diffuse esophageal spasm

Introduction

Introduction to diffuse esophageal fistula Diffuse esophageal spasm is a disease of esophageal dysmotility, a rare esophageal dyskinesia, which can cause difficulty in swallowing and chest pain. The lesion is mainly located in the lower part of the esophagus, and the normal esophageal propulsive peristalsis disappears. Non-propelled continuous contraction replaces, causing the esophagus to spiral or beaded. basic knowledge The proportion of illness: 0.06% Susceptible people: no special people Mode of infection: non-infectious Complications: pneumonia, angina pectoris, arrhythmia, sinus bradycardia, syncope

Cause

Diffuse esophageal spasm

(1) Causes of the disease

The function of the esophagus is to feed the food into the stomach by means of the esophageal sphincter barrier and the peristaltic contraction of the esophagus itself, and to prevent the gastrointestinal contents from flowing back to the esophagus. The esophageal motor dysfunction may be caused by poor esophageal contraction or abnormal esophageal sphincter function, or two. If the esophageal peristalsis is too weak, the food group cannot be delivered. If the peristalsis is too strong or the peristalsis is prolonged, swallowing pain and dysphagia may occur. The abnormal function of the esophageal sphincter may also produce similar symptoms, that is, if the sphincter relaxes. Poor can interfere with the delivery of the bolus. If the sphincter tension is low, the contraction of the gastroesophageal reflux will occur.

Esophageal dysfunction can be divided into primary and secondary, primary include achalasia, diffuse esophageal fistula, etc.; secondary is caused by systemic diseases, diffuse esophageal sputum It belongs to the primary esophageal motor dysfunction, and the cause of the disease is unknown.

Some authors have suggested that diffuse esophageal fistula is an independent disease, which is the result of secondary neuropathy, the so-called "neuromyopathy theory". The disorder of esophageal motor function is caused by the degeneration of the vagus esophageal branch or fiber break, and the nerve. There is no degeneration in the section. Some authors believe that the cause of diffuse esophageal fistula and achalasia may be the same, because 3% to 5% of diffuse esophageal fistula can progress to achalasia, so it is pointed out that diffuse esophageal fistula may be In a transitional period of achalasia formation, Narducci et al. (1985) reported that the nutcracker esophagus can be converted to diffuse esophageal fistula. Ingelfinge (1967) also reported that patients with diffuse esophageal fistula eventually converted to achalasia. Strong achalasia can be a common manifestation of achalasia and diffuse esophageal fistula. It can be seen that these esophageal dyskinesias are closely related.

Other authors suggest that diffuse esophageal fistula may be a syndrome caused by multiple causes, such as mental factors can lead to the disease (such as a history of traumatic episodes often after emotional agitation, typical patients often have neurotic personality, symptoms may be Related to emotional stress), other factors such as gastroesophageal reflux, esophageal corrosive damage, cold drink cold food and other stimulating esophageal mucosa and sensitivity to gastrin can induce esophageal fistula, and sometimes certain drugs, such as domperidone can also induce Diffuse esophageal spasm.

(two) pathogenesis

The lesion mainly invades the esophagus, and the lower segment is most likely to occur at the distal end of the esophagus from the lower esophageal sphincter 5-10 cm, while the proximal esophagus is basically normal. The esophageal muscle layer includes the longitudinal muscle, and the annular muscle layer and mucosal muscle layer are hypertrophic. Especially, the ring muscle is more obvious, the muscle layer can be as thick as 2cm, some patients can have no esophageal muscular layer hypertrophy, histological examination shows that Auerbach plexus still exists, there is focal chronic inflammatory cell infiltration, electron microscopy shows dominance Esophageal vagus esophageal branching or neurofibrillary rupture, collagen increase and mitochondrial rupture, differ from achalasia in that the number of ganglion cells does not decrease.

Prevention

Diffuse esophageal fistula prevention

Pay attention to avoid emotional excitement, nervous incentives, regulate eating habits, avoid eating too cold, overheating and other irritating foods, chew slowly, develop good eating habits, eat slowly, avoid cold and thick food.

Complication

Diffuse esophageal fistula complications Complications pneumonia angina pectoris arrhythmia sinus bradycardia syncope

Pay attention to diet, regulate mood, and actively treat, generally no special complications.

