No full-text available
To read the full-text of this research,
you can request a copy directly from the authors.
[A case report of jackhammer esophagus]
Abstract
A 39-year-old female patient was referred to our hospital with a suspected esophageal motility disorder following a 4-month history of hiccup and dysphagia. Her past medical and family histories were unremarkable. Prior investigation with upper gastrointestinal endoscopy and esophagography failed to demonstrate any obvious pathology. Therefore, high-resolution manometry was performed, which showed the following: an integrated relaxation pressure (IRP) of 11.5mmHg; a distal contractile integral (DCI) of 6543mmHg-s-cm; and, a highest DCI of 9289mmHg-s-cm. A diagnosis of jackhammer esophagus was, therefore, considered. We reported on the details of this case and reviewed the relevant literature.
... JE is considered to be present when the HRM findings show that integrated relaxation pressure (IRP) (mmHg) and distal latency (DL) (sec) are normal and distal contractile integral (DCI) is hypercontractile, i.e., exceeds 8000 mmHg-scm, in 2 or more out of 10 swallows [1]. However, there have been only scattered case reports to date [2][3][4][5][6][7][8]. Many aspects of its clinical course continue to remain unknown. ...
... The duration of illness averaged 8.9 years in our own cases as opposed to 2.5 years in the previously reported cases. Aside from the HRM findings, there have been few reports regarding imaging findings in relation to contrast esophagography and the thickness of the esophageal muscular layer, but only one case in which the contrast esophagography findings were described [3] was negative for abnormal contractions, and of the 3 cases in which the thickness of the esophageal muscular layer was described [3,5,7], 1 case was negative and the other 2 cases were positive. Comparisons between methods of treatment showed that a PPI was used to treat all of our own cases, but the improvement in symptoms was inadequate. ...
... The duration of illness averaged 8.9 years in our own cases as opposed to 2.5 years in the previously reported cases. Aside from the HRM findings, there have been few reports regarding imaging findings in relation to contrast esophagography and the thickness of the esophageal muscular layer, but only one case in which the contrast esophagography findings were described [3] was negative for abnormal contractions, and of the 3 cases in which the thickness of the esophageal muscular layer was described [3,5,7], 1 case was negative and the other 2 cases were positive. Comparisons between methods of treatment showed that a PPI was used to treat all of our own cases, but the improvement in symptoms was inadequate. ...
Background
Although jackhammer esophagus (JE) requires proper treatment, many aspects of its clinical course remain unknown.
Method
The 4 cases were in patients diagnosed with JE among the 447 patients who underwent high-resolution manometry (HRM) at our hospital between January 2009 and October 2014, and we assessed the patients’ background, their HRM, gastrointestinal endoscopy, and contrast esophagography findings, whether they had esophageal muscle thickening, and their clinical course.
Result
All 4 cases were treated for proton pump inhibitor–resistance nonerosive reflux disease, and all of them were ultimately diagnosed as JE. The test findings revealed an esophageal hiatal hernia and esophageal muscle thickening in every case, contrast esophagography showed abnormal contractions in 3/4 cases. During a mean follow-up period of 3.3 years, progression from nutcracker esophagus [NE; the Chicago Classification (2012 version)] to JE was observed during the course of 2 of the 4 patients. In 1 of the 4 patients, the symptoms improved in the absence of treatment. Among the 3 patients who received treatment, the symptoms of 2 patients were controlled within tolerable intensity by conservative treatment, but surgical treatment was selected in the other case. However, because the symptoms recurred after the operation, peroral endoscopic myotomy was performed in addition.
Conclusions
Because the pathology at the onset and clinical course of JE are not uniform, it is necessary to tailor treatment policy to each individual case and to consider altering treatment policy according to the patient’s clinical course.
This study aimed to refine the criteria for esophageal hypercontractility in high-resolution esophageal pressure topography (EPT) and to examine the clinical context in which it occurs.
A total of 72 control subjects were used to define the threshold for hypercontractility as a distal contractile integral (DCI) greater than observed in normals. In all, 2,000 consecutive EPT studies were reviewed to find patients exceeding this threshold. Concomitant EPT and clinical variables were explored.
