Untreated Achalasia Research Articles

Evaluation of esophagogastric junction relaxation by 4-second Integrated Relaxation Pressure in achalasia using High Resolution Manometry with water-perfused catheters.

Relaxation of the esophagogastric junction (EGJ) is now evaluated calculating 4-second integrated relaxation pressure (4-s IRP) by high resolution manometry (HREPT). Solid-state catheters have been used to define abnormal values. Our aim was to evaluate 4-s IRP in esophageal achalasia using HREPT with perfused catheters. From June 2009 to June 2013, 936 HREPT studies have been performed in our unit. Of these, 194 patients having treated achalasia were excluded. Control group was constituted by 695 patients without achalasia, and 47 patients with untreated achalasia constituted the study group. HREPT was performed with water-perfused catheters. To establish the cut-off value for 4-s IRP that better discriminate patients with achalasia from all other patients, a receiver operating characteristic (ROC) analysis was performed. Twenty three of 47 achalasia patients (49%) showed a 4-s IRP under 15 mmHg; and seven (15%) had a value under modified Chicago criteria. A cut-off value for 4-s IRP of 6.5 mmHg, calculated by ROC analysis, highly discriminates achalasia from the rest of the patients and especially from scleroderma patients (area under the curve: 0.997, 95% CI: 0.995-1.000; p < 0.001). Cut-off values for 4-s IRP defined using HREPT with solid-state catheters are not adequate for diagnosing esophageal achalasia with water-perfused systems. A lower value, i.e., 6.5 mmHg, is suggested for this equipment. The diagnostic criteria of esophageal achalasia should be modified for HREPT performed with water-perfused systems.

Mo1892 Are Esophageal Mucosal and Motor Alterations Related to Esophageal Visceral Hypersensitivity in Untreated Achalasia Patients?

Figure 1: Panel A: Swallow, B: TLESR with no Reflux, C: TLESR with reflux. Row 1: TZ line extracted automatically by the computer program (red line) superimposed on the HRM recording. Note the differences in amplitude and duration of the TZ signal, Row 2) Impedance contour map plot, Row 3) Symbolic representation of the normalized TZ signal indicating onset, duration, and a time window index to find lift amplitude on the impedance TZ signal.

Barrett’s Esophagus and Esophageal Adenocarcinoma Are Common After Treatment for Achalasia

Achalasia is characterized by esophageal aperistalsis and impaired relaxation of the lower esophageal sphincter (LES). This contrasts with an insufficient LES, predisposing to gastro-esophageal reflux and Barrett's esophagus. The co-incidence of achalasia and BE is rare. Pneumatic dilatation (PD) may lead to gastro-esophageal reflux, Barrett's esophagus development, and esophageal adenocarcinoma. To determine the incidence of Barrett's esophagus and esophageal adenocarcinoma in achalasia patients treated with PD. We performed a single-center cohort follow-up study of 331 achalasia patients treated with PD. Mean follow-up was 8.9 years, consisting of regular esophageal manometry, timed barium esophagram, and endoscopy. Twenty-eight (8.4%) patients were diagnosed with Barrett's esophagus, one at baseline endoscopy. This corresponds with an annual incidence of Barrett's esophagus of 1.00% (95% CI 0.62-1.37). Hiatal herniation was present in 74 patients and 21 developed Barrett's esophagus compared to seven of 257 patients without a hiatal hernia. Statistical analysis revealed a hazard ratio of 8.04 to develop Barrett's esophagus if a hiatal hernia was present. Post-treatment LES pressures were lower in patients with Barrett's esophagus than in those without (13.9 vs. 17.4 mmHg; p = 0.03). Two (0.6%) patients developed esophageal adenocarcinoma during follow-up. Barrett's esophagus is incidentally diagnosed in untreated achalasia patients despite high LES pressures, but is more common after successful treatment, especially in the presence of hiatal herniation. Patients treated for achalasia should be considered for GERD treatment and surveillance of development of Barrett's esophagus, in particular, when they have low LES pressures and a hiatal herniation.

