Cardia insipidus is a functional disorder due to neuromuscular dysfunction of the esophagogastric junction. The main features are lack of peristalsis and impaired relaxation of the lower esophageal sphincter.
1. Cardia achalasia is a disorder of esophageal motility.
2. Symptoms may not be consistent with the degree of esophageal constriction, which may lead to delayed misdiagnosis. Primary difficulty in swallowing liquids is the classic symptom suggestive of cardia incontinentia.
3, Different subtypes have different responses to treatment.
4, For patients with no contraindications to surgical treatment, myotomy (laparoscopic or endoscopic) or balloon dilation are recommended as treatments that can definitively reduce obstruction.
Signs and symptoms
The most common symptoms of achalasia are listed in Table 1. progressive dysphagia for solids and liquids is the hallmark symptom for the diagnosis of the disease. Esophageal dynamics are performed only after organic obstruction and oropharyngeal-related etiologies have been excluded.
Respiratory-related symptoms are also common, as esophageal contouring is impaired and patients are prone to aspiration. In addition, a diagnosis of connective tissue disease (e.g., scleroderma) should be considered if the patient has both chronic respiratory disease and esophageal motility abnormalities.
Table 1: Symptoms associated with cardia loss
Esophageal symptoms
Dysphagia (90% of patients)
Heartburn (75% of patients)
Reflux or vomiting (45% of patients)
Non-cardiogenic chest pain (20% of patients)
Epigastric pain (15% of patients)
Painful swallowing (<5% of patients)
Other signs and symptoms
Cough or asthma (20-40% of patients)
Chronic aspiration (20-30% of patients)
Hoarseness or sore throat (33% of patients)
Weight loss (10% of patients)
Diagnosis
The diagnosis of cardia incontinentia is determined by a combination of symptoms and relevant tests. When a patient complains of dysphagia, a detailed history is required to identify oropharyngeal dysphagia from esophageal dysphagia by observing the patient’s swallowing movements when drinking water.
When the oropharyngeal etiology is excluded, it is necessary to identify whether the esophageal dysphagia is organic or dynamic. The former can be excluded by upper gastrointestinal endoscopy or radiological examination. If the patient has a previous history of fundoplication or bariatric surgery, he or she may present with signs and symptoms similar to those of cardia achalasia, at which point the focus should be on finding the mechanical factors causing the obstruction, such as anastomotic stricture, overtightening of the iterative suture ring, and fundoplication obstruction.
Table 2, Differential diagnosis and preferred tests for cardia inactivation
Symptoms and signs
Examination
Esophageal dysphagia
Structural esophageal disease
Ulcerative stricture
Gastrointestinal endoscopy, barium meal
Esophageal rings or webs
Gastrointestinal endoscopy, barium meal
Eosinophilic esophagitis
Gastrointestinal endoscopy
Malignant tumor
Gastrointestinal endoscopy, barium meal
Radiation or drug-related esophageal stricture
Gastrointestinal endoscopy, barium meal
Foreign body obstruction
Gastrointestinal endoscopy
Vascular compression
CT, MRI, EUS
Mediastinal masses/external compressions
CT, MRI, EUS
Esophageal dynamic disease
Delayed cardia and esophagogastric outflow tract obstruction
High resolution manometry, barium meal
Esophageal non-contractility
High-resolution manometry
Terminal esophageal spasm
High-resolution manometry
Highly constricted esophagus
High-resolution manometry
Esophageal peristaltic dysfunction
High-resolution manometry
Scleroderma
High-resolution manometry
Gastroesophageal reflux disease
Gastrointestinal endoscopy, high resolution manometry, pH testing
Chagas disease
Barium meal, serology
Oropharyngeal dysphagia
Structural oropharyngeal disease
Malignancy
Laryngoscopy
Cervical spine spur
Fluoroscopy, CT
Zenker diverticulum
Fluoroscopy, gastrointestinal endoscopy
Ring, Web
Gastrointestinal endoscopy, barium meal
Radiation injury
Fluoroscopy, gastrointestinal endoscopy
Pharyngeal infection
Laryngoscopy
Enlarged thyroid gland
Ultrasound, CT
Neuromuscular diseases
Cerebrovascular accident
CT, MRI
Multiple sclerosis
Neurology specialized physical examinations, disease-related laboratory tests and examinations
Parkinson’s disease
Myasthenia gravis
Amyotrophic Lateral Sclerosis
Myotonic dystrophy
Dermatomyositis
Thyroid disease
1. Upper gastrointestinal endoscopy
Endoscopy plus biopsy should be performed in all patients with dysphagia to rule out gastroesophageal reflux disease, eosinophilic esophagitis and esophageal cancer. Endoscopic signs of esophageal motility abnormalities include: twisted or dilated esophagus; accumulation of food or fluid in the esophagus; and difficulty inserting the esophagogastric junction. Patients with cardia loss retardation often develop candidiasis due to esophageal sludge. It is recommended to check esophageal motility when patients with esophageal candidiasis infection have normal immune function.
