Achalasia of the esophagus in children: causes, symptoms, diagnosis, treatment

Achalasia of the esophagus (cardiospasm) is a primary abnormality of the esophagus motor function, characterized by an increase in the tone of the lower esophageal sphincter (NPS), which leads to a violation of its relaxation and a decrease in the peristalsis of the esophagus.

ICD-10 code

K.22.0. Achalasia of the esophagus.

Epidemiology of Achalasia of the Esophagus

The prevalence of achalasia is on average 1 per 10 000, but only 5% of the disease achalasia cardia manifests itself at the age of 15 years.

[1], [2], [3], [4], [5], [6], [7],

What causes achalasia of the esophagus in children?

In the pathogenesis of achalasia, the role of genetic, neurogenic, hormonal and infectious causes is assumed. The possible involvement of genetic factors is indicated by cases of achalasia in siblings, including monozygotic twins. However, population studies have not confirmed the vertical family mode of transmission. In the development of achalasia, the involvement of Herpes zoster and autoimmune mechanisms is possible . In South America, achalasia is considered one of the symptoms of Chagas disease caused by Trypanosoma cruzi. In most cases, the etiology of achalasia can not be established.

See also: Causes of achalasia of cardia

Motor disorders in achalasia are associated with impaired function of postganglionic inhibitory neurons, providing relaxation of the NPS by releasing the vasoactive intestinal polypeptide (VIP) and nitric oxide. As ahalasia develops, there is a degeneration and a sharp decrease in the number of ganglions of the intermuscular plexus in the distal segment of the esophagus. In some cases, inflammatory infiltration of the intermuscular plexus and pronounced perineural fibrosis are described, accompanied by degenerative changes in the smooth musculature of the esophagus and interstitial Cajal-Retzius cells. Violations of motor function in achalasia were noted not only in the esophagus, but also in the stomach, intestine and gallbladder.

Symptoms of achalasia of the esophagus in children

Achalasia of the esophagus in children is much less common than in adults, can occur at any age, often after 5 years. The first symptoms do not attract the attention of the doctor, the diagnosis is set with a delay. S. Nurko published a meta-analysis of clinical symptoms of achalasia in 475 children in 2000: the most frequent symptoms were vomiting during or immediately after eating (80%) and dysphagia (76%).

See also:  Symptoms of achalasia of the cardia

At an early age during feeding, vomiting occurs with unalloyed milk without any admixture of gastric contents, the child "chokes" because the peristalsis of the lower segment of the esophagus is not accompanied by the opening of a cardia. After eating or during sleep, regurgitation, night cough, progressive dysphagia are possible. Patients feel like food passes through the esophagus, complain of pain behind the breastbone, night regurgitation, frequent bronchitis, pneumonia. Chronic malnutrition can lead to a lag in physical development, the progression of anemia.

Diagnosis of oesophagitis in children

The radiograph of the thoracic and abdominal cavity shows a significant widening of the mediastinum and a horizontal level of the fluid with air in the esophagus. In the stomach, there is no gas bubble.

Read also: Diagnosis of achalasia of cardia

In radiopaque studies, the barium suspension is delayed over the narrowed cardia, creating a picture of the "overturned candle flame", the "radish tail", then the barium enters the stomach. The esophagus can be considerably expanded, sometimes acquiring an S-shape.

Endoscopy is necessary to exclude tumors and other organic causes of stenosis. With achalasia, the esophagus is enlarged, remains of food or turbid fluid are visible above the narrowed cardia, but under the light pressure of the endoscope it is always possible to hold the apparatus in the stomach.

Manometry allows you to more accurately assess the nature of motor disorders of the esophagus and tone of the lower esophageal sphincter. Achalasia is characterized by:

• an increase in the tone of the lower esophageal sphincter is approximately twice (in the norm 25-30 mm Hg), sometimes the pressure does not exceed the upper limit of the norm;
• the absence of peristalsis of the esophagus along the entire length, sometimes only low-amplitude shortenings are retained;
• incomplete relaxation of the lower esophageal sphincter (normal relaxation is 100%, with achalasia not exceeding 30%);
• The pressure in the esophagus is higher than the pressure in the fundus of the stomach by an average of 6-8 mm Hg.

Radioisotope scintigraphy with Tc makes it possible to evaluate the features of passage through the esophagus of solid or liquid food with an isotope label. The study can be useful in the differential diagnosis of achalasia and secondary disorders of the esophageal peristalsis (for example, in scleroderma).

[8], [9], [10], [11], [12], [13], [14]

Differential picture of achalasia of cardia

Achalasia should be distinguished from diseases accompanied by esophageal obstruction (congenital esophagus stenosis, mediastinal cysts or tumors, vascular malformations, esophageal stenosis against the background of severe gastroesophageal reflux disease and Barrett's esophagus).

[15], [16], [17], [18], [19], [20]

Treatment of achalasia of the esophagus cardia in children

Conservative treatment of achalasia of cardia

Since the etiology of achalasia is unknown, treatment of the disease is aimed at eliminating symptoms and reducing the functional obstruction of the lower esophageal sphincter. Currently, it is believed that only nitrates and blockers of slow calcium channels have a proven clinical effect.

Nitrates cause a relaxation of smooth muscles, including the lower esophageal sphincter. The greatest clinical efficacy is isosorbide dinitrate (nitrosorbide) in a dose of 5-10 mg per day. When evaluating the data of esophagomanometry, it is established that the drug lowers the tone of the lower esophageal sphincter by 30-65%, resulting in the alleviation of the state of 53-87% of patients. However, with long-term admission, the effectiveness of treatment is reduced, side effects occur (most often headache).

Read also:  Treatment of achalasia of cardia

Blockers of slow calcium channels disrupt the activation of smooth muscle contractions. In clinical studies, nifedipine at a dose of 10-20 mg per day reduces the tone of the lower esophageal sphincter in patients with achalasia, speeding transit through the esophagus. Against the background of long-term treatment (6-18 months), the drug effectively eliminates symptoms of the disease in 2/3 of patients, mostly with a mild form of the disease. Side effects in the form of veins, heat and general hypotension were rare and were expressed only at the beginning of the course of treatment. Studies in children have not been conducted, and therefore the advisability of long-term (multi-year) drug therapy is controversial.

Several studies have shown that injections of botulinum toxin into the lower esophageal sphincter reduce its tone. However, with dynamic observation, it turned out that repeated injections of the drug are necessary to maintain the effect, and the response to treatment decreases with time. These results do not allow to consider injections of botulinum toxin as a method of choice in children.

Pneumatic balloon cardiac dilation is one of the most effective methods of treating achalasia; experience in children confirms the effectiveness of the technique in about 60% of cases. Taking into account the relative simplicity and effectiveness, the method has found wide application in adults and children as the main method of treating achalasia.

[21], [22], [23], [24], [25], [26], [27], [28],

Surgical treatment of achalasia of cardia

Myotomy is indicated if conservative therapy is ineffective. A promising method of treatment is laparoscopic myotomy - an alternative to pneumatic balloon cardiac dilation.