Introduction

Upper esophageal sphincter (UES) abnormalities have been reported in 32.5% of esophageal high-resolution manometric (HRM) studies. UES relaxation is the most prevalent response during transient lower esophageal sphincter relaxation (TLSESR) and is associated with air in the reflux. Spontaneous UES relaxation (SUESR) has been reported in achalasia and cannot be explained by TLSER.

Aims & Methods

The main objeticte is to describe the frequency of SUESR in patients with achalasia, compared with patients with gastroesophageal reflux (GERD) and healthy controls. A retrospective database study including consecutive esophageal HRM studies in a seated position from 2017-2020. A SUERS was defined as an interruption of the UES (isobaric contour of 12 mmHg) in the absence of hypopharyngeal contraction within 15 s of a wet swallow. To evaluate the dynamics of SUERS, the latency from the beginning of a wet swallow to a SUESR and its duration were measured. Additionally, a DCI tool was forced from the lower border of the UES through the upper edge of the LES and 5 s before and after a SUESR (contractile integral, CI). The maximum pressure of the UES 5 seconds before and after a SUESR was measured. GERD studies were included if a 24-hour pH monitoring reported an acid exposure time >6%, and healthy controls were extracted from the laboratory’s database. All HRMs were classified by Chicago 4.0, and a sub-analysis was performed for achalasia subtypes. Frequencies and percentages were used for categorical variables and median and interquartile ranges for continuous variables. Groups were compared with ANOVA, and a two-sided p value was calculated with a significance <0.05.

Results

A total of 179 esophageal HRM studies were included; 120 (67%) were female, the median age was 41 years (30.0-55.3), and the median BMI was 24.6 kg/m² (22.1-27.9). Groups were divided into achalasia (116), GERD (33), and controls (30), from which a total of 57 (30.1%) studies showed SUERS with a prevalence of 49 (42.2%), 4 (12.1%), and 4 (13.3%), respectively. No difference was found in the total number of SUESR between groups (p=0.05). Nevertheless, a statistical difference was observed in the CI post-SUESR (p=0.01) and the UES maximum pressure pre-SUESR (p=0.02), both of which were higher in the achalasia group. Among achalasia subtypes with SUESR, type II (18) showed a higher basal UES pressure (p=0.003), CI pre-SUESR (p=0.004), CI post-SUERS (p=0.01), and UES maximum pressure post-SUERS (p=0.04) (see Table 1).

Conclusion

A prevalence of SUESR of 42.2% was found in patients with achalasia, with higher HRM metrics in achalasia type II. This finding suggests a valve mechanism from the UES and liberation of esophageal pressurization through the SUERS. Further research is required to correlate this finding as a prognostic tool.

References

1. Cerda E, Cortes YC, Valdovinos MA. Spontaneous Relaxation of the Upper Esophageal Sphincter (UES) After Esophageal Pressurization. A Common Finding in Patients With Achalasia. Ga​stroenterology 2010;138(5):S-341.
2. Babaei A, Bhargava V, Mittal RK. Upper esophageal sphincter during transient lower esophageal sphincter relaxation: effects of reflux content and posture. Am J Physiol Gastrointest Liver Physiol 2010;298(5):G601-7.
3. Zavala-Solares MR, Coss-Adame E, Valdovinos-García LR, Torres-Villalobos G, Olavide-Aguilar R, Valdovinos MA. Upper Esophageal Sphincter (UES) Motor Function in Patients With Achalasia Subtypes. The Effect of Esophageal Pressurization and EGJ Ouflow Obstruction. Gastroenterology 146(5):S-883.

Introduction

Upper esophageal sphincter (UES) is a muscular structure located at the transition from the pharynx to the esophagus, with the cricopharyngeal muscle as the most important component. One of its main functions is to prevent esophago-pharyngeal reflux; however, it also plays a key role during swallowing, belching, and vomiting. Data on UES involvement on pathogenesis and clinical manifestations of esophagogastric junction outflow obstruction (EGJOO) are lacking.

Aims & Methods

Aim of this study the UES manometric parameters analysis and study of their impact on pathogenesis and clinical presentation of patients affected by Esophagogastric Junction Outflow Obstruction (EGJOO) according to the Chicago Classification v4.0 criteria. Manometric tracings, epidemiological and clinical data of patients with a diagnosis of EGJOO in our departments between 07/2021- 03/2025 were analyzed. Clinical presentation was evaluated by performing Dysphagia Symptom Questionnaire (DSQ) and Eckardt score. Manometric parameters of UES function (mean basal pressure, mean residual pressure, resting time and recovery time) as well as the other manometric parameters were analyzed and correlated through multifactor statistical analysis (MFA) with clinical and epidemiological data.