Achalasia in the elderly patient: a comparative study

Achalasia is a primary esophageal motor disorder secondary to the degeneration of ganglion cells of the inhibitory intramural myenteric plexus. It affects both sexes similarly and has two peaks of incidence, one in the 3rd to 4th decades of life and the other after 60 years of age. The effect of age on esophageal motility of patients with achalasia is not well known. Studies have shown that healthy older people, when compared to the young, have: a) a lower number of ganglion cells in the intramural myenteric plexus; b) a reduced normal relaxation of the lower esophageal sphincter; and c) a reduced esophageal peristalsis. Thus, as both age and achalasia can produce comparable degenerative changes in the intramural myenteric plexus, it is possible that advanced age could be an important factor in enhancing the clinical and manometric abnormalities commonly found in patients with achalasia. To compare the clinical, radiological and manometric findings in young as compared to elderly (>60 years old) achalasia patients. A retrospective study of a group of patients with untreated achalasia separated into young and elderly patients. Demographic, clinical, serology for Chagas' disease, radiological and manometric data were compared between these groups. The level of significance was P<0.05. The study included 105 patients, 52 young (25 M/27 F, mean age 40 years old) and 53 elderly (21 M/32 F, mean age 70 years old). The elderly group had a higher prevalence of Chagas' disease (P = 0.004) and a lower pressure of the lower esophageal sphincter [26.4 mm Hg vs 31.9 mm Hg] P = 0.001, a difference that persisted when analyzed only elderly and young patients with idiopathic achalasia. Younger patients had a higher prevalence of heartburn (P = 0.001) and chest pain (P = 0.012) than the elderly. Elderly patients with achalasia had a lower esophageal sphincter pressure than the young, even when we excluded patients with Chagas' disease but, as a group, they were less symptomatic.

High Prevalence of Heartburn and Low Acid Sensitivity in Patients with Idiopathic Achalasia

Heartburn is frequently reported by patients with achalasia before treatment. However, the esophageal sensitivity to acid as a possible mediator of this symptom has not been previously evaluated. To evaluate the prevalence of gastroesophageal reflux symptoms and the esophageal sensitivity to acid perfusion in patients with untreated achalasia. Forty patients with achalasia were prospectively evaluated. Forty-three patients with gastroesophageal reflux disease comprised the control group (ten of them with Barrett's esophagus). Symptoms were evaluated by a structured clinical questionnaire. Objective assessment was performed by ambulatory 24-h esophageal pH monitoring and endoscopy. Esophageal sensitivity to acid was evaluated by esophageal perfusion of ClH 0.1 N. Fifteen (37%) of the 40 patients with achalasia presented heartburn, but only four of them had esophagitis and/or abnormal esophageal pH recording. Eight patients had abnormal pH recording. Three patients had esophagitis. The esophagus was sensitive to acid in seven (17%) patients with achalasia, three of them with heartburn and one with abnormal pH recording. In the control group, 40 of 43 (93%) presented heartburn. Acid perfusion was positive in 32 (74%). Sensitivity to acid was lower in patients with achalasia than in those with gastroesophageal reflux disease with or without Barrett's esophagus. The prevalence of heartburn in patients with achalasia is high, although its association with objective indicators of gastroesophageal reflux disease is weak. Patients with achalasia have lower esophageal sensitivity to acid than patients with GERD, suggesting that heartburn is does not arise from this condition.

Barrett's esophagus in untreated achalasia: ‘guess who's coming to dinner’ first

Barrett's esophagus in untreated achalasia: ‘guess who's coming to dinner’ first

Clinical, Radiological, and Manometric Profile in 145 Patients with Untreated Achalasia

Esophageal achalasia is characterized by the absence of esophageal peristalsis and by a dysfunctional lower esophageal sphincter (LES). Descriptions of clinical, radiological, and manometric findings in patients with achalasia usually have been based on small numbers of patients. This study was designed to determine in patients with untreated achalasia: (1) clinical presentation; (2) how often a diagnosis of gastroesophageal reflux disease (GERD) was erroneously made based on the presence of heartburn; (3) manometric profile; (4) relationship between chest pain and the manometric finding of vigorous achalasia. Between 1990 and 2004, a diagnosis of esophageal achalasia was established in 145 patients. None of them had been previously treated (no previous endoscopic or surgical treatment). We evaluated the demographic and clinical characteristics, as well as the results of the endoscopy, barium swallow, esophageal manometry, and ambulatory pH monitoring. We also compared the clinical and the manometric profile of patients with classic and patients with vigorous achalasia. Most patients with untreated achalasia had dysphagia (94%). Regurgitation was present in 76% and heartburn in 52%. Chest pain (41%) and weight loss (35%) were less common. Acid-suppressing medications had been prescribed to 65 patients (45%) who complained of heartburn on the assumption that GERD was present. The LES was hypertensive in 43% of patients only. There was no significant difference in the prevalence, severity, and duration of chest pain in patients with classic and with vigorous achalasia. These results show that in patients with untreated achalasia: (1) dysphagia was the most frequent complaint, but regurgitation and heartburn were frequently present; (2) a diagnosis of GERD based on the presence of heartburn was highly unreliable; (3) the LES was hypertensive in less than half of patients; and (4) the prevalence, severity, and duration of chest pain did not correlate with the manometric finding of vigorous achalasia.

