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Esophageal Spasm

Diffuse Esophageal Spasm

A spasm is a sudden, involuntary contraction of a muscle. Esophageal spasms present as non-cardiac recurring chest pain and may be very problematic. Many patients who present to hospital, will have cardiac chest pain ruled out first, then most of the time investigations are stopped and they will be discharged with no follow-up plan [1].


Presentation

  • Esophageal spasms present with non-cardia chest pain, which is located retrosternally and radiates to the back. Patients also complain of dysphagia which is characterized by difficulty swallowing several seconds after initiating a swallow, a sensation of food getting stuck and regurgitation. There is also a globus sensation, and many patients suffer from heartburn.
  • The symptoms are intermittent and vary in frequency and intensity. Pain may be triggered by particular food and drinks.
  • In the hypertensive variants, pain is the predominant symptom, with mild dysphagia [8].
Weight Gain
  • The need for surgery was obviated by the successful application of relaxation training, which resulted in a significant reduction in painful esophageal spasms, weight gain, and an improvement in psychological state; there was, however, no change in the[ncbi.nlm.nih.gov]
Non-Cardiac Chest Pain
  • DOS is an unusual cause of non-cardiac chest pain (2%) or dysphagia (4%). It occurs most commonly in patients 50 years old but can occur at any age.[radiopaedia.org]
  • Theophylline - improved non-cardiac chest pain in one trial, and may relax the oesophageal wall.[patient.info]
  • Diagnostic yield of 24-hour esophageal manometry in non-cardiac chest pain. Neurogastroenterol Motil . 2016 Aug. 28(8):1186-93. [Medline] . Carlson DA, Pandolfino JE.[emedicine.medscape.com]
Intermittent Dysphagia
  • Four of five patients had episodic nonexertional midchest pain; two patients experienced intermittent dysphagia.[ncbi.nlm.nih.gov]
  • We present a 43-year-old female with paroxysmal chest and abdominal pain, nausea, salivation, and intermittent dysphagia. Esophageal testing demonstrated diffuse esophageal spasm, but smooth muscle relaxants provided no relief.[ncbi.nlm.nih.gov]
  • The usual presentation is intermittent dysphagia with occasional chest pain.[emedicine.medscape.com]
Intermittent Dysphagia
  • Four of five patients had episodic nonexertional midchest pain; two patients experienced intermittent dysphagia.[ncbi.nlm.nih.gov]
  • We present a 43-year-old female with paroxysmal chest and abdominal pain, nausea, salivation, and intermittent dysphagia. Esophageal testing demonstrated diffuse esophageal spasm, but smooth muscle relaxants provided no relief.[ncbi.nlm.nih.gov]
  • The usual presentation is intermittent dysphagia with occasional chest pain.[emedicine.medscape.com]
Deglutition Disorder
  • Keywords Spasm Dysphagia High-resolution manometry Impedance Deglutition Deglutition disorders Abbreviations CDP Contractile deceleration point CFV Contractile front velocity DES Distal esophageal spasm DL Distal latency EPT Esophageal pressure topography[link.springer.com]
Odynophagia
  • Diffuse esophageal spasm typically causes substernal chest pain with nonprogressive dysphagia and odynophagia for both liquids and solids.[ncbi.nlm.nih.gov]
Solid Dysphagia
  • Diffuse esophageal spasm typically causes substernal chest pain with nonprogressive dysphagia and odynophagia for both liquids and solids.[ncbi.nlm.nih.gov]
Chest Pain
  • Of the patients with DES, 159 (73.3%) had dysphagia or chest pain as a reported symptom.[ncbi.nlm.nih.gov]
  • Diffuse esophageal spasm typically causes substernal chest pain with nonprogressive dysphagia and odynophagia for both liquids and solids.[ncbi.nlm.nih.gov]
  • Abstract A patient with dysphagia and chest pain was shown by manometry to have high-amplitude peristaltic esophageal contractions (nutcracker esophagus).[ncbi.nlm.nih.gov]
  • Esophagomyotomy is recommended for selected patients with clinically significant chest pain and/or dysphagia.[ncbi.nlm.nih.gov]
  • In this study, a cohort of 358 subjects was evaluated in a gastrointestinal motility laboratory for dysphagia and/or chest pain. Applying the recently proposed criteria of Richter and Castell, 18 subjects (5%) were diagnosed as having DES.[ncbi.nlm.nih.gov]
Retrosternal Chest Pain
  • Abstract The hypertensive peristaltic (nutcracker) esophagus represents a motility disorder characterized clinically by squeezing retrosternal chest pain and manometrically by high amplitude esophageal peristaltic contractions.[ncbi.nlm.nih.gov]
Flushing
  • Interestingly, no adverse side effects such as headache or flush were observed at any dose of GTN. The blood pressure did not show any changes during the studies in any of the five patients.[ncbi.nlm.nih.gov]
Hyperactivity
  • CONCLUSION: Performing POEM for DES is challenging due to reactive hyperactive spastic contractions during the procedure causing technical difficulties and an extended procedure.[ncbi.nlm.nih.gov]

