The cerebellar tonsils are displaced below the foramen magnum. What does low location of the cerebellar tonsils mean for the body?

Dystopia is the descent of the cerebellar tonsils into the foramen magnum and the spinal canal. This anomaly is also called grade I Chiari malformation.

Usually this diagnosis does not cause any significant disturbances or anxiety to the patient. It often appears between the ages of 30 and 40. During research it is a random “find”.

Causes:

In most cases this is congenital pathology, manifested by a discrepancy between the sizes of the brain and the foramen magnum. Cerebellar dystopia can also be secondary – with frequent and traumatic lumbar punctures.

Symptoms:

The symptom of dystopia rarely manifests itself clinically. However, it is possible that neurological signs– pain in the neck when straining, coughing. It is “shooting” and paroxysmal in nature. Headaches and dizziness occur. The greater the phenomenon of dystopia (varies from a couple of millimeters to several centimeters), the more pronounced the disorders are. With severe prolapse, syringomyelia may occur (enlargement of the canal spinal cord and the formation of cavities around it).

Treatment and measures when symptoms are detected:

If neurological symptoms are negative, treatment is usually not required. Patients with minimal or no symptoms are still kept under the supervision of a neurologist to monitor the progression of the disease.

If neurological symptoms still exist, but in minor manifestations, conservative treatment is possible. Pain relief vascular drugs and non-steroidal anti-inflammatory drugs, muscle relaxants. Recommendations on lifestyle and routine are also needed.

Surgical intervention is the only efficient look treatments for patients with extensive disorders.

Surgical treatment involves expansion cranial fossa and plastic surgery of the dura mater.

Indications for surgery:

• Excruciating headaches that cannot be relieved by medications.
• An increase in cerebral and focal manifestations that can lead to disability.

The main diagnostic technique is magnetic resonance imaging. Computed tomography and x-ray examination will not give a clear picture of the disease.

If an MRI revealed dystopia of the cerebellar tonsils, you should not worry about this. In most cases, the anomaly is asymptomatic and does not require treatment.

Arnold-Chiari malformation type I (Q07.0) is congenital anomaly development of the brain, characterized by displacement of the formations of the posterior cranial fossa into the region of the spinal canal below the level of the foramen magnum.

Prevalence: on average 3.3-8.2 per 100 thousand people. The onset of clinical symptoms is observed in adulthood or adolescence. In 50-75% of cases there is a combination with syringomyelia, in 10% - with hydrocephalus.

Sometimes this pathology has no clinical symptoms, becomes an x-ray finding.

Clinical picture

The main symptoms of Arnold-Chiari malformation type I are as follows:

• pain in the back of the head, may intensify during coughing, straining (35-50%),
• neck pain (15%),
• weakness, numbness in the hands (60%),
• instability when walking (40%),
• double vision, speech impairment, difficulty swallowing - 5-25%,
• noise in the head (15%).

An objective examination of the patient can reveal cerebral syndrome, platybasia (50%), nystagmus, diplopia, dysphagia, dysarthria, hypoesthesia in the neck and arms, spastic paresis in the arms, revitalization of deep reflexes, ataxia.

Diagnosis of Arnold-Chiari malformation

• Magnetic resonance imaging of the brain, cervical spinal cord.

• Consultation with a neuro-ophthalmologist ( stagnant discs optic nerves).

Differential diagnosis:

• Other anomalies of brain development, craniovertebral junction.
• Hereditary ataxias.
• Demyelinating diseases.

Treatment of Arnold-Chiari malformation

Treatment is symptomatic. Prescribed only after confirmation of the diagnosis by a medical specialist. Rise clinical symptoms- indication for suboccipital decompression and bypass surgery.

Essential drugs

There are contraindications. Specialist consultation is required.

• Ketoprofen (non-steroidal analgesic). Dosage regimen: orally 50 mg situationally, no more than 2 times a week.
• (non-steroidal anti-inflammatory drug). Dosage regimen: for relief of acute pain syndrome, the recommended oral dose is 8-16 mg/day. in 2-3 doses. The maximum daily dose is 16 mg. The tablets are taken before meals with a glass of water.
• (painkiller, anti-inflammatory). Dosage regimen: orally, with meals, 10-20 mg/day. in 1 or 2 doses. If necessary, you can temporarily increase the dose to 30 mg/day, once or in divided doses, or reduce it to 10 mg/day.
• (central acting muscle relaxant). Dosage regimen: the initial oral dose is 6 mg (1 capsule). If necessary, the daily dose can be gradually increased by 6 mg (1 capsule) at intervals of 3-7 days. For most patients, the optimal dose is 12 mg per day (2 capsules); V in rare cases may require an increase daily dose up to 24 mg.
• (central acting muscle relaxant). Dosage regimen: orally, after meals, without chewing, with a small amount of water, starting with 50 mg 2-3 times a day, gradually increasing the dose to 150 mg 2-3 times a day.