Achalasia: a rare cause of stridor

Case reportBrian Suffoletto, Kenneth KatzA 56-year-old woman presented to the emergency depart-ment (ED) acutely stridorous. The patient was leaningforward, unable to speak and could not swallow. Thepatient was, however, able to communicate through ges-turing and writing. She confirmed a prior history ofuntreated achalasia, and had eaten a large meal one hourprior to ED presentation.Physical examination (PE) revealed a woman in obviousrespiratory distress. Vital signs included: temperature36.4 C, pulse 92 beats per minute, blood pressure 109/56 mmHg, respiratory rate 30 breaths per minute and aroom air oxygen saturation of 100%. The head, ears, eyes,nose and throat examinations were unremarkable. The neckexamination revealed an audible inspiratory stridor. Pal-pation of the neck did not identify a mass or thyromegaly,however the patient did have tenderness in the anteriorneck. The remainder of the PE was unremarkable.Radiographs were obtained. A cardiothoracic surgeryconsultation was obtained. The insertion of a nasogastrictube (NGT) was attempted to decompress the esophagealdilatation. The NGT repeatedly coiled and was unable to bepassed. An esophagogastroduodenoscopy (EGD) was thenperformed. The endoscope, however, could not be advanceddue to a large impacted food bolus. After 10 l of salinelavage and forceps removal of food pieces the scope wasadvanced through the lower esophageal sphincter (LES),whichwasnotedtohaveincreasedtone.Thepatient’sstridorresolved, and she remained asymptomatic after the EGD.The patient was discharged to home asymptomatic on hos-pital day two to follow up for outpatient surgical myotomy.Comment: Listening to the stridorCamilla Tozzetti, Pietro Amedeo ModestiStridor is rarely encountered in common clinical practice.Even when matched, of all the diagnosis that ever will bemade, achalasia is the most rare. The patient was luckytwice: she had the opportunity to communicate the priorhistory of untreated achalasia, and she met a listeningphysician. Indeed, stridor was a marker of an urgent situ-ation, but the patient history was the real guide to a correctdiagnostic course.Stridor is a loud musical sound of definite and constantpitch (usually about 400 Hz) that indicates upper airwayobstruction. It is identical to wheezing acoustically in everyway except for two characteristics: (1) stridor is confined toinspiration, whereas wheezing is either confined entirely toexpiration (30–60% of patients), or occurs during bothexpiration and inspiration (40–70% of patients) [1, 2]; and(2) stridor is always louder over the neck, whereaswheezing is always louder over the chest [2]. In somepatients with upper airway obstruction, stridor does notappear until the patient breathes rapidly through an openmouth. When stridor is present, an airway diameter lessthan 5 mm can be estimated [3].

Return of Esophageal Function after Treatment for Achalasia as Determined by Impedance-Manometry