1. A large amount of food and saliva retained in the esophagus reflux to the mouth, but not acidic, and some can cause aspiration pneumonia.

2. Others Because the esophagus and the heart have common innervation, the esophageal dysfunction can cause changes in the heart. In addition to the aforementioned angina-like chest pain, it can be accompanied by arrhythmia, such as sinus bradycardia or borderline rhythm. Even esophageal syncope may be associated with vagal reflex.

Symptom

Diffuse esophageal fistula symptoms Common symptoms Dysphagia Esophageal reflux symptoms Post-sternal pain Cough cardiocardia When swallowing, syncope, reflux, esophagus, chest pain, swallowing pain

Most patients were asymptomatic, and only found abnormalities in esophageal X-ray barium meal examination and esophageal manometry. They were called asymptomatic diffuse esophageal fistula. Those with milder disease could have no obvious symptoms, but more in the case of seizures. Symptoms, manifested as post-sternal pain or difficulty in swallowing, or both, the degree of symptoms of difficulty in hypopharyngeal is different, some patients are induced by eating cold drinks, carbonated drinks, semi-solid foods such as meat or soft candy, and Affected by emotional and psychological factors, generally no nausea or vomiting.

1. Post-sternal pain Post-sternal pain is the main symptom of this disease, caused by esophageal fistula, the degree of pain varies from person to person, the severity of the performance varies from mild discomfort to severe cramps or severe cramps, can be radiated to the neck The scapula and upper limbs are similar to the symptoms of angina pectoris, but there is no abnormal change in electrocardiogram and myocardial enzyme spectrum at the time of onset. The pain can be relieved after glyceryl trinitrate or sedative. The pain characteristics are:

1 The attack time can be as long as 1 to 2 hours or half a day, regardless of physical activity;

2 In addition to the main complaint, there is generally no special positive sign;

3 related to mood swings, sometimes diagnosed as neuralgia;

4 symptoms can last for many years, can occur automatically or in the meal, slow or sudden, or intermittent, can also wake up at night to wake up the patient; 5 abnormal esophageal contraction with chest pain, but with or without chest pain Does not affect the diagnosis of this disease.

2. Dysphagia Dysphagia is another major symptom of this disease. It is characterized by difficulty in swallowing any food, including hypopharyngeal fluid and solid food, and is susceptible to emotions. Another symptom of difficulty in swallowing is that the symptoms can be slow or Sudden occurrence, or intermittent seizures, no progressive exacerbation, symptoms are not necessarily accompanied by chest pain, the clinical manifestations vary in severity, some patients may have no such symptoms, or the symptoms are very mild, only the food stays in the sternum Some patients may have a round-trip feeling; while some patients show severe dysphagia when eating, and have obvious swallowing pain, sometimes cold food can aggravate symptoms; sometimes a group can block the esophagus for several minutes to several Hours, food seems to be stagnant in the middle of the esophagus, need to drink water after a meal to relieve symptoms.

3. A large amount of food and saliva retained in the ventral esophagus can be in the reflux mouth (not acidic), and even cause aspiration pneumonia, the symptoms of esophageal spasm pain can be alleviated by nausea.

4. External esophageal performance

(1) Neuropsychiatric performance: Mental factors play an important role in the occurrence and development of this disease, such as overwork and emotional stress, which can interfere with the normal activities of the cerebral cortex and affect the autonomic function, which in turn causes the disease. In general, patients have more psychiatric symptoms and often have a neurotic personality.

(2) cardiac manifestations: patients with diffuse esophageal fistula in addition to angina pectoris-like chest pain, can occur when swallowing syncope, called swallowing syncope or esophageal syncope, is a food mass expansion of the esophagus, causing vasovagal reflex, resulting Due to sinus bradycardia or knot rhythm, treatment with atropine can relieve it.

(3) Respiratory tract performance: rare, and severe coughing and aspiration pneumonia may occur in some patients with severe dysphagia.

(4) Whole body performance: Some patients have fear of eating because of fear of dysphagia and pain, which can lead to malnutrition and weight loss, and generally no vomiting symptoms.

