <0.001, respectively). Using linear regression, proximal BI strongly correlated with total bolus transit time (r(2) = 0.61, P < 0.001) and bolus presence time (BPT; r(2) = 0.63, P < 0.001). Distal BI weakly correlated with acid exposure time (r(2) = 0.16, P < 0.01) and longstanding reflux episodes (r(2) = 0.17, P < 0.01), and strongly correlated with total bolus transit time (r(2) = 0.53, P < 0.001) and BPT (r(2) = 0.58, P < 0.001). By logistic regression, BPT predicted low proximal BI values (odds ratio [OR] 1.052; P < 0.05), whereas both GER indicators (acid exposure time: OR 1.56, P < 0.05; longstanding reflux episodes: OR 2.8, P < 0.05) and BPT (OR 1.66, P < 0.01) predicted low distal BI values., Conclusions: Along the length of esophagus, both bolus transit variables and GER significantly affect BI. This suggests that BI may merely mirror phenomena occurring within the esophageal lumen or wall, limiting its value as a discrete clinical entity to replace variables already used for assessing both GERD and esophageal dysmotility.

After surgical correction of esophageal atresia with or without tracheoesophageal fistula, esophageal body motility dysfunction has been reported in nearly all patients. Using high-resolution esophageal manometry before surgical repair in 2 children with isolated tracheoesophageal fistula, we sought to determine whether dysmotility was present before any surgical insult to test the hypothesis that dysmotility associated with esophageal atresia with or without tracheoesophageal fistula is related to intrinsic primary factors linked to abnormal development of the esophagus. Both had an abnormal esophageal motility: one exhibited hypomotility with distal contraction, whereas the other showed a complete aperistalsis pattern. This suggests that esophageal dysmotility is congenital in nature rather than secondary to surgery.

Background: Esophageal dysmotility, a considerable issue following esophageal atresia (EA) repair, has been reported but has not been precisely described and characterized. Using high-resolution esophageal manometry (HREM), we characterized the esophageal motility patterns in children with repaired EA and compared these patterns of dysmotility with symptomatology., Methods: HREM was performed as an outpatient procedure in patients with repaired EA. The tracings were analyzed using the software provided by the company and were then reviewed visually. Charts were reviewed for medical/surgical histories and symptoms were assessed by a standardized questionnaire., Results: Forty patients (25 boys, 15 girls) with a median age of 8 years (11 months-18 years) underwent an HREM. Thirty-five patients had type C EA and 5 had type A EA. Only 7 patients were asymptomatic at the time of the examination. HREM results were abnormal in all of the patients. Three different esophageal motility patterns were derived from HREM tracing analysis: aperistalsis (15 patients, 38%), pressurization (6 patients, 15%), and distal contractions (19 patients, 47%). Distal contractions pattern was found exclusively in type C EA. Dysphagia was encountered in the 3 groups. Gastroesophageal reflux disease-related symptoms predominated in the aperistalsis group., Conclusions: HREM improves our understanding and allows precise characterization of esophageal dysmotility in patients who have undergone EA repair.

Diffuse esophageal spasm (DES) causes chest pain and/or dysphagia in adults. We reviewed charts of 278 subjects 0 to 18 years of age after esophageal manometry to describe the frequency and characteristics of DES in children. Patient diagnoses included normal motility (61%), nonspecific esophageal motility disorder (20%), DES (13%, n=36), and achalasia (4%). Of patients with DES, the most common chief complaint was food refusal in subjects younger than 5 years (14/24, 58%) and chest pain in subjects older than 5 years (4/12, 33%). Comorbid medical conditions, often multiple, existed in 33 subjects. DES should be considered when young children present with food refusal.

Recently, multichannel intraluminal impedance (MII) was added to the repertoire of tests available to study esophageal (patho)physiology in children. MII has since been used in 2 major areas. First, it has been used as a diagnostic test for gastroesophageal reflux disease (GERD). The main advantage over traditional pH monitoring is its ability to detect both acid and nonacid gastroesophageal reflux (GER) and to discern between liquid and gas GER.Although feasible with multiple pH sensors, the MII technique routinely detects the proximal extent of a GER episode. When a pH sensor is added to the MII-catheter, important information about the acidity of a GER event can be gathered.The second area in which the role of MII has been investigated is that of esophageal function testing. Manometry classically reveals information about esophageal pressure patterns and sphincter function, but does not inform us about bolus flow. MII not only detects the presence of esophageal flow but also adds information on the direction of flow, duration of bolus presence, completeness of bolus clearance, and composition of a bolus.The combination of MII with manometry enables determination of the relationship between esophageal pressures and flow and, therefore, enhances evaluation of esophageal function in terms of assessment of mechanisms of esophageal volume clearance. In addition, this technique will improve our understanding of (patho)physiological mechanisms in pediatric GERD and other esophageal motility disorders.

Gastroesophageal reflux is a physiological phenomenon, occurring with different severity and duration in different individuals. Reflux occurs when this normal event results in the occurrence of symptoms/signs or complications. The pathophysiology of gastroesophageal reflux is complex and diverse, since it is influenced by factors that are genetic, environmental (e.g., diet smoking), anatomic, hormonal, and neurogenic. However, many mechanisms remain incompletely understood. Future research should focus on a better understanding of the physiology of the upper and lower esophageal sphincters, and of gastric motility. The afferent and efferent neural pathways and neuropharmacologic mediators of transient lower esophageal sphincter relaxations and gastric dysmotility require further study. The role of anatomic malformations such as hiatal hernia in children has been underestimated. While therapeutic possibilities are greater in number and largely improved, the outcomes of some treatments are far from satisfactory in many cases. In addition to development of new forms of treatment, research should address better use of currently available medical and surgical treatments.

