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ENeurologicalSci Jun 2024A 74-year-old man developed orthostatic syncope, a feeling of food stuck in his chest, and postprandial vomiting 3 years before presentation. Examination revealed...
A 74-year-old man developed orthostatic syncope, a feeling of food stuck in his chest, and postprandial vomiting 3 years before presentation. Examination revealed severe orthostatic hypotension and cerebellar ataxia, and he was diagnosed with multiple system atrophy (MSA) with predominant cerebellar ataxia. Videofluoroscopic examination of swallowing showed lower oesophageal stricture and barium stagnation within the oesophagus. Oesophagogastroduodenoscopy revealed hypercontraction of the lower oesophagus, and high-resolution oesophageal manometry showed premature contractions of the lower oesophagus and decreased oesophageal peristalsis. The median integrated relaxation pressure in the lower oesophageal sphincter was normal, and achalasia was therefore excluded. Based on the Chicago classification version 4.0, his oesophageal dysmotility was classified as distal oesophageal spasm (DES). The stuck feeling in his chest and vomiting improved following endoscopic balloon dilation. This case suggests that DES can cause oesophageal food stagnation and postprandial vomiting in patients with MSA.
PubMed: 38655009
DOI: 10.1016/j.ensci.2024.100500 -
Cureus Jan 2024Although phrenic nerve and esophageal injury are commonly known risks associated with cryoablation, there is limited literature regarding coronary artery spasm (CAS), a...
Although phrenic nerve and esophageal injury are commonly known risks associated with cryoablation, there is limited literature regarding coronary artery spasm (CAS), a serious and potentially fatal complication of cryoablation. We report the case of a 68-year-old Caucasian female who developed a left main CAS with a significant hemodynamic compromise during cryoablation. The patient, with a history of hyperlipidemia, hypertension, and symptomatic persistent atrial fibrillation, was admitted for elective catheter ablation for atrial fibrillation. During the ablation of the left superior pulmonary vein (LSPV), the patient developed severe hypotension and bradycardia. The patient's monitor revealed ST elevation, confirmed by a 12-lead ECG. Immediate coronary angiography revealed the left main coronary spasm, which improved with nitroglycerine administration with resolution of ST elevation and return of the patient's hemodynamics to stability. The patient's left main CAS was induced by cryoablation of LSPV. Literature on atrial fibrillation ablation-induced CAS is scant, but a Japanese study has shown that it occurs more commonly in cryoablation than in radiofrequency, hot balloon, or laser ablation. The study showed LSPV as the most common site of ablation right before the spasms happened. Further studies about this topic are needed to delineate further the risk factors and the precautions that could prevent CAS. In the meantime, prompt recognition and appropriate intervention are critical for a good patient outcome.
PubMed: 38333489
DOI: 10.7759/cureus.51902 -
American Journal of Physiology.... Apr 2024Major esophageal disorders involve obstructive transport of bolus to the stomach, causing symptoms of dysphagia and impaired clearing of the refluxed gastric contents.... (Review)
Review
Major esophageal disorders involve obstructive transport of bolus to the stomach, causing symptoms of dysphagia and impaired clearing of the refluxed gastric contents. These may occur due to mechanical constriction of the esophageal lumen or loss of relaxation associated with deglutitive inhibition, as in achalasia-like disorders. Recently, immune inflammation has been identified as an important cause of esophageal strictures and the loss of inhibitory neurotransmission. These disorders are also associated with smooth muscle hypertrophy and hypercontractility, whose cause is unknown. This review investigated immune inflammation in the causation of smooth muscle changes in obstructive esophageal bolus transport. Findings suggest that smooth muscle hypertrophy occurs above the obstruction and is due to mechanical stress on the smooth muscles. The mechanostressed smooth muscles release cytokines and other molecules that may recruit and microlocalize mast cells to smooth muscle bundles, so that their products may have a close bidirectional effect on each other. Acting in a paracrine fashion, the inflammatory cytokines induce genetic and epigenetic changes in the smooth muscles, leading to smooth muscle hypercontractility, hypertrophy, and impaired relaxation. These changes may worsen difficulty in the esophageal transport. Immune processes differ in the first phase of obstructive bolus transport, and the second phase of muscle hypertrophy and hypercontractility. Moreover, changes in the type of mechanical stress may change immune response and effect on smooth muscles. Understanding immune signaling in causes of obstructive bolus transport, type of mechanical stress, and associated smooth muscle changes may help pathophysiology-based prevention and targeted treatment of esophageal motility disorders. Esophageal disorders such as esophageal stricture or achalasia, and diffuse esophageal spasm are associated with smooth muscle hypertrophy and hypercontractility, above the obstruction, yet the cause of such changes is unknown. This review suggests that smooth muscle obstructive disorders may cause mechanical stress on smooth muscle, which then secretes chemicals that recruit, microlocalize, and activate mast cells to initiate immune inflammation, producing functional and structural changes in smooth muscles. Understanding the immune signaling in these changes may help pathophysiology-based prevention and targeted treatment of esophageal motility disorders.
