Search

Goals: We surveyed esophageal motility laboratories affiliated with adult pulmonary transplant centers to determine esophageal function testing (EFT) practices., Background: Gastroesophageal reflux and esophageal dysmotility are associated with worse lung transplant outcomes, yet no consensus guidelines for EFT exist in this population., Study: A deidentified online survey was sent to gastrointestinal motility laboratory directors of 49 academic and community-affiliated medical centers that perform lung transplants. Practice characteristics, including annual lung transplant volume and institutional EFT practices pre-lung transplantation and post-lung transplantation were queried. Respondents were categorized by transplant volume into small and large programs based on median annual volume., Results: Among 35 respondents (71% response rate), the median annual transplant volume was 37, and there were 18 large programs. Institutional EFT protocols were used pretransplant by 24 programs (68.6%) and post-transplant by 12 programs (34.2%). Among small and large programs, 52.9% and 72.2% always obtained high-resolution manometry before transplant, respectively. Endoscopy before transplant was performed more often in small programs (n=17, 100%) compared with large programs (n=15,83.3%). Pretransplant endoscopy ( P =0.04), barium esophagram ( P <0.01), and high-resolution manometry ( P =0.04) were more common than post-transplant. In contrast, post-transplant reflux monitoring off-therapy was more common than pretransplant ( P =0.01). In general, pulmonologists direct referrals for EFT and gastroenterology consultation (n=28, 80.0%), with symptoms primarily prompting testing., Conclusions: In the absence of established guidelines, substantial variability exists in pretransplant and post-transplant EFT, directed by pulmonologists. Standardized EFT protocols and gastroenterologist-directed management of esophageal dysfunction has potential to improve lung transplant outcomes., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Goals: Awareness of functional esophagogastric junction outflow obstruction (fEGJOO) has increased, but because there is no consensus on its management, we performed a systematic review of the literature to explore treatment strategies and outcomes., Background: EGJOO is a heterogenous disorder defined by high-resolution manometry parameters of elevated integrated relaxation pressure with preserved esophageal peristalsis. The etiology may be mechanical obstruction or idiopathic, the latter being fEGJOO., Study: The PubMed/MEDLINE, Embase, and the Cochrane library electronic databases were searched through June 2018 for all studies of adult patients describing a treatment strategy for fEGJOO or incomplete lower esophageal sphincter relaxation. The search strategy yielded 1792 studies and 8 (0.4%) met inclusion criteria., Results: All but one included studies were retrospective (n=184 patients). There were 5 interventions described, with botulinum toxin (Botox) injection (n=69) and expectant management (n=82) the most frequently reported, with success rates of 58% and 54%, respectively. There was substantial heterogeneity among patients and treatments were not directly compared, though reported symptom resolution was similar among all strategies with a mean follow-up time of 15 months., Conclusions: There are a variety of management strategies available for fEGJOO and some patients may not require any intervention. However, among 4 potential approaches aimed at disrupting lower esophageal sphincter hypertonicity, the largest existing evidence base supports either a therapeutic challenge of Botox injection or watchful waiting. Ultimately, these data indicate the need for further study with controlled trials to identify a definitive approach.

Esophageal high resolution manometry (HRM) is the gold standard for assessment of esophageal motor disorders, but motor responses to the standard 5 mL water swallow protocol may not provide precision in defining minor motor disorders. Provocative maneuvers, particularly multiple rapid swallows (MRS), have been used to assess deglutitive inhibition during the repetitive swallows, and the contractile response following the final swallow of the sequence. The augmentation of esophageal smooth muscle contraction following MRS is termed contraction reserve. This is determined as the ratio between esophageal body contraction vigor (distal contractile integral, DCI) following MRS to the mean DCI after single swallows, which is ≥1 in the presence of contraction reserve. Reliable assessment of contraction reserve requires the performance of 3 MRS maneuvers during HRM. Absence of contraction reserve is associated with a higher likelihood of late postfundoplication dysphagia and may correlate with higher esophageal reflux burden on ambulatory reflux monitoring. Esophageal motor responses to abdominal compression, functional lumen imaging probe (FLIP) balloon distension, and pharmacologic testing (using edrophonium and cisapride) may correlate with contraction reserve. Other provocative tests useful during HRM include rapid drink challenge, solid and viscous swallows, and standardized test meals, which are more useful in evaluation of esophageal outflow obstruction and dysphagia syndromes than in identification of contraction reserve. Provocative maneuvers have been recommended as part of routine HRM protocols, and while useful clinical information can be gleaned from these maneuvers, further research is necessary to determine the precise role of provocative testing in clinical esophagology.

