achalasia in children
Introduction
Introduction to achalasia in children Esophagealachalasia (also known as esophagealachalasia) is a esophageal functional disorder caused by neuromuscular dysfunction of the esophagus. Its main features are the lack of peristalsis of the esophagus, the lower esophageal sphincter (LES) high pressure and the relaxation response to swallowing action. Clinical manifestations include dysphagia, post-sternal pain, food reflux, and cough and lung infections caused by food reflux due to inhalation of the trachea. basic knowledge The proportion of illness: 0.0002% Susceptible people: children Mode of infection: non-infectious Complications: aspiration pneumonia, bronchiectasis, lung abscess
Cause
Causes of achalasia in children
The cause of achalasia has not been known so far. It is generally thought to be caused by neuromuscular dysfunction. Its pathogenesis is associated with degeneration, reduction or deficiency of Auerbach ganglion cells and defects in parasympathetic distribution in the esophageal muscle layer. At the same time of ganglion cell degeneration, it is often accompanied by inflammation of lymphocyte infiltration, and the cause may be related to infection and immune factors.
Degeneration of plexus ganglion cells leads to primary achalasia. The peristalsis and tension of the esophageal wall are weakened, the end of the esophageal sphincter can not relax, and the food stays in the esophageal lumen, gradually leading to dilatation, elongation and flexion of the esophagus. Food retention can be secondary to esophagitis and ulcers, on the basis of which cancer can occur, the cancer rate is 2% to 7%.
Prevention
Prevention of achalasia in children
The cause of this disease is not yet clear, so there are no reliable preventive measures. Eat small meals, eat and chew, avoid overheating and irritating diet. Psychotherapy and external agents can be given to people with mental stress. Some patients use Valsalva action to promote food from the esophagus into the stomach and relieve post-sternal discomfort. Sublingual nitroglycerin can relieve esophageal spasm, such as empty esophageal emptying. , also known as esophagealachalasia, giant esophagus, is the number of ganglion cells in the esophageal wall plexus, or even disappear, can affect the entire thoracic esophagus, but the most obvious middle and lower esophagus.
Complication
Complications of achalasia in children Complications, aspiration pneumonia, bronchiectasis, lung abscess
Can be complicated by the following symptoms:
First, respiratory complications:
1. Incidence rate:
It occurs in 10% of patients and is more pronounced in children.
2, the cause:
Aspiration pneumonia, bronchiectasis, lung abscess and pulmonary fibrosis are most common in reflux vomiting.
3. Related inspections:
Atypical mycobacteria combined with oil retention in the esophagus can induce chronic lung changes, similar to clinical and X-ray tuberculosis. Find acid-fast bacteria in the sputum, which may be atypical mycobacteria. Don't mistake the tuberculosis.
Second, cancer:
1. Incidence rate:
It is reported that 2% to 7% of patients can be combined with esophageal cancer, especially those with a disease course of more than 10 years, obvious esophageal dilation, and severe retention.
2, the main reason:
Food retention is caused by chronic inflammatory stimuli of esophagitis. Esophageal muscle incision or expansion can not prevent the occurrence of cancer, the diagnosis is often delayed, because the patient's digestive tract symptoms are often mistaken for achalasia, until the cancer grows to a larger volume, the esophagus is blocked and enlarged. .
3. Clinical manifestations:
Weight loss, dysphagia from intermittent to progressive, reflux vomiting is found when bloody sex or anemia occurs.
4. Related inspections:
It can be used for X-ray examination of barium meal, endoscopic biopsy and cytology brushing.
Third, esophagitis:
1, the cause of the disease:
Due to ecchymosis in the esophageal food retention, endoscopic examination can be seen with esophagitis and mucosal ulcers caused by it, ulcers can occur bleeding, a few spontaneous perforation, esophageal tracheal fistula.
Patients with debilitating or who have received antibiotic treatment or neutropenia may have a Candida infection.
2. Related inspections:
There are white spots on the inflammatory mucosa in the mirror. Specimen smear and biopsy can confirm the diagnosis.
3. Treatment:
Should first expand to relieve esophageal retention, the condition can not tolerate strong dilatation can be used to attract drainage to maintain esophageal emptying, while applying antibiotics.
Fourth, other complications:
Due to the dilatation of the esophagus of achalasia, the intraluminal tension is increased, and the complications of the supracondylar diverticulum are treated, which can be treated simultaneously with the treatment of achalasia. A few patients develop joint complications similar to rheumatoid arthritis, and the symptoms can be alleviated after treatment of achalasia.
Symptom
Children with achalasia symptoms common symptoms stomach pain, swallowing difficulties, stagnation, pediatric eating, easy to vomit
Dysphagia
Painless dysphagia is the most common symptom of the disease, accounting for 80%~95%. Symptoms of onset are more slow, but they can be more urgent. They can be mild at first, and only have a feeling of fullness after a meal. Dysphagia is often intermittent, often induced by mood swings, anger, anxiety, convulsions, or eating irritating foods such as cold and spicy. Sometimes it is difficult to swallow when the disease is early, when it is light and heavy, and later it is continuous. A small number of patients have difficulty in ingesting fluids compared to solid foods, and this sign distinguishes them from dysphagia caused by other esophageal strictures. But most patients swallow solids more difficult than liquids, or it is equally difficult to swallow solid and liquid foods.