5. The clinical manifestations of diffuse esophageal fistula in children with diffuse esophageal fistula can be diverse. The older ones are mostly dysphagia and post-sternal pain; the infants are characterized by esophageal reflux symptoms such as aspiration, apnea and pneumonia. Occurred; a small number of children are prone to irritability, or have a paroxysmal change in position, some children with mental retardation are easy to merge with diffuse esophageal fistula.

Examine

Diffuse esophageal fistula examination

The diagnosis of diffuse esophageal fistula depends on imaging examination and esophageal manometry, and other tests are not specific.

X-ray inspection

Esophageal barium meal angiography This examination is a routine examination item and one of the important methods for diagnosing this disease. The typical manifestation of esophageal barium meal examination is that the esophagus is segmental contraction. Generally, it should be observed in multi-axis photos, visible in the left slice. Signs of thickening to the esophageal wall, but its sensitivity and specificity are not high, about 50% can be misdiagnosed as achalasia or other diseases.

Patients with a transient esophageal fistula may have no positive findings during barium meal examination. In clinical symptoms, segmental spasm occurs in the middle part of the esophagus, showing several deep circular symmetric contractions. The esophageal margin is smooth, soft, and the mucosa is normal. After the intramuscular injection of antispasmodic agent can be relieved, diffuse sputum occurs in the lower part of the esophagus.

For suspicious cases, cold or acidified barium meal may be applied, or food barium may be used to induce spasm. X-ray examination is also used to exclude spasms caused by organic diseases of the esophagus (such as malignant tumors).

X-ray images of diffuse esophageal fistula can have the following characteristics: 1 After swallowing, the lower esophageal peristalsis is weakened, the lumen is passively dilated, the expectorant is passed by gravity flow, and 2 sometimes the lower third or lower part of the esophagus For general stenosis, the stenosis segment is longer, and the upper third or upper part of the esophagus has peristaltic contraction and spreads down in a normal manner, reaching the cardia. 3 mild symptoms can be expressed as mild waves from the lower half of the esophagus. Contraction to obvious symmetrical segmental contraction, accompanied by shortening of the longitudinal axis of the esophagus, these contraction waves are the third contraction wave without push function, 4X line digital gastrointestinal machine continuous observation and dynamic playback study can be seen the third esophagus The appearance of contraction wave, even the normal peristaltic discharge of the expectorant can be seen, but when the third strong contraction wave occurs, the expectorant can be retrogradely pushed to the upper part of the esophagus, that is, the expectorant can sometimes pass through the esophagus normally, and sometimes it is separated, and the expectorant Move to the retrograde direction of esophageal peristalsis, 5 severe symptoms can be expressed in the esophagus, the lower 2 / 3 or the whole esophagus strong non-propelled asymmetry contraction, resulting in esophageal imaging beaded or spiral narrow, go The curvature is slightly stiff, and the esophageal lumen is divided into multiple pseudo-chamber-like changes, sometimes difficult to identify with multiple diverticulum (Fig. 3). In some cases, there are cases of true diverticulum or esophageal hiatus, and the size of the esophageal lumen is normal. There was no expansion, and some patients had no abnormal X-ray findings in one esophageal barium meal examination, but the symptoms of esophageal fistula were repeated.

(2) CT scan: CT can be found in the esophageal sacral esophageal wall thickening, smooth, symmetrical, contraction to the circumference of the esophageal wall, located at the distal end of the esophagus, the fat layer around the esophagus is normal.

2. Esophageal manometry and pH monitoring Esophageal manometry is the first choice for the diagnosis of this disease. Drinking water can also induce abnormal esophageal fistula during pressure measurement. The typical features are low-invasive and recurrent high amplitude in the lower and middle esophagus. Long-term contraction of the wave pattern (Figure 4), but clinically only 60% of patients with diffuse esophageal fistula have a typical performance, 40% of other types of esophageal contraction abnormalities.

