pancreatic dystrophy



Overview

  • Disorders of muscle relaxation in the cardia of the esophagus, resulting in the retention of food in the esophagus due to the inability to pass through normally.
  • Difficulty in swallowing, retrosternal pain, vomiting, etc.
  • Unknown to date, generally thought to be due to neuromuscular dysfunction
  • Includes general treatment, drug treatment, endoscopic treatment, and surgical treatment.
  • What is pancreatic achalasia?

    Definition

    Pancreatic achalasia, also known as esophageal achalasia and cardia spasm, is a condition in which there is no peristaltic movement of the esophagus and the lower esophageal sphincter does not relax properly, preventing food from passing through the esophagus. The main clinical manifestation is intermittent dysphagia.

    Types

    According to the size of esophageal diameter

    The degree of esophageal dilatation can be classified into Grade I, Grade II and Grade III.

  • Grade I: mild lesions, esophageal diameter less than 4 cm.
  • Grade II: moderate lesions with esophageal diameter of 4-6 centimeters.
  • Grade Ⅲ: severe lesions with esophageal diameter greater than 6 cm or even curved in an “S” shape, also known as sigmoid esophagus.
  • According to the different endoscopic manifestations

    Cardia laxity can be categorized into 3 types (Ling’s classification).

  • Type I: the lumen is mildly dilated and the wall is smooth without tortuosity.
  • Type II: the lumen is dilated and appears as a ring or semilunar structure after adequate air injection.
  • Type II a: a fine annulus with no meniscus.
  • II b: a semilunar structure appears, but not more than 1/3 of the lumen.
  • II c: a semilunar structure is present and exceeds 1/3 of the lumen.
  • III l: marked lumen dilatation with formation of diverticulum-like structures.
  • III l: diverticulum-like structures are located on the left side.
  • Ⅲ r: diverticulum-like structures are located on the right side.
  • Ⅲ lr: diverticulum-like structures are seen on both the right and left sides.
  • Based on the results of esophageal manometry (HRM)

    Cardia dystrophy can be categorized into 3 types (Chicago classification).

  • Type I (classic): median IRP (integrated relaxation pressure) >15 mmHg and 100% loss of peristaltic contraction of the esophagus.
  • Type II (with esophageal luminal hypertension): in addition to the classic features, there is total esophageal luminal hypertension in ≥20% of swallowing processes.
  • Type III (spasmodic): median IRP ≥15 mmHg, no normal peristalsis in the esophagus, and spasmodic contractions with DCI (distal contraction integral) >450 mmHg-s-cm in ≥20% of swallowing processes.
  • Pathogenesis

  • There are no authoritative or recognized statistics on the incidence of pancreatic achalasia in China.
  • The incidence is about 1 per 100,000 people in western countries such as Europe and the United States.
  • Questions you may be concerned about

    Is it true that a Chinese medicine can cure pancreatic flaccidity?

    There is no method to cure pancreatic dyskinesia completely, and the main purpose of treatment is to relieve the symptoms.

    Dialectical application of traditional Chinese medicine may be able to relieve symptoms such as dysphagia, acid reflux, and vomiting, but it will not be able to achieve a radical cure. If the symptoms are severe or the medication is ineffective, dilatation or surgery is recommended.

    It is recommended that patients go to regular medical institutions.

    What medications are taken for pancreatic achalasia?

    Nitrate drugs and calcium channel blockers can relax the esophageal muscles and relieve the symptoms of dysphagia. Commonly used nitrate drugs are nitroglycerin and commonly used calcium channel blockers are nifedipine and diltiazem.

    Local injection of botulinum toxin into the esophageal muscle also helps to temporarily relax the muscle and relieve symptoms.

    If the patient has symptoms of acid reflux and heartburn, a proton pump inhibitor (PPI) such as omeprazole can be used as prescribed by the doctor for relief.

    What are the consequences of prolonged untreated cardia?

    When left untreated for a long time, food tends to remain in the esophagus, which not only affects the absorption of nutrients, but also leads to further impairment of the esophageal function and aggravation of symptoms such as dysphagia and reflux.

    Chronic stimulation of the esophageal mucosa by food may also lead to congestion, inflammation and even ulceration of the mucosa, and a few patients may even develop cancer.

    Causes

    Causes

    According to the different causes, cardia dystrophy can be categorized into two major groups: primary and secondary.