Goals: The aim of our study was to characterize jackhammer esophagus symptoms and their relationship with the distal contractile integral (DCI) and bolus transit., Background: Jackhammer esophagus is defined by the Chicago Classification version 3.0. This diagnosis is relatively new, with the most current definition being established in 2014. The forerunners of this diagnosis, nutcracker (or hypercontractile) esophagus, have been associated with noncardiac chest pain (NCCP)., Study: A retrospective chart review was performed of motility studies from 2011 to 2016. Studies with a diagnosis of jackhammer esophagus, hypercontractile esophagus, nutcracker, esophagogastric junction outflow obstruction, or hypertensive lower esophageal sphincter were reread using Chicago Classification version 3.0, and were included if they met criteria for jackhammer esophagus. Unpaired t-tests were used for analysis (P≤0.05)., Results: In total, 142 studies were identified with the above diagnoses. After excluding 84 studies, 58 remained for analysis and 17 were found to have jackhammer esophagus (29%). The mean age was 54 (28 to 75), 5 (29%) were males and 12 (71%) were females. The primary indications were NCCP (5), dysphagia (8), and other causes (4) (cough, heartburn, or regurgitation). The mean DCIs were 17,245 mm Hg×s×cm (NCCP), 14,669 mm Hg×s×cm (dysphagia), and 11,264 mm Hg×s×cm (other causes). The mean DCIs were compared: NCCP versus dysphagia (P=0.41), and NCCP versus other causes (P=0.05). Fifteen (88%) had normal bolus transit for both liquid and viscous swallows., Conclusions: In our small sample size, dysphagia was frequently the presenting symptom followed by NCCP. Those with NCCP have a trend toward a higher DCI. Bolus transit appeared to be normal in this patient population. More data are needed to further elucidate the genesis of symptoms and how they relate to the degree of contractility.

Goals: To assess the effect of unilateral versus bilateral lung transplantation (LTx) on esophageal motility and gastroesophageal reflux, and the association with the development of obstructive chronic lung allograft dysfunction (o-CLAD)., Background: We have shown that esophagogastric junction outflow obstruction, incomplete bolus transit, and proximal reflux are all independent risk factors for the development of chronic allograft failure. However, it remains unclear whether these factors are influenced by the type of surgery and how this relates to allograft failure., Study: Patients post-LTx (n=48, 24 female; aged 20 to 73 y) completed high-resolution impedance manometry and 24-hour pH/impedance., Results: Patients who had undergone unilateral LTx were more likely to exhibit esophagogastric junction outflow obstruction (47% vs. 18%; P=0.046) and less likely to exhibit hypocontractility (0% vs. 21%; P=0.058) than those who had undergone bilateral LTx. Although the proportion of patients exhibiting gastroesophageal reflux was no different between groups (33% vs. 39%; P=0.505), those undergoing bilateral LTx were more likely to exhibit proximal reflux (8% vs. 37%; P=0.067). Univariate Cox proportion hazards regression analysis did not show a difference between unilateral versus bilateral LTx in the development of o-CLAD (hazard ratio=1.17; 95% confidence interval, 0.48-2.85; P=0.723)., Conclusion: The type of LTx performed seems to lead to different risk factors for the development of o-CLAD. Physicians should be aware of these differences, as they may need to be taken into account when managing patient's post-LTx.

Background: High-resolution manometry (HRM) is used to assess esophageal motility diseases. Abnormalities in a number of HRM parameters have been reported in patients with dysphagia. However, it is unclear whether some of abnormal HRM parameters are predictive of dysphagia. The aim of this retrospective study was to investigate the roles of HRM parameters in predicting incomplete bolus clearance (IBC) in patients with dysphagia using high-resolution impedance manometry., Methods: A total of 644 wet swallows were reviewed and analyzed in 63 patients with symptoms of dysphagia or reflux who underwent a clinical high-resolution impedance manometry test. IBC was defined based on impedance measurement. The relationship of each of abnormal HRM parameters with IBC was analyzed and their roles in predicting IBC were determined., Results: Patients with symptoms of both dysphagia and reflux showed the highest IBC rate, and patients with symptoms of reflux had the lowest IBC rate. The IBC was more prevalent in the distal esophagus. Premature contractions and peristalsis with large breaks were associated with a higher IBC rate in the proximal esophagus (P<0.05); large breaks, ineffective peristalsis, and abnormalities of the esophageal gastric junction functions were associate with higher IBC rates in the distal esophagus (P<0.05). Abnormalities in a number of motility parameters were able to predict IBC with high specificities and/or high sensitivity, such as pan esophageal pressurization, ineffective peristalsis, and large breaks. Abnormal integrative relaxation pressure of the lower esophageal sphincter with concurrent pan esophageal pressurization, ineffective peristalsis, or large breaks is predictive of IBC with nearly 100% of specificity., Conclusions: Abnormalities in a number of HRM parameters are not only useful in diagnosing esophageal motility diseases, but also valuable in predicting IBC during swallowing.