Workup

There is no laboratory marker that can aid in the diagnosis of these conditions. Since these patients have dysphagia an endoscopy will be required to rule out structural problems. If the symptoms are suggestive of gastroesophageal reflux, empirical treatment may be started. If there is no relief then, the below tests should be done [8]. 

Imaging

The modality of choice is barium swallow and esophageal manometry. A barium swallow will show esophageal spasm as of multiple contractions that appear at the same time causing a corkscrew like appearance. Manometry is the best modality to diagnose esophageal spasm. The variants have characteristic findings. Nutcracker esophagus is characterized by coordinated contractions in the smooth muscle of the esophagus with an excessive amplitude and/or duration. Diffuse esophageal spasm, will have is ≥20 percent premature contractions on esophageal pressure topography.

Esophageal Motility Disorder
  • Complications of botulinum toxin injections for treatment of esophageal motility disorders†. Dis Esophagus . 2017 Feb 1. 30(3):1-5. [Medline] .[emedicine.medscape.com]
  • Several reports have suggested that esophageal motility disorders may progress from one type to another.[ncbi.nlm.nih.gov]
  • Abstract The nutcracker esophagus is a newly defined subset of primary esophageal motility disorders that can be responsible for dysphagia and/or chest pain.[ncbi.nlm.nih.gov]
  • Abstract Distal esophageal spasm (DES) is an esophageal motility disorder that presents clinically with chest pain and/or dysphagia and is defined manometrically as simultaneous contractions in the distal (smooth muscle) esophagus in 20% of wet swallows[ncbi.nlm.nih.gov]
  • The evolution of this patient's disease provides evidence that diffuse spasm and achalasia are different stages of the same disease and lie at opposite ends of a spectrum of related esophageal motility disorders.[ncbi.nlm.nih.gov]
Tertiary Contractions of the Esophagus
  • contractions of the esophagus, corkscrew esophagus is associated with diffuse esophageal spasm Diffuse esophageal spasm (DES) may be present, however, without the appearance of a corkscrew esophagus Usually occurs over the age of 50 Normal esophageal[learningradiology.com]

Treatment

  • There is no totally effective treatment for the hypertensive variants. Initially calcium channel blockers such as diltiazem may be used. Another option are tricyclic antidepressants and imipramine, which have been shown to be effective in small randomized studies.
  • If the initial therapies are not successful, few treatment options remain, including botulinum toxin or a nitric oxide contributing drugs (e.g. isosorbide) or sildenafil.
  • For extreme cases myotomy is done to relive the pain. If all fails then a last resort is esophagectomy [9] [10] [11].

Prognosis

Mortality is rare but the morbidity is significant and will affect the quality of life with possible psychological problems developing, especially if the diagnosis is not made. Patients are frequent visitors to the emergency room and often get discharged once cardiac cases are ruled out, leaving them confused with little relief from their pain. These patients are at increased risk of achalasia [7].

Etiology

The true etiology of the condition and its variants is unknown. There have been hypothesis of nerve disorders and gastroesophageal reflux being a cause. The cause of hypertensive spasms is also unknown, but neuronal disorders appear to be there.

Epidemiology

There is very little data outside the United States of America, where the incidence is believed to be 1 case in every 100,000 population year. Mortality is rare, but the quality of life may be significantly reduced if the condition is not diagnosed or treated well. It is more common in Caucasians and women [3]. Some of these esophageal motility disorders have been associated with psychiatric conditions.

Sex distribution
Age distribution

Pathophysiology

The pathophysiology of the condition and its variants is not completely understood, but it is thought that there is a problem with the innervation of the muscles. Esophageal spasm may occur due to a deficiency of nitric oxide synthesis and degradation. This hypothesis was formed because these spams respond to glycerine trinitrate.
The hypertensive variants are thought to be caused by due to overactivity of excitatory innervation or smooth muscle response to excitatory nerves [4] [5] [6].

