Achalasia cardia is a lack of relaxation or insufficient relaxation of the lower esophagus of neurogenic etiology. It is accompanied by a disorder of the reflex opening of the cardiac orifice during the act of swallowing and a violation of the ingestion of food masses from the esophagus into the stomach. Disease is manifested by dysphagia, regurgitation and pain in the epigastrium. The leading diagnostic methods are radioscopy of the esophagus, esophagoscopy, esophagomanometry. Conservative treatment consists in carrying out pneumocardiodilation; surgical – in performing cardiomyotomy.
ICD 10
K22.0 Achalasia cardia
Achalasia cardia meaning
Achalasia cardia in the medical literature is sometimes referred to by the terms megaesophagus, cardiospasm, idiopathic enlargement of the esophagus. Pathology, according to various sources, in practical gastroenterology accounts for from 3 to 20% of all diseases of the esophagus. Among the causes of esophageal patency disorders, cardiospasm is in third place after esophageal cancer and post-burn scar strictures.
Pathology affects women and men with the same frequency, develops more often at the age of 20-40 years. With this disease, as a result of neuromuscular disorders, the peristalsis and tone of the esophagus decrease, there is no reflex relaxation of the esophageal sphincter when swallowing, which makes it difficult to evacuate food from the esophagus into the stomach.
Achalasia cardia causes
Many etiological theories of the origin of achalasia cardia have been put forward, which link the development of pathology with a congenital defect of the nervous plexuses of the esophagus, secondary damage to nerve fibers in tuberculous bronchoadenitis, infectious or viral diseases; vitamin B deficiency, etc. The concept of violations of the central regulation of esophageal functions is widespread, considering achalasia cardia as a consequence of neuropsychiatric injuries leading to cortical disorder neurodynamics, innervation of the esophagus, discoordination in the work of the cardiac sphincter. However, until the end, the factors contributing to the development of the disease remain unclear.
The leading role in the pathogenesis of achalasia cardia is assigned to the defeat of the departments of the parasympathetic nervous system that regulates the motility of the esophagus and stomach (in particular the Auerbach plexus). Secondary (symptomatic) achalasia cardia can be caused by infiltration of the plexus by a malignant tumor (gastric adenocarcinoma, hepatocellular cancer, lymphogranulomatosis, lung cancer, etc.). In some cases, the Auerbach plexus can be affected by myasthenia gravis, hypothyroidism, poliomyositis, systemic lupus erythematosus.
Pathogenesis
The actual denervation of the upper gastrointestinal tract causes a decrease in peristalsis and tone of the esophagus, the impossibility of physiological relaxation of the cardiac orifice during the act of swallowing, muscle atony. With such disorders, food enters the stomach only due to the mechanical opening of the cardiac orifice, which occurs under the hydrostatic pressure of liquid food masses accumulated in the esophagus. Prolonged stagnation of the food lump leads to the expansion of the esophagus – megaesophagus.
Morphological changes in the esophageal wall depend on the duration of the existence of achalasia cardia. At the stage of clinical manifestations, there is a narrowing of the cardia and expansion of the esophageal lumen, its elongation and S-shaped deformation, coarsening of the mucous membrane and smoothing of the folding of the esophagus. Microscopic changes in achalasia cardia are represented by hypertrophy of smooth muscle fibers, overgrowth in the esophageal wall of connective tissue, pronounced changes in the intermuscular nerve plexuses.
Classification
According to the severity of esophageal dysfunction, there are stages of compensation, decompensation and sharp decompensation. Among the many proposed classification options, the following staging option is of the greatest clinical interest:
- Stage I. It is characterized by intermittent spasm of the cardiac department. There are no macroscopic changes (stenosis of the cardia and suprastenotic expansion of the esophageal lumen).
- Stage II. The spasm of the cardia is stable, there is a slight expansion of the esophagus.
- Stage III. Cicatricial deformation of the muscular layer of the cardia and a sharp suprastenotic expansion of the esophagus are revealed.