The Rome IV diagnostic criteria delineates 5 functional esophageal disorders which include functional chest pain, functional heartburn, reflux hypersensitivity, globus, and functional dysphagia. These are a heterogenous group of disorders which, despite having characteristic symptom profiles attributable to esophageal pathology, fail to demonstrate any structural, motility or inflammatory abnormalities on standard clinical testing. These disorders are associated with a marked reduction in patient quality of life, not least considerable healthcare resources. Furthermore, the pathophysiology of these disorders is incompletely understood. In this narrative review we provide the reader with an introductory primer to the structure and function of esophageal perception, including nociception that forms the basis of the putative mechanisms that may give rise to symptoms in functional esophageal disorders. We also discuss the provocative techniques and outcome measures by which esophageal hypersensitivity can be established.

Background: Jackhammer Esophagus is defined as intact esophageal peristaltic contractions with extremely elevated amplitudes. We conducted a retrospective study to identify the frequency of esophageal hypercontractility and the clinical characteristics of Jackhammer Esophagus., Methods: Charts for the patients referred for manometric study at a tertiary-care motility center were reviewed. Data were collected utilizing the new Chicago classification criteria for Jackhammer Esophagus. Concomitant clinical variables were also explored., Results: Eight patients were identified with Jackhammer Esophagus from a total of 205 (127 female/77 male) patients referred for high-resolution esophageal manometry. Jackhammer patients had an average distal contractile integral (DCI) of 9061 mmHg/ sec/ cm and median maximal DCI of 16,433 mmHg/ sec/ cm. The greatest DCI from 15 swallows was 28,875 mmHg/ sec/ cm. Hypercontractility was associated with multipeaked contractions in every Jackhammer patient. The mean lower esophageal sphincter (LES) pressure was 41 mm Hg with 4 patients having a hypertensive pressure of >40 mm Hg. Three of the 8 (37.5%) Jackhammer group had incomplete LES relaxation by integrated relaxation pressure criteria (>15 mm Hg residual pressure). Dysphagia (8/8) was the dominant indication for the manometric study, whereas the clinical background setting was gastroesophageal reflux disease (4/8) and hiatal hernia (1/8). Treatments included smooth muscle relaxation, antireflux regimens, and pneumatic dilation of the LES., Conclusions: Jackhammer Esophagus, an extreme manometric phenotype, was identified in 4.0% of patients referred to a University Motility Center. The patients with these esophageal hypercontractility states present mainly with dysphagia. A subgroup of Jackhammer did have accompanying incomplete LES relaxation and responded to targeted therapy with pneumatic dilatation.

Background and Aims: Combined multichannel intraluminal impedance and esophageal manometry (MII-EM) measures concomitantly bolus transit and pressure changes allowing determination of the functional impact of esophageal motility abnormalities. Ten years ago our laboratory reported MII-EM results in 350 consecutive patients. Since then high-resolution impedance manometry (HRIM) became available and the definitions of ineffective esophageal motility (IEM) and nutcracker esophagus were revised. The aim of this study was to assess the impact of these developments on esophageal function testing., Methods: From August 2012 through May 2013, HRIM was performed in 350 patients referred for esophageal function testing. Each patient received 10 liquid and 10 viscous swallows. While taking advantage of the new technology and revised criteria, HRIM findings were classified according to the conventional criteria to allow more appropriate comparison with our earlier analysis., Results: Compared with the study performed 10 years ago, the prevalence of normal manometry (36% vs. 35%), achalasia (7% vs. 8%), scleroderma (1% vs. 1%), hypertensive lower esophageal sphincter (LES) (7% vs. 7%), and hypotensive LES (1% vs. 2%) remained the same, whereas the prevalence of distal esophageal spasm (9% vs. 3%), nutcracker esophagus (9% vs. 3%), and poorly relaxing LES (10% vs. 3%) decreased and the prevalence of IEM increased (20% vs. 31%) significantly. Compared with the early study, normal liquid bolus transit was significantly different in patients with hypertensive LES (96% vs. 57%) and poorly relaxing LES (55% vs. 100%)., Conclusions: This study brings to light the increase in prevalence of IEM. In addition, it suggests that the hypertensive LES and poorly relaxing LES may each affect bolus transit in about half of these patients.