2. Barium meal angiography
The “beak sign” on barium radiography is the classic manifestation of cardia incontinence. Other manifestations suggesting the disease include dilatation of the esophagus, filling defect, “screw cone sign” and loss of peristalsis.
3. Esophageal manometry
Esophageal manometry, which uses a manometric catheter to assess esophageal pressure and systolic function, has become the standard test for the diagnosis and staging of achalasia of the cardia. The advent of high-resolution esophageal manometry (HRM) over the past decade has been a great technological breakthrough, enabling full functional monitoring from the pharynx to the stomach and allowing physicians to visualize esophageal power function. The Chicago classification of esophageal motility disorders, based on the characteristics of HRM analysis, is gradually being refined and has gained wide acceptance. (See Figures 1 and 2)
1.jpg
Figure 1. Staging of esophageal disease on the basis of high-resolution manometry and barium meal.
Figure 2, Chicago typing of abnormal esophageal motility disorders (including cardia inactivation).
Treatment
There is no cure for achalasia of the cardia, and the treatment is summarized in Table 3, with the aim of early intervention to prevent serious complications and preserve esophageal structure and function.
Table 3: Current treatment options for cardia failure
Treatment
Duration of remission maintained
Key points
Drug treatment
Relapse upon discontinuation of medication
None
Botulinum toxin injection
6-12mo
Under anesthesia, through endoscopy, operation time <30 min, postoperative observation 60 min
Balloon dilation
2-5y
Under anesthesia, via fluoroscopy and endoscopy, operation time 30 min, postoperative observation 4-6 h
Surgical myotomy
5-10y
General anesthesia, surgical procedure, operating time 90 min, hospitalization 1-2 d
POEM
not available
General anesthesia, through endoscopy, operation time 90 min, hospitalization required 1d
1. Drug treatment
Oral calcium antagonists and nitrate preparations can reduce the pressure of the lower esophageal sphincter by 47%-64% and have some therapeutic effect on dysphagia. However, these drugs have certain side effects (headache, postural hypotension or edema) and do not stop the disease process. Therefore, they cannot be used as long-term treatment and are generally used only in patients who cannot tolerate surgical and endoscopic treatment. Phosphodiesterase inhibitors (e.g., sildenafil) are also used to treat cardia inactivation, to reduce pressure in the esophagogastric junction, and to attenuate terminal esophageal constriction. Sildenafil can be used in patients who are intolerant or ineffective to calcium antagonists and nitrate agents, but its long-term efficacy is uncertain.
2. Botulinum toxin injection
Botulinum toxin injections in the lower esophageal sphincter block the release of acetylcholine from nerve endings, thus treating cardia inactivation. However, most patients relapse or require retreatment within 12 months, and retreatment can make future Heller myotomy very difficult. Therefore, this treatment is not used as a first-line treatment.
3. Balloon dilation
In this method, a cylindrical balloon is inserted under fluoroscopy through the lower esophageal sphincter, and the esophagus is forcibly dilated using a hand-held manometer with air injection. The efficiency is about 62%-90%. If the patient relapses or has poor results after dilatation, re-dilation is usually not effective but does not interfere with subsequent myotomy treatment. In addition, the risk of perforation with balloon dilation is about 0-16% and therefore requires an experienced endoscopist.
4. Myotomy
Heller myotomy is the standard surgical procedure for the treatment of cardia incontinence, in which the circular muscle fibers of the lower esophageal sphincter are separated. The efficiency of laparoscopic Heller myotomy is about 88%-95%, which is better than balloon dilation.
5. Transoral endoscopic myotomy (POEM)
POEM is the newest means of treating cardia failure. The direct success rate of POEM is greater than 90%, but its long-term results remain to be studied.
Prognosis and follow-up
The prognosis of delayed cardia is related to its subtype: (1) Patients with type II benefit most from myotomy or balloon dilation treatment (success rate 96%). (2) Patients with type I have a slightly worse outcome, with a success rate of 81%. (3) Type III patients have the worst prognosis (success rate 66%), probably because muscle spasm is less likely to benefit from treatment targeting the lower esophageal sphincter.
The optimal follow-up strategy is to assess the patient’s symptom relief, nutritional status, and time to emptying of the barium swallow in a staged fashion. Esophageal manometry is also a better follow-up tool if conditions permit and the patient can tolerate it. Although we cannot intervene therapeutically because of an abnormal barium swallow or manometry result alone, with these follow-up results, we can enhance follow-up and thus stop disease progression.
The risk of squamous carcinoma is significantly higher in patients with cardia loss relaxation than in the general population, but there is not sufficient basis to support routine endoscopic screening.
There is no cure for achalasia, and more than 20% of treated patients will develop symptoms within 5 years and require retreatment. In patients with severe esophageal dilatation and in those who fail to respond to treatment, esophagectomy is the last resort.