    Primary

  • Smooth muscle inhibitory motor neuron defect: There is a smooth muscle inhibitory motor neuron defect in cardia, so it can be inferred that smooth muscle inhibitory motor neuron defect may be related to this disease.
  • Auerbach’s ganglion lesions: cardia pallidum may be associated with degeneration, reduction, or absence of Auerbach’s ganglion cells within the esophageal muscularis propria and defective parasympathetic distribution.
  • Inflammation: Ganglion cell degeneration is often accompanied by inflammatory manifestations of lymphocytic infiltration; therefore, it can be inferred that cardia dystrophica may be associated with infections and immune abnormalities.
  • Secondary.

    Also known as pseudopancreatic achalasia, it can be secondary to the following diseases:

  • Malignant tumors: gastric cancer, esophageal cancer, lung cancer, liver cancer, pancreatic cancer, and lymphoma.
  • Parasitic diseases: Chagas disease.
  • Others: amyloidosis, tuberculosis, neurofibromatosis, eosinophilic gastroenteritis.
  • Pathogenesis

  • Normally, the sphincter at the end of the esophagus can be stretched during swallowing, allowing food to pass through and enter the stomach without difficulty.
  • In pancreatic achalasia, the sphincter at the end of the esophagus fails to dilate during swallowing due to various reasons, which in turn prevents food from passing through and accumulates in the esophagus, causing esophageal dilatation and abnormal peristalsis.
  • Symptoms

    Typical symptoms

    Dysphagia, vomiting, and retrosternal pain are the most typical symptoms of the disease.

    Dysphagia

  • The earliest and most frequent symptom of cardia dysphagia.
  • Early symptoms are mild, may only have a sense of stagnation or obstruction behind the sternum when eating, with intermittent episodes, sometimes mild and sometimes severe; later it becomes persistent, often with reflux immediately after swallowing; with the progression of the disease, the esophagus is gradually dilated, and the dysphagia is reduced instead.
  • Vomiting

  • Vomiting can be caused by the accumulation of food in the esophagus to a certain extent, and it is more likely to occur when lying down.
  • It usually occurs within 20 to 30 minutes after eating.
  • Symptoms may be relieved by vomiting of food. In cases of esophagitis or esophageal ulcers, blood may be present in the vomit.
  • Pain

  • The pain is usually located behind the sternum, below the xiphoid process and in the middle-upper abdomen, and occurs during meals, especially when eating irritating foods such as too cold or too acidic foods.
  • The pain is irregular with the progress of the disease, and sometimes nocturnal pain can also occur.
  • Accompanying symptoms

    In pancreatic achalasia, the patient is unable to eat actively due to difficulty in swallowing. In the long run, the patient may suffer from malnutrition, which may be characterized by weight loss, fatigue, and mental depression.

    Complications

    If treatment is not timely and standardized, pancreatic ataxia can lead to the following complications:

    Short-term complications

  • Aspiration pneumonia: Food that does not enter the stomach can enter the pharynx and trachea, which in turn may cause aspiration pneumonia. It is characterized by coughing up sputum, shortness of breath, and fever.
  • Fungal esophagitis: Fungal esophagitis can be triggered by the accumulation and fermentation of food that does not enter the stomach in the esophagus or irritation by drugs. It is characterized by painful swallowing, retrosternal discomfort and burning sensation, and may be accompanied by loss of appetite, nausea and vomiting.
  • Long-term complications

    Esophageal cancer: Long-term pancreatic achalasia carries a certain risk of cancer. It is characterized by choking sensation when swallowing food, pain behind the sternum, etc. Difficulty in swallowing, pallor, loss of weight, weakness, hoarseness, choking and coughing, and difficulty in breathing may occur with the progression of the disease.

    Consultation

    Department of Medicine

    Gastroenterology

    If you experience symptoms such as difficulty swallowing, vomiting, or pain behind the sternum, it is recommended that you seek medical attention.

    Preparation

    How to get to the doctor: registering, preparing documents, and frequently asked questions.

    Tips

    Before going to the doctor, try to keep a record of the symptoms you have experienced and their duration for the doctor’s reference.

    Preparation List

    Symptom list

    Pay particular attention to the time of onset of symptoms, special manifestations, etc.

