OVERVIEW
The disease is also known as high-pressure esophageal peristalsis, supercompressed esophagus, and high-amplitude peristaltic esophagus. It is an independent disease characterized by abnormal esophageal dynamics and symptomatic hyperdynamic esophageal peristalsis (high-amplitude peristaltic contractions with prolonged contraction time frame), and is one of the primary esophageal motility disorders, which can occur at any age, and is more common after the age of 40 years, and is more frequent in females than in males. 1977 Brand et al. firstly reported that in patients with non-cardiogenic chest pain, 41% of them had high amplitude peristalsis. In 1979, Benjami et al. first used the term “nutcracker esophagus” to describe patients with noncardiac chest pain who had esophageal systolic pressures greater than 400 mm Hg.
Etiology
The etiology of nutcracker esophagus is unknown, and it is thought to be part of the progression of primary esophageal motility disorders and probably a precursor to diffuse esophageal spasm. It is also thought that its pathogenesis is related to the response to acid reflux, as well as psychosomatic factors and a lowered pain threshold, and that psychiatric and psychological factors can precipitate the onset of chest pain symptoms in this disease.
Symptoms
Nutcracker esophagus is characterized by angina-like chest pain episodes and dysphagia, and its clinical manifestations are similar to those of diffuse esophageal spasm, with the intensity of pain, frequency of episodes, and location varying from person to person.
1. Chest pain
It is the main clinical symptom of Nutcracker esophagus. Most patients have chest pain, which is often related to exertion. Typical manifestation is chronic, recurrent or intermittent chest pain, often located behind the sternum or under the raphe, and the degree is more intense and colic-like pain. Chest pain can be triggered by acidic food or mental or psychological factors such as depression, anxiety, emotional excitement, etc. It can also be accompanied by radiating pain in the back. The nature of chest pain is similar to angina pectoris, but the patient’s coronary angiography is mostly without abnormal findings. Nutcracker esophageal chest pain is due to increased amplitude and/or prolonged duration of peristaltic contraction of the esophagus, and its incidence is higher than that of diffuse esophageal spasm or pancreatic achalasia.
2. Dysphagia
It is seen in about 70% of patients with nutcracker esophagus. Dysphagia is often associated with episodes of chest pain and can be relieved by nitroglycerin preparations and calcium channel blockers.
3. Heartburn
It is associated with gastroesophageal reflux and increased sensitivity of the esophageal mucosa. Gastric acid reflux may play an important role in the development of symptoms.
Examination
1. Esophageal manometry
Esophageal manometry in nutcracker esophagus is characterized by high amplitude peristaltic contractions with prolonged duration of contraction. The diagnosis can be confirmed by the presence of peristaltic esophageal contractions during an episode of chest pain, but the average contraction amplitude of the lower third of the esophagus exceeds 16 kPa (120 mmHg) or peaks at more than 26.7 kPa (200 mmHg) or lasts for more than 7 seconds.
2. Esophageal X-ray barium angiography
Esophageal X-ray barium contrast of Nutella esophagus can be normal or suggestive of nonspecific esophageal motility dysfunction, which lacks specificity in diagnosing Nutella esophagus, but is important in excluding esophageal organisms and other functional abnormal pathologic changes.
3. Tensilon provocation test
Chest pain and esophageal pressure are induced after intravenous tensilon is given to the patient, and abnormalities are positive. Nutcracker esophagus may record normal esophageal manometry during asymptomatic periods, and the tensilon provocation test may be used in these patients to determine whether their chest pain is related to abnormal esophageal contractions.
4. Standard Acid Perfusion Test
Standard acid perfusion of the esophagus is positive when the patient has an episode of chest pain or abnormal esophageal pressure.
Diagnosis
The symptoms of chest pain caused by nutcracker esophagus are similar to those of angina pectoris, and the drugs used to treat angina pectoris are effective in relieving it, making the diagnosis difficult to make. Some authors have reported that chest pain persisted in patients with a clinical diagnosis of angina pectoris despite coronary artery bypass grafting. The diagnosis was made only after 24-hour intraesophageal pH monitoring and esophageal manometry. Nutcracker esophagus may be considered if the following conditions are present:
1. chronic, recurrent or intermittent episodes of severe chest pain, routine cardiovascular examination and coronary angiography to rule out cardiogenic chest pain.
2. With or without dysphagia.
3. Esophageal manometry showing high amplitude peristaltic contractions and prolonged duration.
4. Esophageal endoscopy and imaging showed no structural abnormality of the esophagus.
5. Positive acid infusion or tensilon provocation test.
Treatment
There is no specific treatment for Nutcracker esophagus.
1. Internal medicine
Drugs can be used to reduce the high amplitude esophageal contraction to alleviate chest pain and dysphagia. Commonly used drugs include anticholinergics and calcium channel blockers, but none of them are consistently effective. In addition, these drugs slow acid clearance from the esophagus while decreasing lower esophageal sphincter pressure, thus exacerbating gastroesophageal reflux. Antisecretory drugs may also improve symptoms, and some studies have demonstrated that antacid therapy is more effective than smooth muscle relaxants in patients with acid reflux. Sedatives or anxiolytics, such as diazepam and alprazolam, may be given to patients with psychiatric or psychological factors, and psychotherapy may be effective in patients with nutcracker esophagus.
2. Surgical treatment
Winters et al. (1984) reported that dilatation therapy is effective for nutcracker esophagus. Surgery is limited to patients whose clinical symptoms are not relieved after long-term formal treatment. Preoperative esophageal function tests should be performed to confirm abnormal esophageal function and psychological evaluation should be performed to exclude psychological factors. Surgery can be performed by esophageal myotomy, with myotomy covering the lower esophageal sphincter, in order to achieve esophageal atony and a significant decrease in contraction amplitude and duration. To prevent postoperative gastroesophageal reflux, anti-reflux surgery is often performed at the same time.