Lymphocytic esophagitis (LE) is an uncommon poorly defined histologic pattern. Its significance is largely unknown. The goal of our study was to characterize LE clinically, histologically, and immunophenotypically. Biopsies of 45 patients with LE and no intraepithelial granulocytes were selected throughout a 36-month period during routine diagnostic work. After reevaluation, complete absence of intraepithelial granulocytes was confirmed in 21 patients (LE-NG group), and few granulocytes were found in 24 patients (LE-FG). The control group consisted of 28 patients with active esophagitis consistent with reflux and overtly increased intraepithelial lymphocytes (REIL). The ratio of CD4:CD8 intraepithelial lymphocytes (IEL)>1 indicated predominance of CD4 IEL; the ratio ≤1 indicated predominance of CD8 IEL. Dysphagia was the primary complaint in 71%, 54%, and 39% of the patients with LE-NG, LE-FG, and REIL, respectively (P=0.04, LE-NG vs. REIL). Importantly, primary esophageal motility abnormalities were found in 10/11 (91%) tested LE-NG patients, 6/10 (60%) LE-FG patients, and 6/11 (54%) REIL patients. CD4 IELs were predominant in 81%, 50%, and 39% of LE-NG, LE-FG, and REIL cases, respectively (P=0.004, LE-NG vs. REIL), and in 90%, 83%, and 88% of the cases with primary motility abnormalities from the same groups. The prevalence of primary motility abnormalities was significantly higher in patients with CD4-predominant esophagitis than in patients with CD8-predominant esophagitis from all groups (21/24 [83%] vs. 2/8 [25%], P=0.005). A distinctive type of LE with predominance of CD4 IEL is associated with primary motility abnormalities suggesting a diagnostic utility of evaluating CD4 and CD8 subpopulations of T cells in LE.

Background: A novel disposable transnasal endoscopy (DTE) with a portable system has been developed to provide unsedated esophagoscopy by modifying capsule endoscopy. The aim of this study was to assess the feasibility of DTE to evaluate esophageal motor function., Materials and Methods: Patients with or suspected esophageal motility disorders and healthy volunteers were enrolled. Participants underwent esophageal high-resolution manometry and DTE in random order on different days. Motility was observed with DTE at 1, 8, and 16 cm above the gastroesophageal junction., Results: Twenty healthy volunteers and 20 symptomatic subjects participated (8 achalasia, 5 scleroderma, 3 diffuse esophageal spasm, 1 hypertensive peristalsis, 1 peristaltic dysfunction, and 22 normal esophageal function). The normal findings on DTE were as follows. As the subject swallowed water, swallow-induced relaxation with elevation of the lower esophageal sphincter caused the endoscope to cross the Z-line into the gastric lumen. After the passage of water and air, complete closure of the lower esophageal sphincter occurred, with the return of the endoscope to its previous position. During the resting stage of the esophageal body, an air bubble could be seen in the center of the radially wrinkled and occluded lumen. The endoscopic diagnosis was in agreement with the clinical diagnosis in all but 2. Most of the participants reported acceptable discomfort during DTE and 62.5% of the subjects preferred DTE to manometry., Conclusions: DTE can accurately characterize normal esophageal motor function, allowing the diagnosis of esophageal motility disorders. DTE has potential widespread applications, especially in outpatient clinics.

Goals: We aim to look at the prevalence of multichannel intraluminal impedance-pH (MII-pH) studies, which are difficult to interpret secondary to low distal baseline impedance (DBI), and characterize them by their respective diagnosis and DBI., Background: Some patients exhibit low DBI because of fluid retention in the esophagus or acute or chronic mucosal changes. Low DBI can make MII-pH difficult to interpret., Study: We reviewed MII-pH reports from patients studied from January 2002 to December 2009. We conducted a computerized search of the final interpretation for the terms "low," "low baseline," "difficult," and "unable." Reflux reports stating difficult or unable to interpret were analyzed. The associated manometry studies were reviewed to obtain the DBI (mean value at 5 and 10 cm above the lower esophageal sphincter in the pretest esophageal resting state)., Results: Of 2809 MII-pH tracings, 38 (1.4%) were classified as difficult to interpret because of low DBI. The most common underlying manometric diagnosis was ineffective esophageal motility at 36.8%, followed by 28.9% with achalasia, and 10.5% with scleroderma esophagus. An additional 15.8% of patients had increased gastroesophageal reflux on MII-pH. In only 7.9% of patients was no obvious reason for the low DBI identified. Of the 38 patients, 92% had a DBI <1000 Ω, and 58% had a DBI <500 Ω., Conclusions: These findings indicate that difficulty in interpreting MII-pH due to low baseline is very infrequent, and they suggest that it is unadvisable to perform MII-pH testing on patients with a DBI <500 Ω on prior MII-esophageal manometry. If needed, pH only testing off acid-suppressing therapy may be more advisable in these patients.