Results

Data from 94 patients (73F/21M, mean age 66 years) were analyzed. 40.4% presented dysphagia, 46,8% GERD related symptoms and 12.8% Non-Cardiac Chest Pain (NCCP) as main clinical presentation at the time of HRM examination. Mean basal pressure of the lower esophageal sphincter (LES) was 50.85 mmHg (R19.5-78.8), mean integrated relaxation pressure (IRP) was 19.15 mmHg (R 15.2-40.4), mean Distal Contractile Integral (DCI) 5.031 (mmHg -cm-s) (R 3.487-7.345), mean Distal Latency 6.4 (R 4-10.2), mean UES basal pressure 53.3 mmHg (R27.8-220), mean UES residual pressure 8.75 mmHg (R 0.2 -70.9), mean UES resting time 181 ms (R48-476), mean recovery time 465 (R46-880). 34 patients (36.2%) had abnormal UES manometric parameters. Multifactorial statistical analysis revealed a statistically significant correlation of the pathological UES residual pressure with abnormal LES basal pressure (p = 0.040), dysphagia as main clinical symptom (p=0.030), LES length (p=0.013) and dysphagia symptom severity evaluated by DSQ: p=0.017 and Eckardt: p=0.041.

Conclusion

UES motility has not been extensively studied on HRM in patients affected by EGJOO. Our study revealed that a significant number of EGJOO patients presented abnormal UES function. UES motility abnormalities in these patients are correlated with abnormal LES function, symptoms severity and particularly characterize patients with predominant dysphagia.

References

1. Singh S, Hamdy S. The upper oesophageal sphincter. Neurogastroenterol Motil. 2005 Jun;17 Suppl 1:3-12. doi: 10.1111/j.1365-2982.2005.00662.x. Erratum in: Neurogastroenterol Motil. 2005 Oct;17(5):773.
2. Omari, T.I., Maclean, J.C.F., Cock, C., McCulloch, T.M., Nativ-Zeltzer, N., O'Rourke, A.K., Szczesniak, M.M., Wu, P.I., Allen, J., Aoyagi, Y., Bayona, H.H.G., Carrión, S., Ciucci, M.R., Davidson, K., Dhar, S.I., Hamdy, S., Howell, R., Jones, C., Knigge, M.A., Moonen, A., Postma, G.N., Puntil-Sheltman, J., Rameau, A., Regan, J., Schar, M. and Rommel, N. (2025), Defining Pharyngeal and Upper Esophageal Sphincter Disorders on High-Resolution Manometry-Impedance: The Leuven Consensus. Neurogastroenterology & Motility e70042

Introduction

Recent studies have evaluated the upper esophageal sphincter (UES) with high-resolution manometry (HRM) in Achalasia, Esophageal-Gastric Junction Outflow Obstruction (EGJOO), and Gastroesophageal Reflux Disease (GERD). There are, however, no studies of UES metrics in Eosinophilic Esophagitis (EoE) patients.

Aims & Methods

The aim of our study was to evaluate the function of UES across EoE, GERD, and Functional Dysphagia. We also studied the relationship of UES metrics with esophageal symptoms, esophageal body contraction, and EGJ-metrics. This pilot study enrolled 30 EoE,18 GERD,and 29 Functional Dysphagia patients diagnosed according to Chicago Classification 3.0. The study data were exported to the online analysis platform Swallow Gateway. UES was assessed in terms of UES Resting Pressure (UES-RP), UES Basal Pressure (UES-BP), UES Integrated Relaxation Pressure (UES-RP), UES Relaxation Time (UES-RT), Basal UES Contractile Integral (Basal UES-CI), Post-deglutitive UES Contractile Integral (Post-deglutitive UES-CI), and Proximal Contractile Integral (PCI) through ten 5 mL wet swallows.

Results

ANOVA analysis showed significantly higher values of Post-deglutitive UES-CI in EoE patients compared with Functional Dysphagia patients (p=0.001). Basal UES-CI and UES-RP showed significantly higher values in EoE patients (p=0.002 and p=0.038) and GERD patients (p<0.001 and p=0.001) compared with Functional Dysphagia patients. A correlation between LES-CI and Post-deglutitive UES-CI, Basal UES-CI, UES-RP (p=<0.001,p=0.027,and p=0.017,respectively), and between LES-BP and Post-deglutitive UES-CI (p=0.019), independently from diagnosis, was shown. No correlations have been demonstrated between UES and EGJ metrics and esophageal symptoms.