Topics: Humans; Esophageal Achalasia; Mast Cells; Manometry; Esophageal Motility Disorders; Muscle, Smooth; Inflammation; Cytokines; Hypertrophy
PubMed: 38290993
DOI: 10.1152/ajpgi.00258.2023 -
Diseases of the Esophagus : Official... Apr 2024Achalasia is a rare esophageal disorder characterized by abnormal esophageal motility and swallowing difficulties. Pain and/or spasms often persist or recur despite...
Achalasia is a rare esophageal disorder characterized by abnormal esophageal motility and swallowing difficulties. Pain and/or spasms often persist or recur despite effective relief of the obstruction. A survey by UK charity 'Achalasia Action' highlighted treatments for achalasia pain/spasms as a key research priority. In this patient-requested systematic review, we assessed the existing literature on pharmacological therapies for painful achalasia. A systematic review of the literature using Medline, Embase and Cochrane databases was performed to identify studies evaluating pharmacological therapies for achalasia. Methodological quality of included randomized controlled trials was assessed using the Cochrane Risk of Bias tool. In total, 70% (40/57) of survey respondents reported experiencing pain/spasms. A range of management strategies were reported. Thirteen studies were included in the review. Seven were randomized controlled trials. Most studies were >30 years old, had limited follow-up, and focussed on esophageal manometry as the key endpoint. Generally, studies found improvements in lower esophageal pressures with medications. Only one study evaluated pain/spasm specifically, precluding meta-analysis. Overall risk of bias was high. The achalasia patient survey identified that pain/spasms are common and difficult to treat. This patient-requested review identified a gap in the literature regarding pharmacological treatments for these symptoms. We provide an algorithm for investigating achalasia-related pain/spasms. Calcium channel blockers or nitrates may be helpful when esophageal obstruction and reflux have been excluded. We advocate for registry-based clinical trials to expand the evidence base for these patients.
Topics: Esophageal Achalasia; Humans; Female; Manometry; Male; Pain; Adult; Randomized Controlled Trials as Topic; Middle Aged; Pain Management; Aged
PubMed: 38282166
DOI: 10.1093/dote/doae005 -
Endoscopy Jun 2024Non-achalasia esophageal motility disorders (NAEMDs), encompassing distal esophageal spasm (DES) and hypercontractile esophagus (HCE), are rare conditions. Peroral... (Observational Study)
Observational Study
BACKGROUND
Non-achalasia esophageal motility disorders (NAEMDs), encompassing distal esophageal spasm (DES) and hypercontractile esophagus (HCE), are rare conditions. Peroral endoscopic myotomy (POEM) is a promising treatment option. In NAEMDs, unlike with achalasia, the lower esophageal sphincter (LES) functions normally, suggesting the potential of LES preservation during POEM.