High-resolution manometry has added significantly to our current understanding of esophageal motor function by providing improved detail and a data analysis paradigm that is more akin to an imaging format. Esophageal pressure topography provides a seamless dynamic representation of the pressure profile through the entire esophagus and thus, is able to eliminate movement artifact and also assess intrabolus pressure patterns as a surrogate for bolus transit mechanics. This has led to improved identification of anatomic landmarks and measurement of important physiological parameters (esophagogastric junction relaxation, distal latency, and contractile integrity). This research has bridged the gap into clinical practice by defining physiologically relevant phenotypes that may have prognostic significance and improve treatment decisions in achalasia, spasm, and hypercontractile disorders. However, more work is needed in determining the etiology of symptom generation in the context of normal or trivial motor dysfunction. This research will require new techniques to assess visceral hypersensitivity and alterations in central modulation of pain and discomfort.

Background and Aims: Intersegmental troughs (ISTs) between striated and smooth muscle contraction segments on high-resolution manometry (HRM) have been linked to hypomotility disorders. We investigated the relationship between ISTs, latency of initiation of smooth muscle contraction, and motor patterns in symptomatic patients and normal controls., Methods: HRM Clouse plots were analyzed in 199 participants (47.2±1.2 y, 112F/87M), categorized into 110 participants with gastroesophageal reflux disease (GERD), 74 symptomatic participants without GERD, and 15 healthy controls. IST length was measured in centimeters and percentage esophageal length, designated extended when ≥20% esophageal length on >30% swallows. Proximal latency was measured as the time interval between onset of skeletal and smooth muscle contraction segments, and designated prolonged when ≥4s in ≥50% of swallows., Results: ISTs of any length were noted in 74.6% swallows and in 92.5% of participants, with a similar frequency across the 3 groups. ISTs and proximal latency were both longer in the GERD group, especially when Barrett esophagus was present, compared with non-GERD patients or controls (P≤0.03 across groups); extended IST and prolonged proximal latency followed similar trends. On multivariate logistic regression, extended IST predicted GERD [odds ratio (OR), 2.30; 95% confidence intervals (CI) 1.18-4.47], as did lower esophageal sphincter pressure <5 mm Hg (OR, 3.79-3.96; 95% CI 1.77-8.49), after controlling for age and sex; prolonged proximal latency predicted both GERD (OR, 2.03; 95% CI 1.01-4.12) and Barrett esophagus (OR 1.91, 95% CI 1.24-2.94)., Conclusions: Measurement of IST and proximal latency add value to HRM analysis, and may be markers of esophageal hypomotility.

Background: Gastroesophageal reflux is implicated in the pathogenesis of asthma and chronic cough. To date most studies have focused on acid reflux measured by pH below the upper esophageal sphincter (UES). The aim of this study was to assess the relationship between cough and reflux through the UES into the pharynx., Methods: Thirty-seven patients with asthma (19) and chronic cough (18) were recruited from the respiratory clinic. Reflux was monitored using a combined multichannel intraluminal impedance and pH probe by detecting (1) bolus reflux episodes within the esophagus and in the pharynx and (2) acidic reflux episodes within the esophagus and in the pharynx. All acid suppressive therapy was stopped for at least 7 days before the study. Demonstration of cough being linked to reflux was achieved using the symptom association probability (SAP). This was calculated using a 2-minute association window between symptoms and bolus entry into the esophagus. SAP was considered positive if >95%., Results: A positive SAP for cough was noted in 7/26 patients reporting symptoms on the day of monitoring. Compared with SAP-negative patients, SAP-positive patients had both a greater number [median (interquartile range), 5(2 to 8) vs. 2(0 to 4), P<0.05] and a higher proportion of reflux episodes crossing the UES into the pharynx [25%(14% to 28%) vs. 7% (2% to 14%), P<0.02]. There was no difference in the number of reflux episodes or acid exposure time in the distal esophagus between SAP-positive and SAP-negative patients. Only 1% to 2% of episodes were detected by the pharyngeal pH sensor., Conclusions: Impedance detected pharyngeal reflux episodes are important factors in symptom production in cough patients.