- Stage IV. It proceeds with pronounced stenosis of the cardiac department and significant dilation of the esophagus. It is characterized by the phenomena of esophagitis with ulceration and necrosis of the mucosa, periesophagitis, fibrous mediastinitis.
In accordance with the radiological signs, there are two types of achalasia cardia. The first type of pathology is characterized by moderate narrowing of the distal segment of the esophagus, simultaneous hypertrophy and dystrophy of its circular muscles. Dilation of the esophagus is moderately pronounced, the expansion area has a cylindrical or oval shape. Achalasia of the first type of cardia occurs in 59.2% of patients.
The second type of achalasia cardia is indicated with a significant narrowing of the distal part of the esophagus, atrophy of its muscular membrane and partial replacement of muscle fibers with connective tissue. There is a pronounced (up to 16-18 cm) expansion of the suprastenotic esophagus and its S-shaped deformation. Achalasia cardia of the first type may eventually progress to the second type. Knowledge of the type of achalasia cardia allows gastroenterologists to foresee possible difficulties during pneumocardiodilation.
Symptoms of achalasia cardia
Clinical manifestations of pathology are dysphagia, regurgitation and chest pains. Dysphagia is characterized by difficulty swallowing food. In some cases, a violation of the act of swallowing develops simultaneously and proceeds stably; usually dysphagia is preceded by influenza or another viral disease, stress. In some patients, dysphagia is initially episodic (for example, with hasty eating), then becomes more regular, making it difficult to pass both dense and liquid food.
Dysphagia can be selective and occur when eating only a certain type of food. Adapting to the swallowing disorder, patients can independently find ways to regulate the passage of food masses – to hold their breath, swallow air, drink food with water, etc. Sometimes, with this disease, paradoxical dysphagia develops, in which the passage of liquid food is more difficult than solid food.
Regurgitation develops as a result of the reverse casting of food masses into the oral cavity with the contraction of the muscles of the esophagus. The severity of regurgitation may be in the nature of a small regurgitation or esophageal vomiting, when copious regurgitation develops with a “full mouth”. Regurgitation can be periodic (for example, during a meal, simultaneously with dysphagia), occur immediately after a meal or 2-3 hours after a meal. Less often, food abandonment may occur in a dream (the so-called night regurgitation): at the same time, food often enters the respiratory tract, which is accompanied by a “night cough”. A slight regurgitation is characteristic of the I – II stage, esophageal vomiting – for the III – IV stages, when there is an overflow and overstretching of the esophagus.
Achalasia cardia may cause pain on an empty stomach or during ingestion when swallowing. Pain is localized behind the sternum, often radiating into the jaw, neck, between the shoulder blades. If at the I – II stage, the pain is caused by muscle spasm, then at the III – IV stage – by developing esophagitis. For achalasia of cardia, periodic paroxysmal pains are typical – esophagodynic crises that can develop against the background of excitement, physical activity, at night and last from several minutes to one hour. A painful attack sometimes passes on its own after vomiting or the passage of food masses into the stomach; in other cases, it is stopped with the help of antispasmodics.
Achalasia cardia complications
Violation of the passage of food and constant regurgitation lead to weight loss, disability, and a decrease in social activity. Against the background of characteristic symptoms, patients develop neurosis-like and affective states. Often, patients are treated for a long time and unsuccessfully by a neurologist for these disorders. Meanwhile, neurogenic disorders almost always regress after the treatment of cardia achalasia.
Diagnostics
In addition to typical complaints and physical examination data, the results of instrumental studies are extremely important in the diagnosis of achalasia cardia. Examination of a patient with suspected achalasia cardia begins with a chest X-ray. If the shadow of an enlarged esophagus with a liquid level is detected on the radiograph, an esophageal radiography with a preliminary intake of barium suspension is shown. The X-ray picture is characterized by a narrowing of the terminal part of the esophagus and the expansion of the overlying area, the S-shaped shape of the organ.