The greatest DCI value observed in any swallow among the control subjects was 7,732 mm Hg-s-cm; the threshold for hypercontractility was established as a swallow with DCI >8,000 mm Hg-s-cm. A total of 44 patients were identified with a median maximal DCI of 11,077 mm Hg-s-cm, all with normal contractile propagation and normal distal contractile latency, thereby excluding achalasia and distal esophageal spasm. Hypercontractility was associated with multipeaked contractions in 82% of instances, leading to the name "Jackhammer Esophagus." Dysphagia was the dominant symptom, although subsets of patients had hypercontractility in the context of esophagogastric junction (EGJ) outflow obstruction, reflux disease, or as an apparent primary motility disorder.
We describe an extreme phenotype of hypercontractility characterized in EPT by the occurrence of at least a single contraction with DCI >8,000 mm Hg-s-cm, a value not encountered in control subjects. This phenomenon, branded "Jackhammer Esophagus," was usually accompanied by dysphagia and occurred both in association with other esophageal pathology (EGJ outflow obstruction, reflux disease) or as an isolated motility disturbance. Further studies are required to define the pathophysiology and treatment of this disorder.
Achalasia (Ach), diffuse esophageal spasm (DES), nutcracker esophagus (NE), and nonspecific motility disorder (NSMD) are described primary esophageal body motility disorders; however, their clinical symptom correlation is poorly understood. The aim of this study is to examine the association between a patient's presenting symptoms and their manometric diagnosis.
Manometric findings and reported symptoms of all patients undergoing esophageal manometry at the Creighton University Medical Center were prospectively entered in a database. Twenty-four-year data from 1984 through 2008 were accessed and analyzed.
Of the 4,215 patients, 130 (3.1%) had Ach, 192 (4.6%) had DES, 290 (6.9%) had NE, 508 (12.1%) had NSMD, and 3,095 (73.4%) had normal esophageal body motility. There was significant symptom overlap between the groups. Ach and DES had a similar symptom distribution, with dysphagia being the predominant symptom. Patients with NE, normal body motility, and NSMD presented predominantly with reflux symptoms. There was an increasing prevalence of esophageal dysmotility (DES and NSMD) with age, and women were found to be more likely to have NE than men.
In an individual, reported symptoms do not correlate with their manometric diagnosis in a predictable fashion, and a thorough physiological assessment should be obtained to understand and diagnose the disease process. Esophageal motility deteriorates with age.
Esophageal motility abnormalities include a series of manometric findings that differ to a significant degree from findings in normal, asymptomatic volunteers.
Current review summarizes conventional and high-resolution esophageal manometry criteria used to define and characterize esophageal hypertensive motility abnormalities. Key
In the conventional esophageal manometry classification scheme hypertensive esophageal motility abnormalities include nutcracker esophagus (average distal contraction amplitude >180 mmHg), hypertensive lower esophageal sphincter (average resting LES pressure >45 mmHg) and poorly relaxing lower esophageal sphincter (average LES residual pressure >8 mmHg). The new, high resolution esophageal manometry scheme includes in the group of hypertensive peristaltic disorders hypertensive peristalsis ("nutcracker esophagus": mean DCI >5000 mmHg*sec*cm) and hypercontractile esophagus ("jackhammer esophagus": at least one contraction with DCI > 8,000 mmHg*sec*cm) and defines a separate group for disorders with impaired esophageal-gastric junction relaxation (mean integrated residual (LES) pressure >15 mmHg).
Hypertensive motility disorders represent a heterogeneous condition subdivided into hypercontractile esophagus and hypertensive peristalsis. Further studies are required to determine the clinical relevance of this new classification.
The objective of this study was to evaluate the association between high-resolution manometry (HRM) and impedance findings and symptoms in patients with nutcracker esophagus (NE).
After institutional review board approval retrospective review of a prospectively maintained database identified patients who were diagnosed with NE as per the Chicago classification (distal contractile integral [DCI] > 5000 mmHg-s-cm) at Creighton University between October 2008 and October 2010. Patients with achalasia or a history of previous foregut surgery were excluded. NE patients were sub-divided into: (i) Segmental (mean distal esophageal amplitude [DEA] at 3 and 8 cm above lower esophageal sphincter [LES] < 180 mmHg) (ii) Diffuse (mean DEA at 3 and 8 cm above LES > 180 mmHg) and (iii) Spastic (DCI > 8000 mmHg-s-cm).