BackgroundTreatment for Achalasia is aimed at the lower esophageal sphincter (LES), although little is known about the effect, if any, of these treatments on esophageal body function (peristalsis and clearance). We sought to measure the effect of various treatments using combined manometry (peristalsis) with Multichannel Intraluminal Impedance (MII) (esophageal clearance). MethodsWe enrolled 56 patients with Achalasia referred to the University of Washington Swallowing Center between January 2003 and January 2006. Each was grouped according to prior treatment: 38 were untreated (untreated achalasia), 10 had undergone botox injection or balloon dilation (endoscopic treatment), and 16 a laparoscopic Heller myotomy. The preoperative studies for 8 of the myotomy patients were included in the untreated achalasia group. Each patient completed a dysphagia severity questionnaire (scale 0–10). Peristalsis was analyzed by manometry and esophageal clearance of liquid and viscous material by MII. ResultsMean dysphagia severity scores were significantly better in patients after Heller Myotomy than in either of the other groups (2.0 vs. 5.3 in the endoscopic group and 6.5 in untreated achalasia, p < 0.05). Peristaltic contractions were observed in 63% of patients in the Heller myotomy group, compared with 40% in the endoscopic group and 8% in untreated achalasia (p < 0.05 for both treatment groups vs. untreated achalasia). Liquid clearance rates were significantly better in both treatment groups: 28% in Heller myotomy and 16% in endoscopic treatment compared to only 5% in untreated achalasia (p < 0.05). Similarly, viscous clearance rates were 19% in Heller myotomy and 11% in endoscopic treatment, vs. 2% in untreated achalasia (p < 0.05). In the subset of patients who underwent manometry/MII both pre- and postoperatively, peristalsis was observed more frequently postoperatively than in preop studies (63% of patients exhibiting peristalsis vs. 12%), as was complete clearance of liquid (35% of swallows vs. 14%) and viscous boluses (22% of swallows vs. 14%). These differences were not significant, however. In the patients who had a myotomy the return of peristalsis correlates with effective esophageal clearance (liquid bolus: r = 0.46, p = 0.09 and viscous bolus: r = 0.63, p < 0.05). There is no correlation between peristalsis and bolus clearance in the endoscopic treatment group. ConclusionsWith treatment Achalasia patients exhibit some restoration in peristalsis as well as improved bolus clearance. After Heller Myotomy, the return of peristalsis correlates with esophageal clearance, which may partly explain its superior relief of dysphagia.

Acute airway obstruction as a complication of untreated achalasia

Acute airway obstruction as a complication of untreated achalasia

Acute Airway Obstruction Due to Untreated Achalasia

Acute Airway Obstruction Due to Untreated Achalasia

Percutaneous Endoscopic Gastrostomy as a Tool to Assist Pneumatic Dilation in a Difficult Case of Sigmoid Esophagus

We describe a case of a long-standing, untreated achalasia with a huge sigmoid esophagus in a 58-year-old Caucasian man who declined surgery. All means of classical endoscopic approach for pneumatic dilation, including the use of an overtube, were impossible because any attempt to propel the balloon dilator made the guide wire pull back out of the stomach because of the large loops and the tortuosity of the esophagus. For this reason, we used, for the first time, a combined approach of percutaneous gastrostomy and endoscopy in order to fix the guide wire at two points, achieving a pneumatic dilation in this way. A few months later, a significant improvement in the symptoms and nutritional status of the patient were observed.

Meal area index: a new technique for quantitative assessment in achalasia by ambulatory manometry during eating.

Ambulatory non-perfused oesophageal manometry was used to study oesophageal body function during consumption of a full meal in patients with achalasia. A measure of oesophageal body activity (the meal area index) was developed by calculating the total area under the pressure curve during eating, above the preprandial baseline oesophageal pressure, per meal minute. Untreated patients with achalasia (n = 13) were compared with normal subjects (n = 42), patients with benign stricture (n = 9) and patients with achalasia who had undergone Heller's myotomy (n = 17). The results showed a high meal area index in achalasia, due to a rise in baseline oesophageal pressure and frequent high-amplitude contractions during eating. This was not seen in normal subjects or patients with stricture. The high meal area index was abolished by successful Heller's myotomy but remained in two patients with persisting dysphagia. Sustained high intraoesophageal pressure is generated during consumption of a solid meal in untreated achalasia, resulting in a unique manometric profile. Manometry during eating using the meal area index permits quantitative assessment of oesophageal body function in achalasia and may aid in the assessment of response to treatment.

Preoperative lower esophageal sphincter pressure has little influence on the outcome of laparoscopic Heller myotomy for achalasia.