METHODS
This retrospective two-center observational study focused on patients undergoing LES-preserving POEM (LES-POEM) for NAEMD. Eckardt scores were assessed pre-POEM and at 6, 12, and 24 months post-POEM, with follow-up endoscopy at 6 months to evaluate for reflux esophagitis. Clinical success, defined as an Eckardt score ≤3, served as the primary outcome.
RESULTS
227 patients were recruited over 84 months until May 2021. Of these, 16 underwent LES-POEM for an NAEMD (9 with HCE and 7 with DES). The median pre-POEM Eckardt score was 6.0 (interquartile range [IQR] 5.0-7.0), which decreased to 1.0 (IQR 0.0-1.8; <0.001) 6 months post-POEM. This was sustained at 24 months, with an Eckardt score of 1.0 (IQR 0.0-1.8; P<0.001). Two patients (12.5%) developed Los Angeles grade A or B esophagitis.
CONCLUSIONS
LES-POEM for NAEMD demonstrates favorable clinical outcomes, with infrequent esophagitis and reintervention for LES dysfunction rarely required.
Topics: Humans; Male; Female; Middle Aged; Retrospective Studies; Esophageal Sphincter, Lower; Aged; Esophageal Motility Disorders; Myotomy; Treatment Outcome; Adult; Natural Orifice Endoscopic Surgery; Esophagoscopy
PubMed: 38183976
DOI: 10.1055/a-2239-7371 -
Medicine Dec 2023Myotonic dystrophy type 1 (DM-1) is a progressive multisystem genetic disorder that causes myotonia and both distal limb and facial/neck muscle weakness by expanding the...
RATIONALE
Myotonic dystrophy type 1 (DM-1) is a progressive multisystem genetic disorder that causes myotonia and both distal limb and facial/neck muscle weakness by expanding the CTG repeats of the DMPK gene in chromosome 19q13.3. General anesthesia is indicated in DM-1 patients owing to their sensitivity to anesthetic drugs such as opioids, hypnotics, and neuromuscular blocking agents.
PATIENT CONCERNS
A 48-year-old male patient underwent a laparoscopic cholecystectomy for gallstones under general anesthesia. He experienced sudden cardiac arrest and respiratory failure the day after surgery. After a thorough review of past medical history, we recognized that 15 years prior, he had been diagnosed with classic type DM-1, but the diagnosis was not self-reported before general anesthesia. Symptoms of severe dysphagia developed subsequently. In a videofluoroscopic swallowing study (VFSS), we observed abrupt aggravation of myotonic dysphagia after general anesthesia. VFSS revealed cricopharyngeal opening dysfunction, with a remaining large residue in the pyriform sinus, resulting in a severe cricopharyngeal achalasia pattern.
DIAGNOSIS
Acute cricopharyngeal achalasia after general anesthesia.
INTERVENTION AND OUTCOME
The patient underwent a dysphagia rehabilitation program that included cricopharyngeal opening exercises and functional electrical stimulation. However, no significant improvement was observed in the cricopharyngeal achalasia in a 3-month follow-up VFSS.
LESSONS
Low body temperature and anesthetic medications such as opioids and hypnotic agents can induce myotonia in the cricopharyngeal muscle.
Topics: Male; Humans; Middle Aged; Deglutition Disorders; Esophageal Achalasia; Myotonic Dystrophy; Myotonia; Spasm; Anesthesia, General
PubMed: 38050269
DOI: 10.1097/MD.0000000000036378 -
Cureus Jul 2023Distal esophageal spasm is characterized by premature contractions of the distal esophageal smooth muscle leading to non-obstructive dysphagia and non-cardiac chest... (Review)
Review
Distal esophageal spasm is characterized by premature contractions of the distal esophageal smooth muscle leading to non-obstructive dysphagia and non-cardiac chest pain. Diagnosis requires the presence of symptoms along with evidence of at least 20% premature contractions in the setting of a normal lower esophageal sphincter relaxation on high-resolution manometry. New updates to the Chicago Classification have improved the diagnostic accuracy of this method. Functional lumen imaging probe is a growing diagnostic modality that gives a more complete picture of esophageal motility. Pharmacologic treatment remains inadequate. Endoscopic myotomy might be of benefit for non-achalasia esophageal motility disorders. More research is required to better understand the pathophysiology and develop safe and long-lasting management for this disease.