  • Is there any difficulty in swallowing, vomiting, or pain behind the sternum?
  • How long have these symptoms been present? Are they relieved?
  • What is your recent appetite?
  • Has there been any recent change in weight?
  • Are there any other symptoms such as nausea, vomiting and fever?
  • Have there been any recent symptoms such as coughing or hoarseness?
  • Have you eaten unclean food or drunk unsanitary water recently?
  • What medications have been taken?
  • Medical History Checklist
  • Any previous gastrointestinal illness?
  • What are the bad habits? Such as smoking and drinking alcohol.
  • Have you had any relevant examinations or treatments?
  • Any history of drug allergy, etc.?
  • Checklist

    Examination results in the past six months, which can be brought to the doctor’s office

  • Esophageal X-ray barium meal imaging
  • Gastroscopy
  • Esophageal manometry
  • Medication list

    Medication used in the last 3 months, if available in boxes or packages, bring with you to the doctor’s office

  • Acid-suppressing drugs: omeprazole, rabeprazole, ranitidine
  • Gastric stimulant drugs: Domperidone, Mosapride
  • Diagnosis

    Disease diagnosis

    Relevant history, characteristic clinical manifestations, imaging, and endoscopic findings are important in the diagnosis of pancreatic achalasia.

    History

    The etiology of pancreatic achalasia is unknown to date, and the relationship to other diseases is not well defined. Therefore, there is usually no relevant history of the disease.

    Clinical manifestations

    Typical symptoms such as dysphagia, retrosternal pain, coughing, lethargy, and fatigue may help the physician diagnose the disease.

    Imaging

  • The main imaging test for pancreatic dysphagia is a barium meal x-ray of the esophagus.
  • Barium meal imaging can clearly show the dilated esophagus and the so-called “bird’s beak sign”, which is the characteristic imaging manifestation of this disease.
  • Barium esophageal X-ray can not only diagnose the disease, but also determine the severity of the disease.
  • Endoscopy

  • Through endoscopy, the doctor can clearly see the mucosa of the esophagus, and can find characteristic pathological changes such as punctate protrusions.
  • Due to the impaired relaxation of the sphincter at the end of the esophagus, there is an obvious increase in resistance when the doctor moves the endoscope through the cardia.
  • The endoscope can also be used to extract diseased tissue for pathologic examination to determine whether cancer is present.
  • Esophageal manometry

    Esophageal manometry is the gold standard for the diagnosis of pancreatic achalasia, which is usually characterized by the loss of esophageal smooth muscle peristalsis, incomplete relaxation of the lower esophageal sphincter, and often a significant increase in lower esophageal sphincter pressure.

    Differential Diagnosis

    Gastroesophageal reflux disease

  • Similarities: Acid reflux, retrosternal pain, and pain and difficulty in swallowing may occur.
  • Differences: Gastroesophageal reflux disease (GERD) does not present with dilatation of the esophagus, which can be differentiated by esophageal X-ray and barium meal contrast, and if there is a “bird’s beak sign”, it is called pancreatic achalasia. In addition, endoscopic damage to the esophageal mucosa can be detected in GERD, which is generally not seen in cardia. It can also be differentiated by endoscopy as well as by esophageal acidity (pH) testing.
  • Nutcracker esophagus

  • Similarities: Both may present with chest pain, dysphagia, acid reflux, and other symptoms, and may be associated with long-term malnutrition, weight loss, and psychological disorders such as anxiety; they are also commonly seen in young adults.
  • Differences: Barium meal X-ray of the esophagus in pancreatic achalasia may show the typical “bird’s beak sign” due to esophageal dilatation, whereas it does not appear in nutcracker esophagus. Therefore, it can be differentiated by this test.
  • Gastric emptying disorder (gastroparesis)

  • Similarities: Both may present with retrosternal pain, acid reflux and weight loss.
  • Differences: Gastric emptying disorder is characterized by delayed gastric emptying, which is common in women or after stomach or pancreatic surgery, while cardia laxity is most common in middle-aged and young people between the ages of 30 and 40 years old, and is usually without a cause; it is characterized by high intragastric pressure and an abnormal gastric electrical rhythm. Therefore, it can be identified with the help of intragastric manometry and electrogastrogram.
  • Treatment

  • Aim of treatment: At present, there is no cure for cardia dystrophy, and the aim of treatment is to release the lower esophageal sphincter relaxation obstacle during swallowing, so that food can pass into the stomach.
  • Treatment principle: There are general treatment, drug treatment, endoscopic treatment and surgical treatment; drug treatment is preferred, and endoscopic and surgical treatment can be carried out when the treatment is not effective.
  • General treatment

  • Slow down the speed of eating.
  • Drink warm water before meals, during meals and continue to drink warm water after meals to help food enter the stomach.
  • Avoid indigestible and irritating foods.
  • Adjust your mental state and avoid excessive anxiety.
  • Medication