It is believed that in untreated patients with achalasia, the lower esophageal sphincter (LES) is almost always hypertensive and a Heller myotomy resolves symptoms by decreasing the LES pressure. The incidence of a normal or hypotensive LES in untreated achalasia patients is unknown. The goals of this study were to determine the incidence of a normal or hypotensive LES in untreated achalasia patients and the outcome of laparoscopic Heller myotomy in achalasia patients with either normal or low LES pressure. Between January 1990 and May 2002, a diagnosis of achalasia was made in 239 patients. Among 109 patients (46%) who had not previously received any form of treatment, 53 patients underwent laparoscopic Heller myotomy and Dor fundoplication. Based on the preoperative LES pressure (normal, 14-24 mmHg) they were divided into three groups: group A--four patients (7.5%), LES pressure <14 mmHg; group B--18 patients (34%), LES pressure 14-24 mmHg; and group C-31 patients (58.5%), LES pressure >24 mmHg. Among the 109 untreated patients, the LES was hypertensive in 49 patients (45%), normal in 29 patients (27%), and hypotensive in 31 patients (28%). The clinical outcome was good among the three groups of patients who underwent laparoscopic Heller myotomy, with poor outcome in only approximately 10% in each group. These data show that in 55% of untreated. achalasia patients the LES pressure is either normal or low, and that laparoscopic Heller myotomy usually relieves symptoms regardless of preoperative LES pressure.

Long-term follow-up of achalasia patients treated with botulinum toxin

Aims. To evaluate long-term efficacy of intrasphincteric injection of botulinum toxin in untreated achalasia patients; to analyse whether age can be a predictor of response; and to verify whether any objective measurements correlate with symptom relief. Materials and Methods. A total of 37 patients (mean age 61.4 ± 17.5 years) were enrolled, all of whom injected endoscopically with 100 U of botulinum toxin. Symptom score, oesophageal manometry and oesophageal radionuclide emptying were assessed prior to treatment and 4 weeks, 3 months and 1 year after botulinum toxin. In the case of failure or relapse (symptom score >2), treatment was repeated. Results. All but 6 patients (83.7%) were in clinical remission one month after botulinum toxin. At 12 months, mean symptom score was 0.9 ± 0.5 (p<0.05 vs basal); mean lower oesophageal sphincter pressure was 22.0 ± 6.3 (p<0.05 vs basal), and 10-min radionuclide retention was 14.0% ± 7.2 (p<0.05 vs basal). Of the 35 patients followed, 12 (34.3%) had a relapse and were re-treated; 4 out of 12 did not respond after re-treatment. Efficacy of first injection of botulinum toxin lasted for a mean period of 15. 6 months (range 2–30). Up to day 31 (83.7%) patients were still in remission. We observed a trend towards a better response to botulinum toxin treatment in patients over 50 years (p=0.053). Moreover, no correlation was found between any objective achalasia measurements and symptom relief (r coefficient between 0.1 and 0.5) Conclusions. Results show that: 1) one or two intrasphincteric injections of botulinum toxin result in clinical and objective improvement in about 84% of achalasia patients and are not associated with serious side-effects; 2) patients over 50 years showed better benefit than younger patients; 3) no correlation was found between any objective measurements and symptom relief.

Vector volume analysis of the lower esophageal sphincter in achalasia and the effect of balloon dilation.

Assessment of the effect of balloon dilation of the lower esophageal sphincter in achalasia currently relies on symptom scores, radiologic studies, or manometry. We have used vector volume analysis which constructs a three-dimensional (3D) pressure profile to examine the effects of balloon dilation on the sphincter. Studies were performed in 26 normal subjects and in 11 patients with achalasia in whom vector volume was determined before and/or after balloon dilation. Vector volume analysis was performed using a multilumen manometry catheter with eight side-reading ports. The catheter was withdrawn through the sphincter using a continuous pull-through technique. A 3D pressure profile was constructed. Vector volume of the lower esophageal sphincter shows a marked radial and linear asymmetry in normal subjects. 3D pressure profiles showed a symmetrical increase in lower esophageal sphincter pressure in untreated achalasia. Mean vector volumes pre and postdilation fell from 254 to 88 cm3 respectively. This was associated with a fall in the mean radial symmetry index from 0.83 to 0.76. Improvement in dysphagia occurred in those patients in whom pressure fell to 15 mmHg or below in at least three adjacent segments. This technique may be a useful tool in the assessment of patients with achalasia and postdilation results may help to predict the long-term outcome of treatment.

Cardiovascular autonomic function in patients with primary achalasia.