2. Food reflux and vomiting
The incidence of food reflux and vomiting in patients with achalasia can reach 90%. As the difficulty of swallowing worsens, the esophagus expands further, and a considerable amount of the contents can remain in the esophagus for hours or days, and will flow back when the body position changes. Vomiting occurs more than 20 to 30 minutes after eating, and can vomit from the previous meal or overnight food. The contents from the esophagus are not in the stomach cavity, so there is no characteristic of vomit in the stomach, but a large amount of mucus and saliva can be mixed. In the case of concurrent esophagitis and esophageal ulcer, the reflux may contain blood.
Patients may have recurrent pneumonia, bronchitis, and even bronchiectasis or lung abscess due to food reflux and aspiration.
3. Pain
About 40% to 90% of patients with achalasia have symptoms of pain, varying in nature, and can be stuffy, burning, acupuncture, cut pain or cone pain. Most of the pain is in the back of the sternum and the upper abdomen; it can also be in the chest and back, right chest, right sternal border and left rib. Pain attacks sometimes resemble angina and can be relieved even after sublingual nitroglycerin tablets. The mechanism of pain may be due to strong contraction of the esophageal smooth muscle or food retention esophagitis. As the difficulty of swallowing gradually increases, the further expansion of the esophagus above the obstruction can gradually reduce the pain.
4. Weight loss
Weight loss is associated with difficulty in swallowing affecting food intake. For dysphagia, patients often take food, slow food, eat food or eat enough soup to wash the food, or stretch the chest and back after eating, deep breathing or suffocating to help swallow movement, make food Enter the stomach to ensure nutrient intake. However, those who have a long course of disease can still have weight loss, malnutrition and vitamin deficiency, while those with cachexia are rare.
5. Other
Patients with achalasia can often have anemia, occasionally bleeding caused by esophagitis. In later cases, the extremely dilated esophagus can compress the internal organs of the chest and produce dry cough, shortness of breath, cyanosis and hoarseness.
Examine
Examination of achalasia in children
First, blood routine:
Anemia can be found in peripheral blood, and there may be bloody infection, increased white blood cell count, and increased neutrophils in patients with aspiration pneumonia.
Second, X-ray inspection:
At the beginning of the disease, the chest radiograph can be normal. As the esophagus expands, a bulge in the right upper edge of the mediastinum can be seen in the posterior anterior chest radiograph. When the esophagus is highly dilated, extended, and curved, it can be seen that the mediastinum widens beyond the right edge of the heart and can sometimes be misdiagnosed as a mediastinal tumor. When a large amount of food and gas are trapped in the esophagus, the air bubbles in the stomach disappear and the liquid level is visible in the esophagus. In most cases, the disappearance of the gastric vesicle is seen.
Third, barium meal inspection:
Esophageal barium meal imaging is an important diagnostic test in cases of achalasia. Pay attention to the morphological and functional characteristics of the esophageal body and the distal sphincter.
It is often difficult to pass the cardia to the lower end of the esophagus, and it is shown as a funnel-shaped stenosis of a symmetrical, mucosal pattern of 1 to 3 cm in length. The upper esophagus exhibits different degrees of expansion, length and curvature, and no peristaltic waves. Such as hot drinks, sublingual nitroglycerin tablets or inhalation of isoamyl nitrite, every esophageal sinus relaxation; such as cold drinks, make the cardia more difficult to relax. Detained food debris can present a filling defect during barium meal angiography, so esophageal drainage and lavage should be performed before examination.
The main feature of the X-ray of achalasia is that the peristalsis of the esophageal body disappears, and the distal sphincter loses the relaxation reaction when swallowing, and the sputum stays at the gastroesophageal junction. The tube wall is smooth and the lumen is suddenly narrowed and changes like a beak.
Fourth, endoscopy:
Endoscopy has little help in the diagnosis of this disease, but it can be used for the differential diagnosis between this disease and esophageal and cardiac cancer.
The more severe the esophageal dilation, the more obvious mucosal inflammation. The mucosa in the lower part of the esophagus is obviously white, thick and rough, and there may be signs of "cracking". Severe cases are nodular changes, and there may be erosions and shallow ulcers. When there are nodules, erosions, ulcers, you should be alert to esophageal cancer.
Five, esophageal pressure measurement:
It can reflect the pathology of the esophagus from the pathophysiological point of view, can confirm or confirm the disease, and can be used as a quantitative indicator of drug efficacy, dilatation and evaluation of esophageal function after esophagotomy.