    Calcium channel blockers

  • Calcium channel blockers can relieve symptoms such as dysphagia.
  • Commonly used drugs include nifedipine and diltiazem.
  • Acid suppressants

  • Proton pump inhibitors (PPI) or H2 receptor antagonists (H2RA) can be used as prescribed by the doctor to relieve symptoms such as acid reflux and heartburn.
  • Commonly used drugs are omeprazole, cimetidine, etc.
  • Nitrates

  • These drugs can effectively relax the smooth muscle of the esophagus, and can relieve symptoms such as dysphagia.
  • Commonly used drugs are nitroglycerin.
  • Botulinum toxin local injection

  • Botulinum toxin local injection can inhibit the excitatory neurons in the esophageal sphincter area, reduce the tension of the esophageal sphincter, and then relieve the symptoms.
  • Botulinum toxin injection is dangerous, therefore, it is recommended to go to a regular medical unit and have the treatment completed by a professional physician.
  • Endoscopic treatment

    Endoscopic treatment is divided into transoral endoscopic myotomy and endoscopic balloon dilatation and stent implantation.

  • Transoral endoscopic myotomy (POEM): It is characterized by small trauma and long-lasting efficacy. It can effectively relieve symptoms such as dysphagia and vomiting. However, complications such as esophageal perforation and bleeding can occur in some cases.
  • Balloon dilatation/stent implantation treatment: It is suitable for those who have poor drug efficacy and obvious dysphagia and vomiting, especially vomiting and choking at night.
  • Surgery

  • The principle of surgical treatment for cardia dystrophy is myotomy, which can directly relieve the muscle relaxation disorder of esophageal cardia.
  • Currently, the commonly used surgical procedures are transthoracic or transcardia myotomy, i.e. Heller’s procedure.
  • Different approaches can be taken depending on the patient’s specific situation. For example, traditional open surgery via the chest or abdomen, and less invasive laparoscopic surgery.
  • Complications of surgery are gastroesophageal reflux disease, which can be treated with fundoplication.
  • Prognosis

    Cure

    After timely and scientific treatment, cardia dystrophy can generally relieve symptoms and significantly improve the quality of life, but it is difficult to achieve a cure in the true sense of the word.

    Harmfulness

    There are the following dangers of cardia flaccidosis:

    Affecting life

  • Dyspepsia may cause difficulty in swallowing and affect normal eating.
  • Pancreatic achalasia may present with retrosternal pain, coughing, lethargy, and fatigue, which affects the quality of daily life.
  • Complications

    Complications such as aspiration pneumonia, fungal esophagitis, personality changes, and even esophageal cancer can occur with pancreatic achalasia.

    Impact on life expectancy

    There is a 2% to 7% risk of cancerous transformation of pancreatic achalasia, which usually turns into esophageal cancer and can cause death in severe cases, affecting normal life expectancy.

    Daily life

    Daily life

    Dietary regulation

  • Regular diet and small meals are recommended to avoid esophageal irritation caused by eating too much at one time.
  • Daily diet should include a variety of foods, fresh vegetables, fruits and essential nutrients.
  • In case of manifestations such as weight loss, it is recommended to visit the dietetics department at the same time as treatment and consult a professional dietitian for dietary plans.
  • Lifestyle habits

  • Ensure a reasonable amount of sleep.
  • Avoid excessive stress and should be relaxed.
  • Quit smoking and drinking.
  • Mood Adjustment

    Positively adjust the bad mood and keep a good state of mind.

    Special Matters

    Cardia laxity may induce esophageal cancer, so patients with this disease should have regular medical checkups.

    Follow-up

  • Follow the doctor’s instructions for regular follow-up.
  • If the condition does not improve during the treatment or becomes more serious, it is recommended to consult a doctor.
  • Prevention

    Since the cause and pathogenesis of pancreatic achalasia are not known, there are no effective prevention methods, but the following are recommended.

  • People who have a family history of the disease or who have an immediate family member diagnosed with the disease are recommended to have regular medical checkups.
  • Do not overeat, but eat smaller and more frequent meals.
  • Eat a light and easily digestible diet, do not eat too greasy.
  • Do not eat spicy and stimulating food, cold food, hard food, or food with too high temperature.
  • Quit smoking and drinking.
  • Drink less strong tea, strong coffee, etc.
  • Avoid unnecessary medications.
  • Exercise in moderation, with a balance of aerobic as well as strength training.
  • Ensure that you are in a good mood and get enough sleep.
  • Seek immediate medical attention if you feel unwell.