Impaired gastrointestinal function outside the esophagus has been found in achalasic patients. Moreover, achalasia may occur in diseases in which a systemic dysautonomia is evident. These findings raise the question of whether a generalized subclinical alteration of autonomic control is also present in primary achalasia. Cardiovascular reflex tests and power spectral analysis of heart rate variability were studied in patients with primary achalasia to establish whether autonomic nervous system changes are present in districts other than the gastrointestinal tract. Nineteen normotensive patients with untreated primary achalasia and with no history of cardiac, renal, or endocrinological diseases were examined. Cardiovascular reflex tests included: the tilt test (10 min at 65 degrees), Valsalva maneuver (40 mm Hg for 15 sec), deep breathing (6 breaths/min), and sustained handgrip (30% of maximal effort for 5 min). The parameters evaluated were systolic and diastolic blood pressure (continuously recorded), ECG, oronasal and thoracic respiration, tachogram, and plethysmogram. To evaluate the balance between parasympathetic and sympathetic functions, power spectral analysis of the heart rate variability was carried out. Each patient was paired with two sex- and age-matched healthy controls. In achalasic patients the head-up tilt test, Valsalva maneuver, deep breathing test, and sustained handgrip did not show significant differences from the control group. Low-frequency (LF) and high-frequency (HF) spectral powers and the ratio of LF to HF did not differ in both groups. This study failed to disclose impaired cardiovascular autonomic control in achalasic patients. We suggest that in primary achalasia the defect is limited to the gastrointestinal tract.

Oesophageal tone in patients with achalasia

Background—The diagnosis and classification of oesophageal motility disorders is currently based on assessment of the phasic contractile activity of the oesophagus. Tonic muscular contraction of the oesophageal body (oesophageal tone)...

Effect of pneumatic dilation on gastroesophageal reflux in achalasia.

The aims of this study were to assess the effect of pneumatic dilation on gastroesophageal reflux in achalasia, differentiate esophageal acid due to lactate from acid due to gastroesophageal reflux, and determine if chest pain and heartburn are reliable indicators of gastroesophageal reflux. Eight untreated achalasia patients underwent pre- and postdilation esophageal fluid/food residue lactate and pH analysis, esophageal manometry, 24-hr pH monitoring, and symptom assessment. All patients had a successful clinical outcome and a decrease in lower esophageal sphincter pressure from 29.1 +/- 12.7 to 14.7 +/- 3.8 mm Hg (mean +/- SD; P = 0.04). Abnormal acid exposure was present in two patients before and two patients after dilation. Postdilation acid exposure was mild. Lactate was detected before dilation in all patients. A lactate concentration >2 mmol/liter was associated with acidic residue and one abnormal 24-hr pH profile. There was no correlation between an abnormal 24-hr pH test and age, lower esophageal sphincter pressure, or duration of symptoms prior to treatment. Chest pain and heartburn were unrelated to drops in pH. Gastroesophageal reflux is rare in untreated achalasia and esophageal acidity may result from ingestion of acidic foods or production of lactate. Mild gastroesophageal reflux occurs after dilation but is of no clinical significance. Chest pain and heartburn are not indicators of acid reflux in achalasia.

Five year prospective study of the incidence, clinical features, and diagnosis of achalasia in Edinburgh.

With the increasing availability of manometry, patients with achalasia are often referred at an early stage when they lack the classic features of established disease. A prospective five year study of the presenting features of untreated achalasia referred to our department was undertaken. Twenty men and 18 women presented throughout adult life, with a mean age at the time of diagnosis of 44 years (range 17 to 76 years). The presenting symptoms were dysphagia: for solids (100%) and for liquids (97%), chest pain (74%), and weight loss (60%). Endoscopy was reported as normal in 15 patients and achalasia was suggested in only 21 of 33 barium examinations. Fourteen had been treated for gastrooesophageal reflux but none had been misdiagnosed as having cardiac or psychiatric disease. The annual incidence of achalasia in the Lothian region is 0.8/100,000 of population. Persistent dysphagia is the cardinal symptom of achalasia which presents throughout adult life. Nevertheless, recent onset achalasia is often misdiagnosed as gastrooesophageal reflux disease. Because endoscopy is frequently normal and the diagnosis is often not made by radiology, manometric investigation is necessary if the condition is to be recognised and treated at an early stage.