(Arnold-Chiari malformation) is a disease in which the brain structures located in the posterior cranial fossa are lowered in a caudal direction and exit through the foramen magnum. Depending on the type, Chiari malformation may manifest as headache in the back of the head, pain in the cervical spine, dizziness, nystagmus, fainting, dysarthria, cerebellar ataxia, laryngeal paresis, hearing loss and tinnitus, visual impairment, dysphagia, respiratory apnea, stridor, sensitivity disorders, muscle wasting and tetraparesis. Chiari malformation is diagnosed by performing an MRI of the brain, cervical and thoracic spine. Chiari malformation, accompanied by persistent pain or neurological deficit, is subject to surgical treatment (posterior fossa decompression or shunt surgery).

General information

In the area where the skull connects with spinal column There is the foramen magnum, at the level of which the brain stem passes into the spinal cord. Above this hole is the posterior cranial fossa. It contains the pons, medulla oblongata and cerebellum. Chiari malformation is associated with the exit of part of the anatomical structures of the posterior cranial fossa into the lumen of the foramen magnum. In this case, compression of the structures of the medulla oblongata and spinal cord located in this area occurs, as well as a violation of the outflow of cerebrospinal fluid from the brain, leading to hydrocephalus. Together with platybasia, assimilation of the atlas, etc., Chiari malformation is a congenital malformation of the craniovertebral junction.

Chiari malformation occurs, according to various sources, in 3-8 people per 100 thousand population. Depending on the type, Chiari malformation may be diagnosed in the first days after the birth of a child or become an unexpected finding in an adult patient. In 80% of cases, Chiari malformation is combined with syringomyelia.

Causes of Chiari malformation

Until now, Chiari malformation remains a disease about the etiology of which there is no consensus in neurology. A number of authors believe that Chiari malformation is associated with a reduced size of the posterior cranial fossa, leading to the fact that as the structures located in it grow, they begin to exit through the foramen magnum. Other researchers suggest that Chiari malformation develops as a result of an increased size of the brain, which at the same time pushes the contents of the posterior cranial fossa through the foramen magnum.

Provoke the transition from a mildly expressed anomaly to a pronounced one clinical form may hydrocephalus, in which the total volume of the brain increases due to enlargement of the ventricles. Since the Chiari malformation, along with dysplasia of the bone structures of the craniovertebral junction, is accompanied by underdevelopment of the ligamentous apparatus of this area, any traumatic brain injury can lead to aggravation of the herniation of the cerebellar tonsils into the foramen magnum with manifestation clinical picture diseases.

Classification of Chiari malformation

Chiari malformation is divided into 4 types:

It is characterized by prolapse of the cerebellar tonsils below the foramen magnum. It usually appears in adolescence or adulthood. It is often accompanied by hydromyelia, an accumulation of cerebrospinal fluid in the central canal of the spinal cord.

Appears in the first days after birth. In addition to the cerebellar tonsils, in this pathology, the cerebellar vermis, medulla oblongata, and fourth ventricle also exit through the foramen magnum. Chiari malformation type II is much more often combined with hydromyelia than type I, and in the vast majority of cases is associated with myelomeningocele, a congenital spina bifida.

It differs in that the cerebellum and medulla oblongata, which descend through the foramen magnum, are located in the meningocele of the cervical occipital region.

It consists of hypoplasia (underdevelopment) of the cerebellum and is not accompanied by its displacement in the caudal direction. Some authors attribute this anomaly to Dandy-Walker syndrome, in which cerebellar hypoplasia is combined with the presence of congenital cysts of the posterior cranial fossa and hydrocephalus.

Chiari II and Chiari III malformations are often observed in combination with other dysplasias nervous system: heterotopia of the cerebral cortex, polymicrogyria, anomalies of the corpus callosum, cysts of the foramen of Mozhandi, kinking of the Sylvian aqueduct, hypoplasia of the subcortical structures, tentorium and falx of the cerebellum.

Symptoms of Chiari malformation

Most often in clinical practice Chiari malformation type I occurs. It is manifested by liquor-hypertension, cerebellobulbar and syringomyelic syndromes, as well as damage to the cranial nerves. Typically, Chiari I malformation manifests itself during puberty or in adulthood.

CSF hypertensive syndrome, which is accompanied by Chiari I malformation, is characterized by headache in the back of the head and cervical region, aggravated by sneezing, coughing, straining or straining the neck muscles. Vomiting may occur, regardless of food intake and its nature. When examining patients with Chiari malformation, increased tone neck muscles. Among cerebellar disorders speech impairment (dysarthria), nystagmus, and cerebellar ataxia are observed.

Damage to the brain stem, the nuclei of the cranial nerves and their roots located in it is manifested by decreased visual acuity, diplopia, swallowing disorder, hearing loss of the type cochlear neuritis, systemic dizziness with the illusion of rotation of surrounding objects, tinnitus, sleep apnea syndrome, repeated short-term losses consciousness, orthostatic collapse. Patients who have a Chiari malformation report increased dizziness and tinnitus when turning their head. Turning the head in such patients can provoke fainting. Atrophic changes in half of the tongue and laryngeal paresis may be observed, accompanied by hoarseness and difficulty breathing. Possible tetraparesis with a large decrease in muscle strength in upper limbs than in the lower ones.