Conclusion

These results emphasize the importance of UES analysis, which may be an additional tool to understand novel pathophysiological mechanisms underlying rare diseases such as EoE.

Introduction

Esophageal Achalasia (EA) is a rare esophageal motility disorder, characterized by impaired peristalsis and incomplete relaxation of the Lower Esophageal Sphincter (LES) in response to swallowing​​​​​. Key symptoms of the disease include dysphagia, food regurgitation, and retrosternal pain, significantly impacting the quality of life (QoL) and often leading patients to restrict their food intake. Consequently, EA patients often adopt self-managed dietary practices that may result in nutritional imbalances and deficiencies, given the lack of specific dietary guidelines.

Aims & Methods

This retrospective study aimed to describe and evaluate the dietary patterns, eating habits, and nutritional status of achalasic patients. Additionally, the secondary aim was to explore potential associations between food rheology and symptomatology. Patients with a manometric diagnosis of EA were recruited from our outpatient gastrointestinal pathophysiology center. Disease severity was assessed with Eckardt Symptoms Score (ESS), symptoms were evaluated with Modular Questionnaire Roma IV and QoL with the MD Anderson Dysphagia Inventory (MDADI). The MDADI outcomes were stratified into severe, moderate, mild and not perceived disability. Patients also filled out a food diary for 15 days, recording ingested food for each meal and symptoms onset. Estimated caloric intake and macronutrient composition were compared to those recommended by the Italian Society of Human Nutrition (SINU).

Results

Forty-three achalasic patients were included (M 24, F 19, mean age 56.7 +- 15.8 yrs). The median BMI was 23.7 (18.8-32.2), 34 patients (79%) had an active disease (ESS ≥ 3). Most patients reported mild to moderate disability (respectively 42% and 35%), while 21% reported severe disability and 2% perceived no disability. Disease activity, measured by ESS, strongly correlated with perceived disability (r= 0.59; p <0.0001), while no significant correlation was found between ESS and BMI. The diary analysis showed a mean caloric intake of 1572±373 kcal versus the estimated needs of 2293,71±374 kcal based on anthropometric parameters and physical activity, resulting in a mean caloric deficit of 720 ± 416 kcal (p<0.0001). Macronutrient analysis also reported an unbalanced distribution: carbohydrates 43.3%±7.3 vs recommended 55-59%, protein 18.2%±4.5 vs 15-18%, total fat 37.6%±7.7 vs 26–27% and fibre 14g±5.2 vs 25-30g. Solid food caused significantly more symptoms than semiliquid and liquid ones: dysphagia (35.5% vs 3.5% vs 5.7%, p<0.0001), chest pain (10% vs 12.1% vs 0.2%, p<0.0001), regurgitation (8.9% vs 1.4% vs 1.9%, p=0.001), cough (5% vs 0.2% vs 0.6%, p=0.0002). Bread, pasta, and pizza (50-60%), fresh fruit (44%), lean meat (39.5%), crackers, snacks, and vegetables (30-35%) were the most commonly reported triggers of dysphagia. Additionally, 60% of patients reported worsened symptoms with cold foods, while 53% reported improvement with hot foods.

Conclusion

This study underscores the significant impact of EA on QoL, influenced by disease activity. Achalasic patients tend to modify their diets to minimize symptoms, favoring easily swallowable soft and fatty foods while reducing intake of solid foods rich in carbohydrates and fiber. Thus, comprehensive nutritional assessment is strongly recommended for EA patients to mitigate dietary imbalances and manage possible deficiencies promptly.

Introduction

Outcome after treatment of achalasia is usually assessed by the Eckardt score (ES). The timed barium esophagogram (TBE) is used to objectively assess esophageal clearance after treatment. High-resolution manometry with impedancemetry (HRiM) provides information on esophageal clearance of liquids in addition to motility parameters. The aim of this study was to compare esophageal clearance determined by HRiM and TBE in patients with achalasia treated by POEM.

Aims & Methods

Consecutive patients treated with POEM had pre and post-POEM assessment with ES, TBE, and HRiM. Treatment failure was defined by an ES > 3 at 3 months post-POEM. Incomplete esophageal clearance (IEC) was defined by the presence of an esophageal stasis > 2 cm at 5 minutes in HRiM and/or TBE. All of the data available pre and post-POEM at 3 months was pooled for analysis. The correlation between ES, TBE and HRiM was evaluated by Spearman's method.