Prevention

The true etiology and pathophysiology are not known and prevention is difficult to implement. The spasms may be triggered by certain food and drinks, these can be avoided to reduce the attacks. Use of pureed food has been shown to reduce the frequency of the spasms.

Summary

Esophageal spasms can be classified into two different types, diffuse esophageal spasm, with the second form having hypertensive peristalsis.

  • Diffuse esophageal spasms has uncoordinated normal amplitude contractions with rapid propagation.
  • Hypertensive peristalsis is better known as the nutcracker esophagus and is characterised by coordinated contractions with a high amplitude and pressure. There is another variant called the jackhammer esophagus that is a more severe variant with longer a prolonged contraction and is more diffuse [2]. 

Patient Information

  • Definition: Esophageal spasm is a distressing condition where food pipe which carries food from the mouth to the stomach, called the esophagus has involuntary contraction with uncoordinated movement of substances. There are subsets of this condition, e.g. the nutcracker esophagus where the muscles are coordinated, but they contract and squeeze the food pipe tight, causing symptoms.
  • Cause: The cause is not known but research is ongoing to try and find out. It is thought in some cases acid from the stomach coming back up the food pipe could be cause.
  • Symptoms: Esophageal spasms present with central chest pain. Patients also complain of difficulty in swallowing and regurgitation of food. There is also a sensation of a lump in the throat, and many patients have heartburn. The symptoms are intermittent and vary in frequency and intensity. Pain may be triggered by certain types of food. In the hypertensive variants, pain is the predominant symptom, with mild difficulty in swallowing.
  • Diagnosis: The diagnosis is made by seeing how the food pipe contracts. This can be done by doing a barium swallow (you are made to swallow a contrast medium while X-rays are being taken). The other test is done by placing a small pipe in the food pipe and measuring the pressures along the pipe while you swallow. The doctor may also order an endoscopy to see the food pipe to make sure there I nothing blocking it and no acid is come out backward into the food pipe. 
  • Treatment: There are tablets that are used to help treat the condition. If that doesn’t work then injection with botulism toxin (Botox) is used to reduce the spasm. The patients should avoid foods and drinks that cause or worsen the spasms.

References

Article

  1. Pope JH, Aufderheide TP, Ruthazer R, et al. Missed diagnoses of acute cardiac ischemia in the emergency department. N Engl J Med 2000; 342:1163.
  2. Floch M, et al. Esophageal Motility Disorders. In: Netter's Gastroenterology. 2nd ed. Philadelphia, Pa: Saunders; 2010:Chapter 14.
  3. Katz PO, Dalton CB, Richter JE, et al. Esophageal testing of patients with noncardiac chest pain or dysphagia. Results of three years' experience with 1161 patients. Ann Intern Med 1987; 106:593.
  4. Konturek JW, Gillessen A, Domschke W. Diffuse esophageal spasm: a malfunction that involves nitric oxide? Scand J Gastroenterol 1995; 30:1041.
  5. Jung HY, Puckett JL, Bhalla V, et al. Asynchrony between the circular and the longitudinal muscle contraction in patients with nutcracker esophagus. Gastroenterology 2005; 128:1179.
  6. Mujica VR, Mudipalli RS, Rao SS. Pathophysiology of chest pain in patients with nutcracker esophagus. Am J Gastroenterol 2001; 96:1371.
  7. Khatami SS, Khandwala F, Shay SS, Vaezi MF. Does diffuse esophageal spasm progress to achalasia? A prospective cohort study. Dig Dis Sci 2005; 50:1605.
  8. Song CW, Lee SJ, Jeen YT, et al. Inconsistent association of esophageal symptoms, psychometric abnormalities and dysmotility. Am J Gastroenterol 2001; 96:2312.
  9. Cattau EL Jr, Castell DO, Johnson DA, et al. Diltiazem therapy for symptoms associated with nutcracker esophagus. Am J Gastroenterol 1991; 86:272.
  10. Miller LS, Parkman HP, Schiano TD, Cassidy MJ, Ter RB, Dabezies MA, et al. Treatment of symptomatic nonachalasia esophageal motor disorders with botulinum toxin injection at the lower esophageal sphincter. Dig Dis Sci. Oct 1996;41(10):2025-31
  11. Salvador R, Costantini M, Rizzetto C, Zaninotto G. Diffuse esophageal spasm: the surgical approach. Dis Esophagus. Feb 10 2011

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Last updated: 2018-06-22 08:45