In cases where Chiari I malformation is combined with syringomyelia, syringomyelic syndrome is observed: dissociated sensory disturbances, numbness, muscle wasting, pelvic disorders, neuroarthropathy, disappearance of abdominal reflexes. At the same time, some authors point out the discrepancy between the size and location of the syringomyelic cyst, the prevalence of sensitivity disorders, the severity of paresis and muscle wasting.

Chiari II and Chiari III malformations have similar clinical manifestations, which become noticeable from the first minutes of a child’s life. Chiari II malformation is accompanied by noisy breathing (congenital stridor), periods of short-term respiratory arrest, bilateral neuropathic paresis of the larynx, and impaired swallowing with reflux of liquid food into the nose. In newborns, Chiari II malformation is also manifested by nystagmus, increased muscle tone in the upper extremities, cyanosis skin occurring during feeding. Movement disorders can be expressed in varying degrees and progress to tetraplegia. Chiari III malformation has a more severe course and is often a developmental disorder of the fetus that is incompatible with life.

Diagnosis of Chiari malformation

Neurological examination and standard checklist neurological examinations(EEG, Echo-EG, REG) do not provide specific data to establish the diagnosis of Chiari malformation. As a rule, they reveal only signs of a significant increase intracranial pressure, i.e. hydrocephalus. X-rays of the skull reveal only the bone abnormalities that may accompany Chiari malformation. Therefore, before the introduction of tomographic research methods into neurological practice, diagnosing this disease presented great difficulties for a neurologist. Now doctors have the opportunity to give such patients an accurate diagnosis.

It should be noted that MSCT and CT of the brain, with good visualization of the bone structures of the craniovertebral junction, do not allow a sufficiently accurate assessment of the soft tissue formations of the posterior cranial fossa. Therefore, the only reliable method for diagnosing Chiari malformation today is magnetic resonance imaging. Its implementation requires immobility of the patient, so in young children it is performed in a state of medicated sleep. In addition to MRI of the brain, to identify meningoceles and syringomyelic cysts, it is also necessary to conduct MRI of the spine, especially its cervical and thoracic regions. At the same time, MRI studies should be aimed not only at diagnosing Chiari malformation, but also at searching for other anomalies of the development of the nervous system, which are often combined with it.

Treatment of Chiari malformation

Asymptomatic Chiari malformation does not require treatment. In cases where Chiari malformation is manifested only by the presence of pain in the neck and occipital region, conservative therapy is carried out, including analgesic, anti-inflammatory and muscle relaxant drugs. If Chiari malformation is accompanied by neurological disorders (paresis, disorders of sensitivity and muscle tone, disorders of the cranial nerves, etc.) or pain that is not amenable to conservative treatment, then surgical treatment is indicated.

The most common treatment for Chiari malformation is craniovertebral decompression. The operation involves widening the foramen magnum by removing part occipital bone; elimination of compression of the trunk and spinal cord due to resection of the cerebellar tonsils and the posterior halves of the first two cervical vertebrae; normalization of cerebrospinal fluid circulation by suturing into solid meninges patches made of artificial materials or allograft. In some cases, Chiari malformation is treated with shunt surgery to drain cerebrospinal fluid from the dilated central canal of the spinal cord. Cerebrospinal fluid may drain into the chest or abdominal cavity(lumboperitoneal drainage).

Prediction of Chiari malformation

The type to which the Chiari malformation belongs has important prognostic significance. In some cases, Chiari I malformation may remain asymptomatic throughout the patient's life. Chiari III malformation in most cases leads to fatal outcome. When neurological symptoms Chiari I malformation, as well as Chiari II malformation great importance has timely implementation surgical treatment, since the resulting neurological deficit is poorly restored even after a successful operation. According to various data, the effectiveness of surgical craniovertebral decompression is 50-85%.

As you know, there are 6 tonsils in the human body. Each of them performs important function. Above the foramen magnum are the cerebellar tonsils, which is how they are usually located. Ectopic tonsils are the most common abnormality of the cerebellum, namely displacement or misplacement of the tonsils. Scientifically, such an anomaly is usually called the Arnold-Chiari anomaly.

Symptoms of the development of tonsil ectopia

The disease is characterized by the descent (unilateral or bilateral) of the cerebellar tonsils into the spinal canal through the foramen magnum. An important point in this case, it is that any symptoms appear only at thirty, and sometimes at forty years. If ectopia of the tonsils is not accompanied by any symptoms and is discovered incidentally as a result of other tests, then there is no need to treat it.

The question of drug treatment or surgical intervention arises only if the ectopia shows symptoms.

The causes of this disease have not yet been identified by medicine. What is known is that genetic inheritance has an influence. With ectopia of the tonsils, the following neurological syndromes may be observed. The patient is suffering

• for neck pain, especially when coughing or straining,
• headache.
• In some cases, a symptom of the disease is hearing loss,
• dizziness,
• noise in ears,
• stopping breathing during sleep,
• as well as periodic fainting.