The esophageal manometry of this disease is characterized by: 1 simultaneous contraction: the upper, middle and lower segments of the esophagus contract simultaneously, and positive waves appear at all points on the manometer. Some authors suggest that the contraction at least 30% of the swallowing action The cause of esophageal contraction can be used as the diagnostic criteria for this disease, 2 repetitive contraction: that is, more than 3 times of contraction after swallowing, the patient generally has at least 30% of esophageal abnormal reactions during swallowing, ie at the same time or Repeated high amplitude contraction and abnormal phase, which is the basis of esophageal fistula, 3 high amplitude non-pushing contraction: generally refers to the contraction wave with amplitude above 24.0 kPa (180mmHg), repeated high amplitude contraction is a typical manifestation of this disease, The average esophageal contraction time is more than 7.5s. It has been used as the diagnostic criteria for this disease. However, there are fewer clinically consistent with this standard. The original peristaltic contraction of 4 esophagus still exists. The contraction wave can pass through the full length of the esophagus. The middle part of the esophagus began to be replaced by uncoordinated pressure waves. The peristaltic wave was interrupted or spread to the distal end of the esophagus. This is a characteristic of the disease. 5 lower esophageal sphincter relaxation work Normal, the pressure can be kept normal during esophageal manometry; in a few patients, the lower esophageal sphincter relaxation function is impaired, and the esophageal resting pressure is elevated during esophageal manometry, which is to eliminate esophageal spasm and pain caused by gastroesophageal reflux. Can be used for 24h esophageal pH monitoring.

24h esophageal pH monitoring can identify esophageal spasm and chest pain caused by gastroesophageal reflux.

3. Esophagoscopy endoscopic examination is mainly used to exclude esophageal organic lesions. No clinical findings can be found without endoscopic examination, mucosa is normal, the lens body passes without resistance; if the patient's clinical symptoms are obvious, then the examination Endoscopy may not be accessible and should be checked with a sedative or under general anesthesia.

4. Acid perfusion test (Bernsteins test) can induce esophageal spasm and chest pain, clinically used to determine the sensitivity of esophageal acid to identify esophageal chest pain and angina pectoris; electrocardiogram can be no abnormalities, these inspections can help Diagnosis and differential diagnosis.

Diagnosis

Diagnosis and diagnosis of diffuse esophageal fistula

Chest pain and intermittent difficulty in swallowing in the medical history are suspicious clues. There is no positive finding in physical examination. Endoscopy is mainly used to exclude other diseases. The diagnosis depends on X-ray examination and pressure test.

Differential diagnosis

1. The main distinguishing point of angina pectoris and diffuse esophageal fistula.

2. achalasia achalasia also has dysphagia, post-sternal pain and nausea. The symptoms of achalasia are characterized by lower esophageal sphincter pressure and lower esophageal sphincter during swallowing. X-ray examination shows the lower end of the esophagus. Stenosis, the upper part of the esophagus, etc., the main identification points of achalasia and diffuse esophageal fistula.

3. Nutcracker esophagus or supersqueezer esophagus, characterized by excessive peristaltic contraction of the esophagus and/or prolonged contraction time, causing angina pectoris-like chest pain, and the cause of the disease is not Clear, and diffuse esophageal spasm belongs to the primary esophageal dyskinesia, and its relationship with gastroesophageal reflux and diffuse esophageal fistula remains to be further studied. Some authors believe that the disease is a precursor to diffuse esophageal fistula. The differential diagnosis is difficult. The determination of the esophageal pressure of the nutcracker esophagus can be clearly diagnosed. The characteristics of esophageal manometry at the onset of chest pain are:

1 more occurs in the lower part of the esophagus, high amplitude, peristaltic contraction, the average contraction amplitude of the lower third of the esophagus can exceed 16.0kPa (120mmHg), or the peak exceeds 26.7kPa (200mmHg), or the peristaltic contraction time exceeds 6s.

2 The lower esophageal sphincter function is normal, can be relaxed during meals, there is a normal stage I esophageal peristaltic wave, while the asymptomatic esophageal pressure can be normal, the main distinguishing characteristics of the nutcracker esophagus and diffuse esophageal fistula.

4. Esophageal damage caused by scleroderma Scleroderma is an autoimmune disease characterized by hardening of collagen fibers in the skin and various systems. Combined with typical skin changes, esophageal X-ray barium meal examination shows that esophageal peristalsis is weakened, esophageal test Abnormal pressure, abnormal lower sphincter tension in the lower esophagus, etc., easy to identify with this disease.

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