Background: Combined multichannel intraluminal impedance and manometry provides simultaneous evaluation of bolus transit and pressure changes within the esophagus. The aim of this study was to analyze and to compare distal esophageal impedance values between healthy volunteers and patients with normal and abnormal esophageal manometry., Materials and Methods: We analyzed multichannel intraluminal impedance and manometry studies in 130 individuals (79 women, mean age 53 y, age range 17 to 85 y). There were 20 healthy volunteers and 20 patients with normal manometry. Patients with abnormal manometry were separated into nutcracker esophagus (n=20), distal esophageal spasm (n=20), ineffective esophageal motility (IEM, n=20), achalasia (n=20), and scleroderma esophagus (n=10). Manometric and MII parameters were assessed during 10 liquid and 10 viscous swallows. MII findings included esophageal impedance values and number of complete and incomplete bolus transits (CBTs). Esophageal impedance values from 2 distal impedance measuring segments (5 and 10-cm above lower esophageal sphincter) were assessed over a 2 to 3 seconds interval before the first liquid and the first viscous swallow, and 2 to 3 seconds after the tenth viscous swallow. The average values of esophageal impedance measured at 5 and 10-cm above lower esophageal sphincter (distal esophageal impedance) were calculated before liquid [distal baseline impedance (DBI)] and after 10 liquid swallows [distal liquid impedance (DLI)] and after 10 viscous swallows [distal viscous impedance (DVI)]. The correlations between DLI and DVI and number of CBT for liquid and viscous as well as distal esophageal amplitude (DEA) for liquid and viscous were also assessed using Pearson correlation coefficient., Results: Patients with achalasia or scleroderma esophagus had significantly lower DBI, DLI, and DVI than healthy volunteers, patients with normal manometry, nutcracker esophagus, or distal esophageal spasm. Patients with IEM had significantly lower DBI, DLI, and DVI than healthy volunteers or patients with nutcracker esophagus. Patients with IEM had significantly lower DLI and DVI than patients with normal manometry and significantly higher DVI than patients with achalasia. Overall, there was a significant correlation between DLI and CBTs during 10 liquid swallows (r=0.7, P<0.0001), DVI and CBTs during 10 viscous swallows (r=0.6, P<0.0001), DLI and DEA during 10 liquid swallows (r=0.5, P<0.0001), and DVI and DEA during 10 viscous swallows (r=0.5, P<0.0001)., Conclusions: Our results suggest that evaluation of distal esophageal impedance may assist in recognition and diagnosis of esophageal motility abnormalities.

Two recent advances have revolutionized the performance of clinical esophageal manometry; the introduction of practical high resolution manometry (HRM) systems and the development of sophisticated algorithms to display the expanded manometric dataset as pressure topography plots. We utilized a large clinical experience of 400 consecutive patients and 75 control subjects to develop a systematic approach to analyzing esophageal motility using HRM and pressure topography plots. The resultant classification scheme has been named as the Chicago Classification of esophageal motility. Two strengths of pressure topography plots compared with conventional manometric recordings were the ability to (1) delineate the spatial limits, vigor, and integrity of individual contractile segments along the esophagus and (2) to distinguish between loci of compartmentalized intraesophageal pressurization and rapidly propagated contractions. Making these distinctions objectified the identification of distal esophageal spasm, vigorous achalasia, functional obstruction, and nutcracker esophagus subtypes. Applying these distinctions made the diagnosis of spastic disorders quite rare: spasm in 1.5% of patients, vigorous achalasia in 1.5%, and a newly defined entity, spastic nutcracker, in 1.5%. Ultimately, further clinical experience will be the judge, but it is our expectation that pressure topography analysis of HRM data, along with its well-defined functional implications, will prove valuable in the clinical management of esophageal motility disorders.

Symptoms of chest pain and dysphagia are common in the adult population. Most patients initially undergo an evaluation to exclude anatomic causes (ie, esophagitis, stricture) and cardiovascular disease as the etiology of these symptoms. Patients with persistent symptoms may then be referred for specialized testing of the esophagus, including esophageal manometry. Disorders of esophageal motility, which include achalasia, diffuse esophageal spasm, nutcracker esophagus, hypertensive lower esophageal sphincter, and ineffective motility are often identified in these patients. Unfortunately, the etiology of these disorders has not been well characterized and the treatment has not been standardized. This review will briefly discuss the impact, etiology, and diagnosis of esophageal motility disorders, and then focus on the medical management of these disorders using evidence from well-designed, prospective studies, where available.

Esophageal manometry is a specialized procedure used to evaluate lower and upper esophageal sphincter pressure, esophageal body contraction amplitude, and peristaltic sequence. The procedure is clinically useful in evaluation of a patient with nonstructural dysphagia, unexplained or noncardiac chest pain, a compendium of symptoms suggested because of gastroesophageal reflux disease, and in the preoperative evaluation for antireflux surgery. Manometric findings in 95 normal subjects evenly distributed across age groups were reported in 1987, and are the values still used in our and most laboratories today. The subsequent review will offer our "view" on the clinical utility of esophageal manometry, on the basis of years of experience and performance techniques that have remained constant over decades.