With ectopia of the tonsils there is whole line possible violations, depending on the stage of its development.

Previously, medicine required more time and effort to diagnose ectopia of the tonsils, since x-rays and CT scan do not give a very clear picture. Now, using MRI, you can get a clear picture of the posterior cranial fossa, spinal cord, etc., which will show a violation of the location of the cerebellar tonsils.

Features of treatment of tonsil ectopia

People who have been diagnosed as a result of an "incidental" discovery should not worry. The disease will not affect your well-being or life expectancy in any way. If you feel some symptoms of ectopia of the tonsils, you should still consult a doctor, otherwise there may be consequences, for example, an abnormality of the spine.

Treatment of the disease in this case can be surgical and medicinal. During the operation, the volume of the cranial fossa is increased. If among the symptoms of tonsil ectopia you only have pain syndrome, then you can limit yourself to non-steroidal anti-inflammatory drugs, and in this case surgery is not necessary.

However, the following manifestations are possible:

• pain in the cervical-occipital region, aggravated by coughing and straining;
• headache;
• decreased visual acuity;
• nystagmus (oscillatory movements of the eyeballs);
• impaired swallowing (choking when eating, drinking);
• unsteadiness of gait, instability, frequent falls;
• movement coordination disorder: the patient’s movements are unclear, uncoordinated, and sweeping;
• violation of deep sensitivity (the sense of perception of one’s own body is impaired, for example, a person with eyes closed does not feel what position his hands are in);
• decreased pain and temperature sensitivity of the upper extremities;
• breathing problems (up to stopping) - in severe cases.

Forms

• Arnold-Chiari malformation 1type: descent of part of the cerebellum (its outgrowths - tonsils) below the plane of the foramen magnum with compression of the brain stem.
• Arnold-Chiari malformation 2type: In addition to the cerebellar tonsils, the medulla oblongata and midbrain (structures located just above) also descend into the foramen magnum. Often accompanied by non-fusion of the spinal arches, hydrocephalus (impaired outflow of cerebrospinal fluid from the cavities of the brain. Liquor is a fluid that provides nutrition and metabolism in the brain).
• Arnold-Chiari malformation 3type: with this form of anomaly, a herniation of the brain (encephalocele) is formed, emerging through a defect in the bones of the skull. Part of the cerebellum gets into this hernia, which is manifested by imbalance, unsteadiness of gait, nystagmus (rhythmic oscillatory movements of the eyes from side to side).
• Arnold-Chiari malformation type 4: this form is characterized by congenital underdevelopment of the cerebellum. Most often ends in death.

Causes

The reasons for the development of this anomaly are not reliably known.

It is possible that the risk of having a child with Arnold-Chiari malformation is increased by the following factors affecting the body of a pregnant woman:

• drug abuse during pregnancy;
• smoking and drinking alcohol during pregnancy;
• frequent viral colds, as well as rubella and others viral diseases during pregnancy.

TO congenital causes Abnormal development of the skull bones may be attributed to:

• too small posterior cranial fossa (which normally houses the cerebellum and occipital lobe brain);
• increase in the size of the foramen magnum.

Acquired causes may include:

• traumatic injuries to the child’s skull and brain due to birth trauma(during the passage of the birth canal);
• hydrodynamic impact of cerebrospinal fluid into the walls of the central canal of the spinal cord (cerebrospinal fluid is the cerebrospinal fluid that provides nutrition and metabolism in the brain): if the circulation of cerebrospinal fluid between the cavities (ventricles) of the brain and the spinal cord is disrupted, cerebrospinal fluid under pressure can damage the spinal cord, stretching its central channel.

LookMedBook reminds: the sooner you seek help from a specialist, the greater your chances of maintaining health and reducing the risk of complications:

Diagnostics

The diagnosis is made based on a neurological examination and the results of instrumental examination.

• Neurological examination: may reveal unsteadiness of gait, nystagmus (rhythmic oscillatory movements of the eyes from side to side), sensory disturbances, and swallowing.
• Analysis of complaints and medical history:
  • how long ago did complaints of headaches, sensory disturbances, unsteadiness of gait, paralysis (inability to move the limbs) appear?
  • whether the disorders were present at birth or developed later;
  • whether the child had a head or neck injury during childbirth.

• MRI (magnetic resonance imaging) of the brain and the cervical and thoracic spinal cord allows you to study the structure of the brain layer by layer to detect abnormalities in brain development.
• It is also possible to consult a therapist or neurosurgeon.

Treatment of Arnold-Chiari malformation

The volume and type of treatment depend on the severity of the disease.

• Conservative (non-surgical) treatment (if the main symptom of the disease is minor pain in the cervical-occipital region): painkillers, anti-inflammatory drugs, and muscle relaxants (drugs that relax muscles) are used for treatment.
• Surgical treatment (in the absence of effect from conservative therapy or the presence of severe neurological symptoms (for example: numbness, weakness in the limbs)). Purpose surgical treatment is to eliminate factors that contribute to compression of brain structures, as well as normalize the circulation of cerebrospinal fluid (normally it circulates freely between the ventricles of the brain and the central canal of the spinal cord).