Results

Fifty patients with a median age of 65 years (23-84) were included in this study (85% with type 2 achalasia). The clinical response rate at 3 months was 94%. TBEM/HRiM results were available in (29/33) pre-POEM patients and (43/46) post-POEM patients at 3 months. Overall, an IEC was found in 91% on TBE and in 50.8% on HRiM. The correlation between the ES and IEC in HRiM and TBE were respectively 0.66 and 0.31. The correlation between esophageal clearance values between TBE and HRIM was low (r=0.375). At 3 months after POEM, IEC was present in 90.7% and 21.7% for TBE and HRiM, respectively (p<0.001). In univariate and multivariate analysis, only IEC in HRiM was significantly associated with ES > 3 (RR = 17.0 (p < 0.001)). The HRiM AUC was 0.872 vs. 0.707 for the TBE (p<0.001). The diagnostic performance of HRiM to predict an ES > 3 was Se: 85%; Sp: 77%; PPV: 74%; NPV: 87% vs. Se: 95%; Sp: 11%; PPV: 42%; NPV: 83% for the TBE. Data at one year will be available for the presentation.

Conclusion

In achalasia, the evaluation of esophageal clearance by HRiM is better correlated with ES than TBE and may be a relevant criterion for pre and post-therapeutic evaluation. Additional studies are underway to validate the relevance of this parameter more specifically in the evaluation of treatments for esophageal achalasia.

References

1. Lin Z, Nicodème F, Lin CY, Mogni B, Friesen L, Kahrilas PJ, et al. Parameters for quantifying bolus retention with high-resolution impedance manometry. Neurogastroenterol Motil. 2014 Jul;26(7):929–36.
2. Conchillo JM, Selimah M, Bredenoord AJ, Samsom M, Smout AJPM. Assessment of oesophageal emptying in achalasia patients by intraluminal impedance monitoring. Neurogastroenterol Motil. 2006 Nov;18(11):971–7.
3. Cho YK, Lipowska AM, Nicodème F, Teitelbaum EN, Johnston ER, Gawron A, et al. Assessing Bolus Retention in Achalasia using High Resolution Manometry with Impedance: A Comparator Study with Timed Barium Esophagram. 2015;15.
4. de Oliveira JM, Birgisson S, Doinoff C, Einstein D, Herts B, Davros W, et al. Timed barium swallow: a simple technique for evaluating esophageal emptying in patients with achalasia. Am J Roentgenol. 1997 Aug;169(2):473–9.
5. Kostic S, Andersson M, Hellström M, Lönroth H, Lundell L. Timed barium esophagogram in the assessment of patients with achalasia: Reproducibility and observer variation. Dis Esophagus. 2005 Jun 1;18(2):96–103.

Clinical Case Summary

Achalasia is a rare motor disorder characterized by esophageal aperistalsis and incomplete relaxation of the lower esophageal sphincter, commonly complicated by esophageal dilation with subsequent sigmoidization of the esophagus, pneumonia or lung abscesses, due to food regurgitation and aspiration[1]. Although an esophageal hemorrhage may occasionally occur due to esophageal stasis and esophagitis, severe bleeding from achalasia is exceedingly rare[2],[3].
We present the case of a 52-year-old female with a history of stroke and long-lasting achalasia which presented with sudden onset of hematemesis and altered general status. On admission the patient required fluid resuscitation and blood transfusion but without evidence of active bleeding. An upper gastrointestinal endoscopy was performed that revealed a dilated esophagus with a large adherent blood clot and no signs of active bleeding. After fragmenting and extracting the clot using a Dormia basket, multiple irregular ulcers were observed in the middle and lower esophagus, one with a visible vessel without active bleeding, presumed to be the bleeding source. Endoscopic treatment consisted of hemostasis using a metal clip for the esophageal ulcer with high-risk stigmata and balloon dilation with a 35mm polyethylene balloon for the achalasia. Further treatment included high-dose proton pump inhibitors, parenteral nutrition and the patient was scheduled to return for another session of pneumatic dilation.
The particularity of the case lies in the presence of a rare complication of achalasia, which can predispose to stasis ulcers, leading to possibly life-threatening esophageal bleeding.

References

1. Savarino E, Bhatia S, Roman S, Sifrim D, Tack J, Thompson SK, Gyawali CP. Achalasia. Nat Rev Dis Primers. 2022 May 5;8(1):28.
2. Momodu II, Wallen JM. Achalasia. [Updated 2023 Jul 31]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-
3. Cho JH. Massive esophageal bleeding in long-standing achalasia complicated by esophageal carcinoma and aspirin-induced stasis ulcer: Case report. Medicine (Baltimore). 2019 Jul;98(30):e16519.