Background: Nutcracker esophagus (NE) is a manometric finding defined by peristaltic contractions with a mean distal esophageal amplitude (DEA) >180 mm Hg. This threshold has been selected as it exceeds the average DEA in healthy volunteers by 2 SDs. Since its introduction the clinical significance of this finding has been challenged, as many patients with NE are asymptomatic., Aim: To evaluate whether defining NE based on a different DEA threshold would be clinically more meaningful., Methods: Retrospective review of prospectively collected manometry data between October 2001 and December 2003. Using previously published normal DEA values (mean and SD) patients with NE were stratified into 3 groups: group A (2 to 3 SD above mean): DEA 180 to 220 mm Hg; group B (3 to 4 SD above mean): DEA 220 to 260 mm Hg; and group C (>4 SD above mean): DEA >260 mm Hg. Symptoms, esophageal acid exposure, bolus transit data, and lower esophageal sphincter data were reviewed., Results: The stratification of 56 NE patients into groups A, B, and C were 31, 16, and 9, respectively. The proportion of patients presenting with chest pain increased from 23% in group A to 69% in group B and 100% in group C. Patients in group C had significantly (P<0.05) higher mean lower esophageal sphincter pressure, shorter bolus transit time, and lower frequency of abnormal reflux., Conclusions: A revised definition of NE to include patients with a DEA >260 mm Hg, and possibly those with >220 may have greater clinical relevance.

Catheter based high frequency intraluminal ultrasound (HFIUS) imaging is a powerful tool to study esophageal sensory and motor function and dysfunction in vivo in humans. It has provided a number of important insights into the longitudinal muscle function of the esophagus. Based on the ultrasound images and intraluminal pressure recordings, it is clear that there is synchrony in the timing as well as the amplitude of contraction between the circular and the longitudinal muscle layers of the esophagus in normal subjects. On the other hand, in patients with spastic disorders of the esophagus, there is an asynchrony of contraction related to the timing and amplitude of contraction of the two muscle layers during peristalsis. Achalasia, diffuse esophageal spasm, and nutcracker esophagus (spastic motor disorders of the esophagus) are associated with hypertrophy of the circular as well as longitudinal muscle layers. A sustained contraction of the longitudinal muscle of the esophagus is temporally related to chest pain and heartburn and may very well be the cause of symptoms. Longitudinal muscle function of the esophagus can be studied in vivo in humans using dynamic ultrasound imaging. Longitudinal muscle dysfunction appears to be important in the motor and sensory disorders of the esophagus.

Background: In GERD patients, ineffective esophageal motility (IEM), a hypocontractile disorder, is the most common motor abnormality. IEM has been associated with reflux in both the supine and upright position, prolonged esophageal clearance, and delayed of bolus transport. IEM has been equally present in erosive and in nonerosive GERD., Goal: Considering that reflux has been found to be more severe in erosive GERD than in nonerosive GERD patients and that IEM delays esophageal clearance, our hypothesis is that patients with erosive GERD have more severe IEM than those with nonerosive disease., Study: A retrospective review of consecutive manometries of patients with the chief complaint of heartburn and a diagnosis of IEM were performed, and patients with both erosive and nonerosive GERD were selected. According to the number of ineffective contractions, IEM was stratified into three groups: 30% to 40%, mild; 50% to 60%, moderate; and greater than 60%, severe. We also registered the number of low amplitude, failed, and normal waves in each manometry of both groups. We evaluated 110 patients: 70 (64%) with erosive GERD and 40 (36%) with nonerosive GERD. The percentage of mild, moderate and severe IEM was similar in erosive and in nonerosive GERD patients, as well the number of low amplitude, failed or normal waves (P < 0.5)., Conclusion: There were no differences between the severity of IEM in erosive and in nonerosive GERD patients.

Background: Esophageal motility abnormalities have been observed in patients with gastroesophageal reflux disease., Goals: The aim of the present study was to determine if esophageal motor disorders in patients with a positive response to the omeprazole test are related to the existence of reflux or they are concomitant findings., Study: A 24-hour pH monitoring and a stationary manometry were performed on 128 patients: 49 of them had normal manometry, 31 hypotensive lower esophageal sphincter, 29 motor disorder in esophageal body, and 19 hypotensive lower esophageal sphincter and motor disorder in esophageal body., Results: We found an association between the presence of abnormal reflux and motor disorder in esophageal body (chi test; P < 0.05). However, ineffective esophageal motility was the disorder most strongly related to reflux, whereas the hypercontractile disorders were not clearly attributed to it., Conclusions: Esophageal manometric abnormalities should be considered cautiously before considering a motor disorder as a consequence of abnormal reflux.