Complications and consequences

• Rise intracranial hypertension, development of hypertensive-hydrocephalic syndrome (excessive accumulation of fluid in the cranial cavity).
• Breathing problems (even to the point of stopping).
• Congestive pneumonia (inflammation of lung tissue due to stagnation of blood in the lungs. Blood stagnation leads to malnutrition of the lungs and the active proliferation of microorganisms entering them) due to the fact that patients often become unable to move independently.

Prevention of Arnold-Chiari malformation

Due to insufficient data on the causes of the disease, preventive measures are reduced to rules healthy image mother's life during pregnancy:

• good nutrition (eating fresh vegetables and fruits, meat products, taking vitamins);
• quitting smoking and alcohol;
• application medicines only as prescribed by a doctor.

Additionally

Prerequisites for prolapsed tonsils are:

• reduction in the size of the posterior cranial fossa (in which the cerebellum and occipital lobe of the brain are normally located): in this case, the cerebellum does not have enough space and it seems to “push out” its bottom part from the cranial cavity;
• increase in the size of the foramen magnum: allows the cerebellar tonsils to exit the cranial cavity.

The descent of the cerebellar tonsils into the foramen magnum or cagal of the spinal cord is called dystopia. And sometimes this pathology is called Chiari malformation. As a rule, such a disease does not entail significant disorders or obvious symptoms and has no cause for concern for the patient. Often this pathology manifests itself after reaching 30–40 years of age. It is usually discovered during examinations for other reasons. Therefore, you should know about this disease so that it does not come as a surprise to the patient.

In order to understand what a low location of the cerebellar tonsils is, you need to clearly know clinical symptoms and how pathology is detected. And since this condition is accompanied by frequent headaches, you must first establish their cause, and then begin treatment. This disease is detected by MRI.

Reasons for appearance

Cerebellar dystopia, as a rule, is a congenital pathology. It occurs when an organ is displaced during the embryonic period. It occurs secondary only when frequent punctures are performed or with lumbar injuries. Other reasons for the appearance of this disease not found.

The tonsils of the cerebellum are very similar to those found in the larynx. IN normal position they are located above the BZO of the skull. And deviations in their development and position can lead to not only dystopia. The most common occurrence is prolapse of the cerebellar tonsils below the level of the skull.

Until now, Chiari disease is a pathology, the causes of which neurologists have not come to a common opinion. Some are of the opinion that this anomaly occurs when the size of the fossa behind the cranial outlet to the spinal canal decreases. This often leads to such consequences during the growth of tissues that are located in the box. They exit into the occipital exit canal. Other experts believe that the disease begins to develop due to an increase in the volume of brain tissue in the head. In this case, the brain begins to push the cerebellum and its tonsils through the posterior cranial fossa into the occipital cranial foramen.

Causes the progression of a pronounced anomaly and its transition to the “clinic”, such as hydrocephalus. At the same time, the overall size of the brain increases, especially the cerebellar tissue. Chiari pathology, together with the underdeveloped ligamentous apparatus of the brain, is accompanied by dysplasia of bone tissue. Therefore, any traumatic brain injury often leads to an increased decrease in the level of location of the tonsils and cerebellum.

Types of anomalies

There are such types of abnormal deviations as dystopia and Chiari malformation.

In turn, Chiari disease is divided into four different types:

1. Type I is characterized by the location of the tonsils below the level of the foramen magnum. This pathology is usually detected in adolescents and adults. It is often accompanied by an accumulation of spinal cerebrospinal fluid in the central canal, where the patient’s spinal cord is located.
2. Type II is characterized by manifestation immediately after birth. In addition, in addition to the tonsils, in the second type of pathology, the cerebellar vermis with part of the medulla oblongata and the ventricle emerge into the foramen of the occipital part. The second type of anomaly is much more often accompanied by hydromyelia than with the pathology described in the first case. In most cases this pathological deviation associated with congenital hernias formed in various parts of the spinal cord.
3. Type III is distinguished by not only the tonsils descending through the hole, but also the cerebellum along with the tissues of the medulla oblongata. They are located in the cervical and occipital regions.
4. Type IV is an underdevelopment of cerebellar tissue. This pathology is not accompanied by their displacement in the caudal direction. But the anomaly is most often accompanied by a congenital cyst located in the cranial fossa and hydrocephalus.

Types II and III often appear in combination with symptoms of nervous system dysplasia, for example, heterotopia of the cerebral cortex tissue, foramen cysts, etc.

Symptoms

The most common among the anomalies is the pathology of the first type. With it, the manifestation of liquor-hypertensive syndrome is often possible, as well as cerebellar-bulbar and syringomyelic phenomena, work disorders nerve endings inside the skull.