Forty-one patients (segmental: 13, diffuse: 4, spastic: 24) satisfied study criteria. Patients with segmental NE would have been missed by conventional manometry criteria as their DEA < 180 mmHg. A higher percentage of patients with spastic NE (63%) had chest pain when compared to patients with segmental NE (23%) and diffuse NE (25%). There was a significant positive correlation between chest pain severity score and DCI while there was no significant correlation between dysphagia severity and DCI.
In patients diagnosed with NE using the Chicago classification presence and intensity of chest pain increases with increasing DCI. The present criteria (> 5000 mmHg-s-cm) seems to be too sensitive and has poor symptom correlation. Adjusting the criteria to 8000 mmHg-s-cm is more relevant clinically.
Achalasia, diffuse esophageal spasm, nutcracker esophagus, and the hypertensive lower esophageal sphincter are considered primary esophageal motility disorder. These disorders are characterized by esophageal dysmotility that is responsible for the symptoms. While there is today a reasonable consensus about the pathophysiology, the diagnosis, and the treatment of achalasia, this has not occurred for the other disorders. A careful evaluation is therefore necessary before an operation is considered.
Obesity is a risk factor for GERD and a potential modulator of esophageal motility.
To assess whether obese patients differ from non-obese patients in terms of esophageal motility and reflux.
Patients (n = 332) were categorized in GERD and controls after clinical assessment, esophageal manometry, and pH monitoring. Non-obese (BMI 16-29.9) and obese (BMI 30-68) were compared in regard of distal esophageal amplitude (DEA), LES pressure (LESP), manometric diagnosis, and esophageal acid exposure (EAE).
Obese showed higher DEA in both controls (122 ± 53 vs. 97 ± 36 mmHg, p = 0.041) and GERD patients (109 ± 38 vs. 94 ± 46 mmHg, p < 0.001), higher LESP in GERD patients (20.5 ± 10.6 vs. 18.2 ± 10.6 mmHg, p = 0.049), higher frequency of nutcracker esophagus in controls (30 vs. 0%, p = 0.001), lower frequency of ineffective motility in GERD patients (6 vs. 20%, p = 0.001), and higher EAE in both controls [total EAE: 1.6% (0.7-5.1) vs. 0.9% (0.2-2.4), p = 0.027] and GERD patients [upright EAE: 6.5% (3.8-11.1) vs. 5.2% (1.5-10.6), p = 0.048]. Multiple linear regression showed that BMI was associated either with EAE (p < 0.001), DEA (p = 0.006), or LESP (in men, p = 0.007).
Obese patients differed from non-obese in terms of esophageal motility and reflux, regardless of the presence of GERD. Obese patients showed stronger peristalsis and increased acid exposure in the esophagus.
Nutcracker esophagus (NE) is a well-described esophageal motility disorder often implicated as the cause of chest pain (CP). The aim of this study was to analyze the role of peristaltic amplitude, lower esophageal sphincter (LES) pressure, and 24 h pH scores in patient symptomatology.
After obtaining Institutional Review Board approval, a retrospective review of manometric data from 1984 to 2008 at the esophageal center was done to identify patients meeting NE criteria (mean distal esophageal body amplitude of >180 mmHg). The data for patient's symptoms, manometric findings including the amplitude of the distal two esophageal body peristalses, LES pressure, and 24 h pH score were extracted and analyzed.
Out of 4,923 patients, 313 (6.4%) patients met the manometric criteria for NE, and of these, 298 patients had complete manometry data along with at least 1 reported symptom. CP was associated with LES competence, with a significantly higher percentage of patients with high LES pressure complaining of CP (p < 0.05). There was no relationship of with the mean amplitude of esophageal body pressure (p > 0.05) or with distal esophageal acid exposure (p > 0.05).
CP is a commonly reported symptom in patients with manometry findings of NE. However, CP is related to LES competence rather than the amplitude of the esophageal body waves or 24 h pH monitoring scores.