With the help of esophagoscopy, the stage and type of achalasia cardia, the presence and severity of esophagitis are clarified. To exclude esophageal cancer, an endoscopic biopsy is performed, followed by a morphological examination of the biopsy. To assess the contractile function of the esophagus and the tone of the cardiac sphincter, esophageal manometry is performed, recording intraesophageal pressure and peristalsis. A typical manometric sign of achalasia is the absence of a reflex of opening the cardia when swallowing.
A valuable diagnostic criterion for cardia achalasia is the results of pharmacological tests with carbacholine or acetylcholine: when they are administered, non-impulsive disorderly muscle contractions occur in the thoracic esophagus and an increase in the tone of the lower esophageal sphincter, which indicates denervation hypersensitivity of the organ. Differential diagnosis of achalasia cardia is carried out with benign tumors of the esophagus, esophageal diverticula, cardioesophageal cancer, esophageal strictures.
Treatment
Treatment involves the elimination of cardiospasm and can be carried out using conservative or surgical methods, sometimes drug therapy. A conservative method of eliminating achalasia cardia is pneumocardiodilation – balloon expansion of the cardiac sphincter, which is carried out in stages, with cylinders of different diameters with a sequential increase in pressure. With the help of cardiodilation, overstretching of the esophageal sphincter and a decrease in its tone are achieved. Complications of balloon dilation can be cracks and ruptures of the esophagus, the development of reflux esophagitis and cicatricial strictures of the cardiac sphincter.
A stable result of treatment of achalasia cardia is achieved after surgical intervention – esophagocardiomyotomy – dissection of the cardia followed by plastic surgery (fundoplication). The operation is indicated with a combination of achalasia cardia with a hernia of the esophageal orifice of the diaphragm, diverticula of the esophagus, cancer of the cardiac part of the stomach, failure of instrumental dilation of the esophagus, its ruptures.
If disease is combined with duodenal ulcer, selective proximal vagotomy is additionally indicated. In the presence of severe peptic erosive-ulcerative reflux esophagitis and pronounced atony of the esophagus, proximal resection of the stomach and abdominal part of the esophagus is performed with the imposition of invagination esophagogastroanastomosis and pyloroplasty.
Drug therapy plays an auxiliary role and is aimed at prolonging remission. For this purpose, it is advisable to prescribe antidophaminergic drugs (metoclopramide), antispasmodics, small tranquilizers, calcium antagonists, nitrates. In recent years, the management of botulinum toxin has been used to treat achalasia cardia. Important points are compliance with a sparing diet and diet, normalization of the emotional background, exclusion of overstrain.
Prognosis and prevention
The course of achalasia cardia is slowly progressing. Untimely treatment of pathology is fraught with bleeding, perforation of the esophageal wall, the development of mediastinitis, general exhaustion. Achalasia cardia increases the risk of esophageal cancer. After pneumocardiodilation, a relapse in 6-12 months is not excluded. The best prognostic results are associated with the absence of irreversible changes in esophageal motility and early surgical treatment. Patients are shown dispensary observation of a gastroenterologist with the necessary diagnostic procedures.
Literature
- Achalasia cardia in a premature infant. Shettihalli N, Venugopalan V, Ives NK, Lakhoo K. BMJ Case Rep. 2010 link
- Esophageal achalasia of unknown etiology in infants. Upadhyaya VD, Gangopadhyaya AN, Gupta DK, Sharma SP, Kumar V, Gopal SC. World J Pediatr. 2008 Feb;4(1):63-5. link
- Achalasia of the cardia in children: a worldwide survey. Myers NA, Jolley SG, Taylor R. J Pediatr Surg. 1994 Oct;29(10):1375-9. link
- Esophageal achalasia in children. Fernández Eire P, Queizan de la Fuente A, Mariño Espuelas JM, González Utrilla J. Cir Pediatr. 1991 Jul;4(3):125-9. link
- Family case of achalasia cardia: case report and review of literature. Evsyutina YV, Trukhmanov AS, Ivashkin VT. World J Gastroenterol. 2014 Jan 28;20(4):1114-8. link