Liquor-hypertensive syndrome is pain in the back of the head and neck muscles, which intensifies during sneezing, coughing or straining the neck muscle tissue. Often the pain is accompanied by vomiting that is not associated with eating. Many symptoms of the pathology appear depending on the position of the cerebellar tonsils relative to the opening that is located in the occipital fossa of the skull. Also observed:

• increased tone of the cervical muscles;
• speech dysfunction;
• deterioration of the organs of vision and hearing;
• deviations when swallowing;
• frequent dizziness accompanied by noise in the head;
• a feeling of rotation of the environment;
• brief fainting;
• pressure changes during sudden movements;
• tongue atrophy;
• hoarseness of voice;
• breathing and sensory disorders different parts bodies;
• attacks of numbness;
• disorders in the pelvic organs;
• weakening of the muscles of the limbs.

Anomalies of types II and III have similar symptoms, noticeable from the first moments after the birth of the baby. The second type is accompanied by noisy breathing, as well as unexpected attacks of respiratory arrest, neuroparesis of laryngeal tissues. Deviations in the swallowing process are also observed.

Signs of dystopia are rarely obvious. But neurological manifestations are still possible:

• attacks of “shooting” pain in the neck muscles with increased tension or coughing;
• frequent pain in the head area;
• attacks of dizziness and fainting.

If the prolapse of the tonsils is severe, sometimes there is a widening of the canal connecting the brain and spinal cord, and cavities form around the canal.

Diagnostic methods

Main modern method diagnosing dystopia is MRI. In this case, neither CT nor X-ray studies provide full picture pathology.

No tests are suitable for diagnosing Chiari syndrome. standard methods such as EEG, EchoEG or REG, because they do not allow an accurate diagnosis. An examination by a neurologist will also not determine the anomaly. All these methods can only show suspicion of increased pressure inside the skull. X-rays of the skull should also not be done, since they only show abnormalities of bone tissue that may accompany pathology. Therefore, before the introduction of tomography into diagnostic practice, diagnosing this disease was problematic. Modern diagnostic methods make it possible to accurately determine the pathology.

In the case of high-quality visualization of the bone tissue of the vertebral junction, methods such as MSCT or CT do not provide a sufficiently accurate picture. The only one in a reliable way Today, the only way to diagnose Chiari malformation is by MRI.

Since research with this method requires the patient to remain immobile, young children are put into artificial sleep with the help of medications. An MRI of the spinal cord is also performed. It is aimed at diagnosing any abnormal abnormalities in the functioning of the nervous system.

Treatment

Conservative treatment methods are possible only for very minor deviations. It all depends on what the patient’s condition is at the time of going to the doctor. In this case, treatment is aimed at removing painful symptoms non-steroidal drugs or muscle relaxants. Correction of the regime is also necessary.

The only one effective method treatment for extensive deviations is surgical intervention, which consists of expanding the cranial fossa and plasticizing the dura mater tissue.

Indications for surgical treatment are:

• debilitating headaches that cannot be relieved by medications.
• build-up brain manifestations leading to disability.

If the abnormal deviation occurs without any noticeable signs, no treatment is required. In cases where painful sensations in the area of ​​the neck and back of the head conservative therapy, in which analgesics and aseptic agents are used medicinal substances, as well as muscle relaxants.

When Chiari malformation is accompanied by neurological dysfunction or when a conservative course of therapy does not produce results, surgery is prescribed.

Often when treatment courses for Chiari syndrome, the method of craniovertebral decompression is used. The operation involves widening the opening of the occipital part by removing part bone tissue, cutting off the cerebellar tonsils and parts of two vertebrae of the neck. Thanks to this, the turnover of cerebrospinal fluid in the brain tissue is normalized as a result of a patch made from an allograft or artificial material. Chiari syndrome is sometimes treated with a shunt, which allows cerebrospinal fluid to drain from the central canal. Through surgical operation Cerebrospinal fluid can be drained into the vessels of the chest or peritoneum.

Forecast

Chiari malformation type 1 can be asymptomatic throughout life. And the third type of pathology almost always leads to fatal outcome, if not implemented timely treatment. In the event of the appearance of neurological signs of the disease of the first or last type, timely surgical treatment is very important, because the resulting lack of neurological functions will be poorly restored, even if the manipulations are successfully performed. According to various sources, the effectiveness of surgery is observed in approximately half of the episodes.