Background: Traditionally 10 wet swallows are used to assess esophageal motility. Given this relative small number of swallows concerns have been raised whether the abnormal findings based on 10 wet swallows would persist with another set of 10 wet swallows., Purpose: To evaluate whether 10 wet swallows during esophageal manometry is an adequate sampling of esophageal motility., Methods: Over a 4-month-period 100 consecutive patients (excluding patients with achalasia) were given 20 liquid (5 ml each) swallows. The standard manometry technique in our laboratory was performed using 10 wet swallows with pressure transducers placed 4 cm and 9 cm above the lower esophageal sphincter (LES). The diagnosis was based on the initial 10 swallows. The additional 10 swallows were blinded and separately analyzed, then compared with the initial swallows for each patient., Results: Of the 100 studies (64 women; mean age 51; range 21-85), 91 had a consistent diagnosis for both pairs of 10 swallows. The number of abnormal swallows (ineffective or simultaneous) differed between the 2 sets of 10 swallows in 9 patients, but, when the first 10 were analyzed vs. all 20 swallows only 2 patients had discordant diagnoses. There was an excellent agreement between the 1st and 2nd set of 10 swallows (kappa = 0.846) and between the first 10 and all 20 swallows (kappa = 0.965)., Conclusions: Ten wet swallows are sufficient for making a manometric diagnosis, because additional swallows are likely to change the diagnosis in only 2% of patients.

Clinical reports suggest that nonsteroidal anti-inflammatory drugs (NSAIDs) use might induce esophageal symptoms and damage, including esophagitis, but experimental data are conflicting, and some indicate that NSAIDs improve mucosal damage. It is not known whether patients with endoscopically diagnosed esophagitis during NSAID use have different baseline reflux patterns from patients with reflux esophagitis and no NSAID use. Two groups of patients with and without chronic NSAID use and esophagitis were prospectively studied. Twenty-four-hour pH monitoring and esophageal manometry were performed in all patients who were free of NSAID and any other drug use during tests. Esophageal motility and reflux patterns did not differ in patients with esophagitis regardless of the presence or absence of NSAID use. However, the subgroup of patients with grades II and III esophagitis and chronic NSAID use had a significantly greater lower esophageal sphincter (LES) pressure and a less severe intraesophageal pH profile than patients without NSAID use. In agreement with clinical reports, these results suggest that NSAID use may aggravate an otherwise milder acid-related esophageal disease in a subgroup of patients.

The hypertensive lower esophageal sphincter is an infrequent primary esophageal motor disorder characterized by elevated mean lower esophageal sphincter pressure (greater than or equal to 3 SD from that of controls), sphincter relaxations greater than 75%, and normal peristaltic activity in the esophageal body. This disorder is frequently associated with the nutcracker esophagus. We report our clinical and manometric experience with the isolated hypertensive lower esophageal sphincter (i.e., unassociated with other motor disorders), which constituted 2.7% of all patients who complained of dysphagia or chest pain referred for manometry during the period from October 1982 to February 1991.

We report a middle-aged woman with nutcracker esophagus who progressed to classic achalasia in two years. Several previous reports have documented progression of nutcracker esophagus to diffuse esophageal spasm and diffuse esophageal spasm to achalasia, but the only previous report of nutcracker esophagus progressing to achalasia was in a child. Our case suggests that, in some instances, nutcracker esophagus and achalasia may share the same pathogenesis.

The hypertensive lower esophageal sphincter (LES) (mean LES pressure greater than 45 mm Hg; LES relaxation greater than 75%; normal peristalsis) is a poorly characterized motility disorder associated with chest pain and dysphagia. Therefore, we carried out a multidisciplinary study to assess esophageal pressures and function in 15 symptomatic hypertensive LES patients (3 men, 12 women; mean age, 53 years). On-line computer analysis showed a significant (p less than 0.05) increase in LES pressure (55.5 versus 14.9 mm Hg) and residual pressure (6.8 versus 1.1 mm Hg) as well as a decrease in percentage of LES relaxation (87 versus 93%) in patients compared with age-matched controls. All patients had normal peristalsis but 7 of 15 had nutcracker esophagus (mean distal amplitude, 216 mm Hg). No patient had evidence of impaired liquid transport on barium esophagram. The emptying of solids as assessed by radionuclide scans was normal in 14 of 15 patients. Of the 12 patients who completed both psychological inventories, nine had elevated scores on scales assessing anxiety and somatization. The heterogenous nature of this disorder is illustrated by a patient with a changeable narrowing in the distal esophagus associated with the transient impaction of a marshmallow. Dysphagia but not chest pain improved after pneumatic dilatation. We conclude that the hypertensive LES is a heterogenous disorder. Despite abnormal LES parameters, most patients have normal esophageal function, and frequent psychological abnormalities may contribute to their report of symptoms. A minority have abnormal esophageal transit.