Abstract Late phase III migrating motor complex activity has been said to be primarily retroperistaltic but has not been assessed with high resolution manometry or three-dimensional colour pressure plots (pressure/time/distance). Duodenal phase III was examined in healthy young volunteers (seven male, two female) with a 20-lumen assembly. With the most proximal sidehole in the distal antrum, after a 4.5-cm interval 18 sideholes at 1.5-cm intervals spanned the duodenum with a final sidehole 3 cm beyond. Fasting pressures were recorded until phase III occurred. Comparisons were made between proximal (P) and distal (D) duodenum during early (E) (first 0.5-1 min) and late (L) (last 0.5-1 min) phase III. With colour pressure analysis, 121 of 180 pressure wave (PW) sequences were purely antegrade, two purely retrograde and 57 bidirectional. Ten of fifty-seven bidirectional PW sequences were complex, branching to become two separate sequences. Bidirectional sequences occurred more frequently in late than early phase III (L 43 vs. E 14 of 57), but their occurrence did not differ between proximal and distal duodenum (P31 vs. D 24 of 57). Antegrade propagation velocity was faster in late compared with early phase III (L 28.50 vs. E 17.05 mm s(-1); P = 0.006), but did not differ between proximal and distal duodenum. Colour pressure analysis also indicated an intermittent segmental pattern to phase III, with each subject exhibiting a change in velocity or direction, or a relative failure of peristalsis somewhere along the duodenum during part of phase III. Duodenal phase III is not homogenous and, in contrast with previous studies, does not primarily constitute a retroperistaltic pump. Colour pressure analysis is useful in interpreting intraluminal pressure profiles and may improve the sensitivity and specificity of clinical studies.
The named primary esophageal motility disorders (PEMDs) are achalasia, diffuse esophageal spasm (DES), nutcracker esophagus (NE), and hypertensive lower esophageal sphincter (HTN-LES). Although the diagnosis and treatment of achalasia are well defined, such is not the case with the other disorders.
(1) Symptoms do not reliably distinguish PEMDs from gastroesophageal reflux disease; (2) esophageal function tests are essential to this distinction and to identifying the type of PEMD; (3) minimally invasive surgery is effective for each condition; and (4) the laparoscopic approach is better than the thoracoscopic approach.
University hospital tertiary care center.
Retrospective review of a prospectively collected database. Patients and
A diagnosis of PEMD was established in 397 patients by esophagogram, endoscopy, manometry, and pH monitoring. There were 305 patients (77%) with achalasia, 49 patients (12%) with DES, 41 patients (10%) with NE, and 2 patients (1%) with HTN-LES. Two hundred eight patients (52%) underwent a myotomy by either a thoracoscopic or a laparoscopic approach.
Ninety-nine patients (25%) had a diagnosis of gastroesophageal reflux disease at the time of referral and had been treated with acid-suppressing medications. In achalasia and DES, a thoracoscopic or laparoscopic myotomy relieved dysphagia and chest pain in more than 80% of the patients. In contrast, in NE the results were less predictable, and the operation most often failed to relieve symptoms.
These results show that (1) symptoms were unreliable in distinguishing gastroesophageal reflux disease from PEMDs; (2) esophageal function tests were essential to diagnose PEMD and to define its type; (3) the laparoscopic approach was better than the thoracoscopic approach; (4) a laparoscopic Heller myotomy is the treatment of choice for achalasia, DES, and HTN-LES; and (5) a predictably good treatment for NE is still elusive, and the results of surgery were disappointing.
Although the etiology of gastroesophageal reflux disease (GERD) is multifactorial, the pathophysiology of the disease in morbidly obese patients remains incompletely understood. The aims of this study were to compare in morbidly obese (body mass index (BMI) > or =35) and nonmorbidly patients (BMI <35) with GERD: (a) lower esophageal sphincter (LES) profile; (b) esophageal body function; and (c) esophageal acid exposure. We reviewed esophageal manometry and ambulatory 24-hour pH monitoring studies of 599 consecutive patients with GERD (DeMeester score >14.7). Patients were divided into two groups according to the BMI: (1) 520 patients (86.8%) with BMI <35 and (2) 79 patients (13.2%) with BMI > or =35. While the DeMeester score was not different between the two groups, morbidly obese patients had higher LES pressure and higher amplitude of peristalsis in the distal esophagus (DEA). Among these patients, LES and DEA pressures were often hypertensive. A linear regression model showed that BMI, LES pressure, LES abdominal length, and DEA were independently associated with the DeMeester score. These data showed that: (a) BMI was independently associated to the severity of GERD; and (b) in most morbidly obese patients with GERD, reflux occurred despite normal or hypertensive esophageal motility. These findings show that the pathophysiology of GERD in morbidly obese patients might differ from that of nonobese patients, suggesting the need for a different therapeutic approach.