Arnold-Chiari malformation is classified as a defect of the cranio-vertebral zone. It is formed in the posterior cranial fossa (PCF), when its volume is insufficient, the posterior parts of the brain and cerebellum are displaced towards the foramen magnum, and the current is disrupted cerebrospinal fluid. The back of the skull forms the so-called posterior cranial fossa, in which the hemispheres and the cerebellar vermis, the pons, and the medulla oblongata are located, which passes into the spinal cord after passing through the foramen magnum. The foramen magnum is limited by the bony base of the skull and is not capable of changing its diameter; any displacement of brain structures is fraught with a discrepancy between their size and the diameter of the foramen, wedging and pinching of nervous tissue, the consequences of which can be fatal. Vital nerve centers responsible for activity are concentrated in the medulla oblongata. of cardio-vascular system and breathing, so not only neurological deficits will be a manifestation of the disease. In severe cases, vital functions are depressed and the patient may die. Displacement of the cerebellar hemispheres leads to a stop in the circulation of cerebrospinal fluid with hydrocephalus, which further aggravates the existing disorders. Arnold-Chiari malformation can be congenital, developing in the fetus and combined with other developmental abnormalities, and its clinical manifestations do not always appear immediately. In some cases, a significant period of time passes before the manifestation of the pathology, or a situation arises that provokes the manifestation of a previously asymptomatic anomaly; in other patients, it may even turn out to be an accidental finding on MRI. Often the pathology is acquired in nature and occurs under the influence external reasons, while the brain and skull at birth have a normal structure.

Causes and mechanism of development of posterior fossa defect (PCD)

There is no consensus on the etiology of Chiari malformation. Scientists put forward various theories, each of which is completely justified and has a right to exist.

Previously, the anomaly was considered exclusively congenital defect, however, observations of specialists showed that only a small part of the patients had defects during intrauterine development, while the rest acquired them during life.

The causes of acquired pathology of the cranio-vertebral junction are considered to be uneven growth rates of nervous tissue of the brain and bone base skull, when the brain grows much faster than the bone container in which it is located. The resulting volume discrepancy is the basis of Arnold-Chiari disease. Congenital form pathology is combined with bone dysplasia, leading to underdevelopment of the skull bones, as well as a violation of the formation of the ligamentous apparatus, and any external influence, trauma can dramatically aggravate the manifestations of pathology. The combination of a cranial fossa defect with other disorders of uterine development and congenital syndromes. Neurologists have formulated two main mechanisms for the formation of pathology:

• Reducing the size of the PFC with normal volumes parts of the brain (probably due to disturbances during the prenatal period).
• Increasing the volume of the brain itself while maintaining correct parameters cranial fossa and foramen magnum, when the brain pushes its caudal sections towards the foramen magnum.

Since the anomaly can be congenital, among the causes are those that can change normal course pregnancy and intrauterine development:

1. Abuse of medications, drinking alcohol and smoking during pregnancy, especially during pregnancy early stages when the organs and systems of the embryo are just being formed;
2. Viral lesions in pregnant women, among which infections with a teratogenic effect - rubella, cytomegalovirus, etc. - are especially dangerous.

Arnold-Chiari malformation also occurs for a number of acquired reasons when the brain and bones of the skull are initially correctly developed. The following can lead to its appearance after birth:

1. Birth injuries, both spontaneous and during obstetric care;
2. Traumatic brain injuries and hydrodynamic impact of the cerebrospinal fluid on the walls of the spinal cord canal in the event of a violation of the cerebrospinal fluid dynamics (this happens in adults);
3. Hydrocephalus.

Hydrocephalus can be a provoking factor, since an increase in the volume of contents in the skull, even due to fluid, inevitably entails an increase in pressure and a displacement of the brain in the caudal (posterior) direction. On the other hand, it is a manifestation of the anomaly itself, when cerebellar prolapse causes a blockade of the cerebrospinal fluid pathways and an increase in the pressure of the cerebrospinal fluid circulating through the cavities of the brain.

Types and degrees of Arnold-Chiari malformation

Depending on the presence of certain changes in the brain and bone base of the skull, it is customary to distinguish several types of Arnold-Chiari anomaly:

• Chiari malformation type 1, when there is a downward displacement of the cerebellar tonsils, is usually detected in adults and adolescents, and is often combined with impaired cerebrospinal fluid dynamics and accumulation of cerebrospinal fluid in the central canal of the spinal cord (hydromyelia). Possible compression of the brain stem.

Arnold-Chiari malformation type 1 is the most commonly diagnosed and has a fairly favorable prognosis

• Arnold-Chiari malformation type 2 - manifests itself already in newborns, as there is a displacement of a much larger volume of the brain than with type 1: the cerebellar tonsils and its vermis, the medulla oblongata with the fourth ventricle, and possibly the midbrain formations. Usually, with stage 2 defects, there is a disruption of the flow of cerebrospinal fluid with hydromyelia. The disease is often combined with the presence congenital hernia spinal cord and vertebral anomalies.
• Type 3 of the disease is characterized by protrusion of the pia mater with the substance of the brain in the occipital region, which also includes the cerebellum and medulla oblongata.

Arnold-Chiari malformation type 3 in the picture

• Arnold-Chiari malformation type 4 is manifested by underdevelopment of the cerebellum when the latter is reduced, and therefore does not descend more distally to the canal in the bone. The pathology makes the newborn unviable and usually ends in death.

As for the degrees of severity:

Arnold-Chiari malformation of the 1st degree can be considered one of the mildest variants of the pathology, since there are practically no malformations of the brain itself, and the clinic may be completely absent, appearing only under unfavorable conditions - trauma, neuroinfection, etc. Malformations of the second and third degree, in turn, are often combined with various malformations of nervous tissue - hypoplasia of some parts of the brain and subcortical nodes, displacement of gray matter, cysts of the cerebrospinal fluid tract, underdevelopment of the brain convolutions.

