Cardia achalasia, also known as cardia spasm and megaesophagus, is a disease in which the lower esophageal sphincter is not fully relaxed due to neuromuscular dysfunction in the cardia of the esophagus, and food cannot pass smoothly and is retained, thus gradually causing decreased esophageal tone and peristalsis and dilatation of the esophagus. The main features are lack of peristalsis, high pressure of the lower esophageal sphincter (LES) and a reduced relaxation response to swallowing movements. Clinical manifestations include dysphagia, retrosternal pain, food reflux, and cough and lung infection due to inadvertent aspiration of food into the trachea.
The pathogenesis is thought to be related to degeneration, reduction or absence of Auerbach ganglion cells in the esophageal musculature and defects in parasympathetic distribution. The degeneration of ganglion cells is often accompanied by inflammatory manifestations of lymphocytic infiltration, and perhaps the etiology is related to infection and immune factors.
1.Difficulty in swallowing
Painless dysphagia is the most common and earliest symptom of this disease. The onset of the disease is slow, but it can also be urgent, and the initial onset can be mild, with only a feeling of fullness after meals. Dysphagia is usually intermittent and is often triggered by mood swings, anger, apprehension, shock or the consumption of cold, spicy or other irritating foods. At the beginning of the disease, dysphagia is intermittent, sometimes mild and sometimes severe, but later it becomes persistent. A few patients have more difficulty in swallowing liquids than solid food.
2.Pain
It can be dull pain, burning pain, pins and needles pain, cutting pain or cone pain. The pain is mostly in the posterior sternum and upper middle abdomen; it can also be in the back of the chest, the right side of the chest, the right sternal margin and the left quarter rib area. The pain attacks sometimes resemble angina pectoris and may even be relieved by sublingual nitroglycerin tablets. With the gradual increase of dysphagia and further dilatation of the esophagus above the obstruction, the pain is gradually reduced.
3.Food reflux
As the dysphagia worsens and the esophagus dilates further, a considerable amount of contents can be retained in the esophagus for several hours or days, and then reflux out when the body position changes. The contents of reflux from the esophagus do not have the characteristics of vomit in the stomach because they have not entered the gastric cavity, but they can be mixed with large amounts of mucus and saliva. In case of complications of esophagitis and esophageal ulcer, the reflux may contain blood.
4.Weight loss
Weight loss is associated with dysphagia affecting the intake of food. For dysphagia, although the patient mostly takes the choice of food, slow food, drink more soup to wash down the food when eating or after eating, or straighten the chest and back after eating, forceful deep breathing or discard the air and other methods to assist the swallowing action. Long-term disease may have weight loss, malnutrition and vitamin deficiency.
5. Bleeding and anemia
Patients may often have anemia and occasionally bleeding due to esophagitis.
6.Other
Due to the increased tone of the lower esophageal sphincter, patients rarely experience erratic reflux, which is an important feature of the disease. In advanced cases, the extremely dilated esophagus may compress the organs in the chest cavity and produce dry cough, shortness of breath, cyanosis and hoarseness.
1.Barium X-ray of esophagus
Barium swallow examination shows dilated esophagus, weakened esophageal peristalsis, stenosis of the end of the esophagus in the shape of a bird’s beak, and smooth mucosa at the stenosis, which is typical of patients with cardia loss retardation. Henderson et al. classified esophageal dilatation into three grades: grade I (mild), with esophageal diameter less than 4 cm; grade II (moderate), with diameter 4-6 cm; grade III (severe), with diameter greater than 6 cm and even curved in the shape of S.
2.Esophageal kinetic test
The pressure in the high-pressure area of the lower esophageal sphincter is often more than twice as high as normal, and the pressure in the lower segment of the esophagus and sphincter does not drop during swallowing. The pressure in the lumen of the middle and upper esophagus is also higher than normal. The peristaltic wave of esophagus is irregular and small in amplitude. Subcutaneous injection of acetylcholine chloride 5-10mg can enhance esophageal contraction in some cases and significantly increase the pressure in the middle and upper esophageal lumen, and can cause severe pain behind the sternum.
3.Gastroscopy
Gastroscopy can exclude organic strictures or tumors. The endoscopic features of pancreatic stenosis include: (1) most patients have a moderate to large amount of residual food in the esophagus, mostly in a semifluid state covering the wall, and the mucosal edema thickens, resulting in the loss of normal esophageal mucosal color; (2) the body of the esophagus is dilated, with varying degrees of distortion and deformation; (3) the wall of the tube may show segmental constriction rings, resembling diverticula bulging; (4) the degree of pancreatic stenosis varies, until complete atresia cannot be passed. It should be noted that sometimes it is easy to overlook the disease when the perceived resistance of the body through the cardia is not very obvious.