PubMed: 37551217
DOI: 10.7759/cureus.41504 -
Journal of Neurogastroenterology and... Oct 2023Multiple sclerosis (MS) is an inflammatory disease characterized by the demyelination of primarily the central nervous system. Diffuse esophageal spasm (DES) and...
BACKGROUND/AIMS
Multiple sclerosis (MS) is an inflammatory disease characterized by the demyelination of primarily the central nervous system. Diffuse esophageal spasm (DES) and achalasia are both disorders of esophageal peristalsis which cause clinical symptoms of dysphagia. Mechanisms involving dysfunction of the pre- and post-ganglionic nerve fibers of the myenteric plexus have been proposed. We sought to determine whether MS confers an increased risk of developing achalasia or DES.
METHODS
Cohort analysis was done using the Explorys database. Univariate logistic regression was performed to determine the odds MS confers to each motility disorder studied. Comparison of proportions of dysautonomia comorbidities was performed among the cohorts. Patients with a prior diagnosis of diabetes mellitus, chronic Chagas' disease, opioid use, or CREST syndrome were excluded from the study.
RESULTS
Odds of MS patients developing achalasia or DES were (OR, 2.09; 95% CI, 1.73-2.52; < 0.001) and (OR, 3.15; 95% CI, 2.89-3.42; < 0.001), respectively. In the MS/achalasia cohort, 27.27%, 18.18%, 9.09%, and 45.45% patients had urinary incontinence, gastroparesis, impotence, and insomnia, respectively. In the MS/DES cohort, 35.19%, 11.11%, 3.70%, and 55.56% had these symptoms. In MS patients without motility disorders, 12.64%, 0.79%, 2.21%, and 21.85% had these symptoms.
CONCLUSIONS
Patients with MS have higher odds of developing achalasia or DES compared to patients without MS. MS patients with achalasia or DES have higher rates of dysautonomia comorbidities. This suggests that these patients have a more severe disease phenotype in regards to the extent of neuronal degradation and demyelination causing the autonomic dysfunction.
PubMed: 37528077
DOI: 10.5056/jnm22173 -
Revista de Gastroenterologia de Mexico... Jul 2023The solid test meal (STM) is a challenge test that is done during esophageal manometry and appears to increase the diagnostic yield of the study. The aim of our analysis...
INTRODUCTION AND AIMS
The solid test meal (STM) is a challenge test that is done during esophageal manometry and appears to increase the diagnostic yield of the study. The aim of our analysis was to establish the normal values for STM and evaluate its clinical utility in a group of Latin American patients with esophageal disorders versus healthy controls.
MATERIAL AND METHODS
A cross-sectional study was conducted on a group of healthy controls and consecutive patients that underwent high-resolution esophageal manometry, in which STM was done at the final part of the study and consisted of asking the subjects to eat 200 g of precooked rice. The results were compared during the conventional protocol and the STM.
RESULTS
Twenty-five controls and 93 patients were evaluated. The majority of the controls (92%) completed the test in under 8 min. The STM changed the manometric diagnosis in 38% of the cases. The STM diagnosed 21% more major motor disorders than the conventional protocol; it doubled the cases of esophageal spasm and quadrupled the cases of jackhammer esophagus, whereas it demonstrated normal esophageal peristalsis in 43% of the cases with a previous diagnosis of ineffective esophageal motility.
CONCLUSIONS
Our study confirms the fact that complementary STM during esophageal manometry adds information and enables a more physiologic assessment of esophageal motor function to be made, compared with liquid swallows, in patients with esophageal motor disorders.
PubMed: 37419857
DOI: 10.1016/j.rgmxen.2023.05.009