Sixth, esophageal emptying check:
Including the nuclear vegetarian tube passage time, esophageal tincture emptying index determination and drinking water test, etc., are helpful to determine the esophageal emptying function, but also to evaluate the efficacy of treatment on esophageal function.
Seven, methacholine test:
After subcutaneous injection of 5-10 mg of methacholine in normal subjects, there was no significant increase in the pressure of esophageal peristalsis. However, in patients with this disease, 1 to 2 minutes after injection, strong contraction of the esophagus can occur; the pressure in the esophagus increases suddenly, resulting in severe pain and vomiting.
Extreme expansion of the esophagus does not respond to the drug, resulting in a negative test result; gastric cancer involving the esophageal intermuscular plexus and some diffuse esophageal fistula, this test can also be positive. It can be seen that this test lacks specificity.
Diagnosis
Diagnosis and diagnosis of achalasia in children
Diagnosis can be based on medical history, clinical presentation, and related examinations.
Differential diagnosis
It should be differentiated from the following conditions:
First, pseudo achalasia:
1. Clinical manifestations:
People have symptoms of dysphagia, X-ray examination of the esophageal body is dilated, the distal sphincter can not relax, pressure measurement and X-ray examination are no peristaltic waves.
This occurs in the submucosal layer of the esophageal junction and the invasive lesion of the intestinal muscle plexus.
The most common cause is gastric cancer infiltration, other rare diseases such as lymphoma and amyloidosis, liver cancer can also find similar signs.
2. Related inspections:
There was no pre-expansion in the microscopy, and the instrument could not pass the instrument because the infiltrated lesion was stiff. In most cases, a biopsy can be diagnosed, and sometimes a probe is needed to confirm the diagnosis.
Second, no motility abnormalities:
1 Introduction:
Pedicure can cause a period of distal esophageal squirming and cause difficulty in diagnosis. Because of esophageal involvement, it often precedes skin manifestations.
2. Clinical manifestations:
Esophageal manometry showed that the proximal end of the esophagus was often unaffected, while the peristaltic wave of the esophageal body was minimal, and the distal sphincter was often weak, but the relaxation was normal.
3, a good crowd:
Abnormal motility can also be seen in the accompanying peripheral neuropathy, such as in patients with diabetes and multiple sclerosis.
Third, dysphagia after vagus nerve cutting:
1. Incidence rate:
Dysphagia can occur after the vagus nerve is cut by the chest or abdomen. Temporary dysphagia can occur in approximately 75% of patients after high-selective vagus nerve ablation. In most cases, the symptoms can gradually disappear after 6 weeks.
2. Related inspections:
In the line and pressure test, it can be seen that the distal esophageal sphincter can not relax and occasionally have no peristalsis, but rarely need expansion and surgical treatment. According to the medical history can be identified.
Fourth, elderly esophagitis:
1, the cause:
The disorder of esophageal motor function in the elderly is due to the degeneration of the organ on the esophagus.
2. Clinical manifestations:
Most elderly people find that esophageal dysfunction is poor in the pressure test, both primary and secondary peristalsis are impeded, and there is often no peristaltic contraction after swallowing or spontaneous. The number of relaxations of the lower esophageal sphincter is reduced or absent, but the resting pressure in the esophagus does not increase.
Fifth, Chagas disease:
1 Introduction:
Mad et al (1999) reported a case of esophageal leiomyosarcoma associated with Chagas's esophagus. The patient developed hypopharyngeal infarction due to the slow-developing Chagas esophagus at the age of 19, and received cardiomyotomy at the age of 49. Esophageal leiomyosarcoma was found at the age of 6 years due to aggravation of swallowing and surgical resection. Lung and liver metastases occurred in 14 months and died after 6 months.
2. Clinical manifestations:
There can be a giant esophagus, a parasitic disease that is prevalent in South America, and affects the whole body.
Its clinical manifestations are not easily distinguished from achalasia. The gastrointestinal muscles are degraded by secondary parasitic infections, which are similar to primary achalasia in physiological, pharmacological and therapeutic responses.
In addition to esophageal lesions, Chagas has other visceral changes. Before the diagnosis, it must be determined that the patient has lived in South America or South Africa, and the past infection history of trypanosomiasis can be determined by fluorescence immunoassay and complement fixation test.
6. Esophageal and cardiac cancer:
1, the cause:
The stenosis caused by the cardia cancer is caused by the infiltration of the cancer tissue into the wall.
2. Clinical manifestations:
The membrane is damaged, and ulcers, lumps, etc. can be formed. The lesions are mainly on one side of the tube wall. The stenosis is poor in passive dilatation. The endoscope has a large resistance. If the stenosis is severe, it often fails to pass, and the strong insertion lens is easy to cause perforation.
3. Related inspections:
The achalasia is that the LES can not relax, only the lower end of the esophagus is closed, the esophageal mucosa has no obvious abnormalities, and the lower end of the esophagus and the cardia wall are well dilated. Therefore, the endoscope can smoothly enter the gastric cavity by a little resistance. .