Background: Feeding disorders are one of the main clinical features in PRS, which combines a posterior U-shaped cleft palate, retrognathia, and glossoptosis. The aim of this study was to evaluate the oral and esophageal motor function of children with PRS without additional neurologic symptoms., Methods: All children hospitalized with Pierre Robin syndrome either isolated (n = 27) or associated with Stickler syndrome (n = 8) were included. Clinical evaluation of their oroesophageal disorders and systematic esophageal manometry were performed., Results: Feeding disorders were always present, but type of disorder varied from one child to another. Esophageal disorders were frequent and seemed to be resistant to classic gastroesophageal reflux treatment. Eighty-six percent of the children required nasogastric tube feeding for a mean duration of 8.6 months. Esophageal manometric abnormalities were noted in 50% of the children: lower esophageal sphincter hypertonia, failure of lower esophageal sphincter relaxation at deglutition, and esophageal dyskinesia. These clinical and manometric disorders showed a trend to spontaneous regression after 12 months., Conclusion: In the current Pierre Robin syndrome series, clinical and manometric anomalies of oroesophageal motility were always present. The authors identified an unusual manometric pattern that has also been described in situations of neurovegetative instability. It could reflect dysregulation of the control of the central pattern generators of swallowing in the brain stem.

Background: Feeding impairment frequently complicates the course of children with neurologic disorders and places them at risk for malnutrition and growth failure. Although feeding abnormalities have been reported in female patients with Rett syndrome, the mechanisms that account for these findings have not been elucidated fully. This study was designed to characterize the clinical features of oropharyngeal and gastroesophageal dysfunction and their impact on the dietary intake and nutritional status of female subjects with Rett syndrome., Methods: The clinical features of oropharyngeal and gastroesophageal dysfunction in 13 female patients with Rett syndrome, (age range, 3.7 to 25.7 years) were characterized by an oral feeding assessment, swallowing function study, and upper gastrointestinal series. Growth, nutritional status, and body composition were determined by stadiometry and anthropometry. Dietary intakes were determined from 3-day food records., Results: Oropharyngeal dysfunction and gastroesophageal dysmotility were present in 100% and 69%, respectively, of the study patients with Rett syndrome. The scope and severity of these abnormalities were apparent only by videofluoroscopy. Abnormalities of oropharyngeal function included poor tongue mobility, reduced oropharyngeal clearance, and laryngeal penetration of liquids and solid food during swallowing. Esophageal dysmotility included absent primary or secondary waves, delayed emptying, atony, the presence of tertiary waves, spasm, and gastroesophageal reflux. Gastric dysmotility included diminished peristalsis or atony. Lower dietary energy intakes were associated with persistence of residue in the valleculae and pyriform sinuses and less body fat., Conclusion: The prevalence of oropharyngeal dysfunction and gastroesophageal dysmotility warrants early diagnostic evaluation and intervention strategies to improve the nutritional status of girls and women with RS.

Esophageal acidity may remain stable for long periods at or near pH 4.0, potentially influencing the estimation of risk of esophagitis in esophageal pH monitoring assessment. Our aim was to determine the frequency and clinical significance of a prolonged stable pH around 4.0 (PSpH4) (pH 3.5-4.5) by retrospective examination of 503 pH records from children and by analysis of clinical information, pH parameters, motility studies, and esophageal biopsies. The frequency, duration, and proportion of total time with PSpH4 were quantified, and the ratio of the time for which the pH was < pH 4.0 during PSpH4 episodes to the total reflux time below pH 4.0 was calculated [stable/reflux (S/R) rate]. One hundred fifty-eight PSpH4 episodes were identified in 80 records. The average duration was 60 min and the S/R rate was 30%. Sixty-one percent of PSpH4 episodes started in the postprandial period (p < 0.05). Proportionally more episodes of PSpH4 occurred with an increased total percentage reflux time (p < 0.01). In recordings with PSpH4, the average number of reflux episodes remained normal, even when the percentage reflux time was > or = 10%. No correlation was shown between PSpH4 and esophageal dysmotility, esophagitis, cardiorespiratory symptoms, or prematurity. PSpH4 did not occur in recordings obtained after fundoplication. We conclude that PSpH4 is common and may result in an overestimation of risk of esophagitis. Qualitative assessment of pH monitoring is necessary along with conventional quantitative measurements.

The aim of the present study was to investigate esophageal motor function by means of krypton-81m esophageal transit scintigraphy and to compare the results with the functional and morphological data obtained by means of triple lumen manometry and endoscopy. In acute and subacute stages of the disease, all clinical, anatomical, and functional parameters were in good agreement, revealing significant impairment. In chronic stages, the severity of the dysphagia was not correlated to the importance of the residual stenosis. Conversely, 81mKr esophageal transit and manometric's findings were in good agreement with the clinical symptoms, during the entire follow-up period ranging between 3 months to 7 years. The 81mKr test is undoubtedly the easiest and probably the most physiological technique currently available for long-term functional evaluation of caustic esophagitis.

Two small infants with gastroesophageal reflux disease and esophagitis are reported. Esophageal manometry revealed in both patients severe abnormalities consisting of aperistalsis and simultaneous low-amplitude motor waves. In one of the patients, defective relaxation of lower esophageal sphincter was also noted. Short-term intensive treatment with H2 antagonists resulted in symptomatic and endoscopic improvement as well as in manometric normalization. It is suggested that severe esophagitis may affect control mechanisms of esophageal motility, resulting in loss of coordination and decreased amplitude of contractions.