1.Clinical manifestations
Intermittent food stagnation, obstructive sensation, non-progressive dysphagia. Some patients have difficulty entering liquid food than solid food, there is regurgitation, for just swallowed food. There may be dull chest pain and respiratory symptoms due to food reflux at night. The nutritional status is fair.
2.Barium meal examination.
3.Endoscopy of esophagus.
4.Esophageal pressure measurement.
The diagnosis can be confirmed if the above items or 1, 2 or 4 are present. The diagnosis can also be confirmed if only 2 or 4 are present but scleroderma, esophageal cardia cancer and amyloidosis can be excluded.
1.Angina pectoris
Mostly induced by exertion, but cardia is induced by swallowing, and there is difficulty in swallowing, this point can be distinguished.
2.Esophageal neurosis (such as hysteria)
Most of the symptoms are foreign body obstruction from the pharynx to the esophagus, but there is no choking symptom when eating. Benign esophageal stricture and reflex esophageal spasm caused by gastric and gallbladder lesions. The esophagus is only mildly dilated.
3.Esophageal cancer, cardia cancer
The X-ray of cancerous esophageal stricture is characterized by local mucosal destruction and disorder; the stricture is moderately dilated, while pancreatic atelectasis often results in extreme dilatation.
4.Secondary cardia stenosis
The latter is also known as pseudostenosis, which refers to abnormal esophageal motility similar to primary cardia caused by malignant tumors such as gastric cancer, esophageal cancer, lung cancer, liver cancer, pancreatic cancer, lymphoma, Chagas disease, amyloidosis, nodular disease, neurofibromatosis, eosinophilic gastroenteritis, chronic idiopathic pseudo-intestinal obstruction, etc.
Complications
1. Inhaled respiratory tract infection
Bronchial and pulmonary infections can occur when esophageal reflux is exhaled into the airway, especially during sleep. About 1/3 of patients may develop nocturnal paroxysmal choking cough or recurrent respiratory infections.
2. Complications of the esophagus itself
The disease may be followed by esophagitis, esophageal mucosal erosion, ulceration and bleeding, press-out diverticulum, esophage-tracheal fistula, spontaneous esophageal rupture and esophageal cancer.
3. Cardia achalasia can be complicated by esophageal cancer or cardia cancer
The incidence is 0.3%-15%. The reason may be the long-term stimulation of esophageal mucosa by retained material, ulceration and malignant mucosal epithelial proliferation, etc. 1.Medical treatment
Take sedative and antispasmodic drugs, such as oral 1% procaine solution, sublingual nitroglycerin tablets, and the calcium antagonist nifedipine, which has been tried in recent years, can relieve the symptoms. To prevent food overflow into the respiratory tract during sleep, use high pillows or padding the head of the bed.
2.Endoscopic treatment
In recent years, with the deepening of minimally invasive concept and the emergence of new medical techniques and equipment, endoscopic treatment of cardia loss is widely used and many new advances have been made. Traditional endoscopic treatment means can mainly include endoscopic balloon dilation and stent implantation treatment, microscopic injection of botulinum toxin type A and endoscopic microwave incision and sclerotherapy.
3.Transoral endoscopic myotomy (POEM)
POEM surgery is performed without skin incision, through endoscopic circumferential myotomy of the cardia to restore the physiological function of the esophagus to the maximum extent and reduce the complications of the surgery. 95% of the patients can eat early after the surgery, and the dysphagia is relieved after the surgery, and the incidence of reflux esophagitis is low. Due to the short operation time, small trauma, exceptionally fast recovery and reliable efficacy of POEM, it is perhaps the best choice for the treatment of cardia achalasia at present.
4.Surgical treatment
Surgical treatment should be performed for patients with moderate or severe and poor results of traditional endoscopic treatment. Myotomy of the cardia (Heller procedure) is still the most commonly used procedure. It can be done transthoracically or transabdominally, or thoracoscopically or laparoscopically. The main long-term complication is reflux esophagitis, and thus there are many advocates for additional anti-reflux surgery, such as fundoplication around the end of the esophagus at 360 degrees (Nissen procedure), 270 degrees (Belsey procedure), 180 degrees (Hill procedure) or suturing the fundus to the ventral segment and anterior wall of the esophagus (Dor procedure).