Manifestations of Arnold-Chiari syndrome

The symptoms of Arnold-Chiari syndrome are determined by its type and the nature of the displacement of the PCF structures. It is often asymptomatic and discovered incidentally during brain examinations. In adults, the appearance of symptoms can be triggered by a head injury; in children, some forms of the disease are noticeable already in the first hours and days of life. Type I anomaly is diagnosed most often and can manifest itself in adolescence or adulthood with the following syndromes:

1. Hypertensive;
2. Cerebellar;
3. Bulbar;
4. Syringomyelic;
5. Phenomena of defeat cranial nerves.

Hypertension syndrome is caused by an increase in intracranial pressure due to blockage of the outflow of cerebrospinal fluid by displaced parts of the brain. It manifests itself:

• Headaches in the back of the head, especially when sneezing or coughing;
• Nausea and vomiting, after which the patient does not feel relief;
• Neck muscle tension.

Signs of cerebellar involvement (cerebellar syndrome) include speech disorders, motor function, balance, nystagmus. Patients complain of unsteadiness of gait, instability of body position in space, difficulty fine motor skills and clarity of movements. Damage to the brainstem seems dangerous due to the location of the nuclei of the cranial nerves and vital nerve centers. Stem symptoms include:

1. dizziness; double vision and decreased vision;
2. difficulty swallowing;
3. hearing loss, tinnitus;
4. fainting, hypotension, sleep apnea.

Adult carriers of Arnold-Chiari malformation indicate an increase in dizziness and tinnitus, as well as paroxysms of loss of consciousness when turning and tilting the head. Due to compression of the trunks of the cranial nerves, atrophy of half of the tongue and impaired movement of the larynx with a disorder of swallowing, breathing and voice formation appear.

With the formation of cavities and cerebrospinal fluid cysts against the background of obstructed flow of cerebrospinal fluid in patients with variant I of malformation, signs of syringomyelic syndrome appear - sensory disorder, skin numbness, muscle wasting, dysfunction pelvic organs, decrease and disappearance of abdominal reflexes, peripheral neuropathies and changes in the joints. Sensitivity disorders are accompanied by a disturbance in the perception of one’s own body, when the patient, having closed his eyes, cannot say in what position his arms or legs are. Sensitivity to pain and temperature also decreases. According to the observations of neurologists, the diameter and localization of a spinal cord cyst do not necessarily affect the severity and prevalence of sensory and motor disorders or muscle wasting. With type 2 and 3 syndrome, the course of the pathology is much more severe; symptoms appear in the child immediately after birth. Breathing disorders are characteristic - stridor (noisy breathing), attacks of stopping it, as well as bilateral laryngeal paresis, which provokes swallowing disorders when liquid food enters the nasal passages. The second type of anomaly in babies in the first months of life is accompanied by nystagmus, increased muscle tone in the arms, and bluish skin, which are especially noticeable when feeding a baby. Movement disorders are variable, their manifestations change, tetraplegia is possible - paralysis of both the upper and lower extremities.

Arnold-Chiari malformation of the third and fourth variants is severe, This is a congenital pathology that is incompatible with normal life activities, so the prognosis for such a diagnosis cannot be considered favorable.

Arnold-Chiari malformation can lead to complications caused by blockade of cerebrospinal fluid flow, damage to the cranial nerve nuclei, and infringement of stem structures. The most common are:

• Hypertension-hydrocephalic syndrome - an increase in intracranial pressure due to blockage of the cerebrospinal fluid outflow pathways, possible in both children and adults;
• Breathing disorders, apnea;
• Infectious and inflammatory processes - bronchopneumonia, urinary infections, which are associated with supine position patient, impaired swallowing and breathing, and pelvic organ function.

At severe course pathology can cause coma, cardiac and respiratory arrest, which leads to death in a matter of minutes. Resuscitation measures They allow you to provide vital functions, but, unfortunately, it is almost impossible to bring the brain back to life and eliminate the irreversible consequences of compression of its parts.

Diagnosis and treatment of Arnold-Chiari malformation

Based on the characteristics of the symptoms and on the basis of an examination by a neurologist, it is impossible to make a diagnosis of Chiari malformation. Encephalography and studies of the blood vessels of the head will also not provide any information regarding the causes of neurological disorders, but may show the presence high blood pressure in the skull. X-ray, CT, MSCT will indicate the presence of defects in the skull bones, which are characteristic of this pathology, but the condition of the soft tissue structures and nervous tissue cannot be determined. Accurate diagnosis of the anomaly has become possible thanks to the use of MRI, through which the doctor can determine both bone defects and development options of the brain itself, its vessels, the level of location of the sections relative to the cranial bones, their sizes, the volume of the posterior cranial fossa and the width of the foramen magnum. MRI can be considered the only accurate and most reliable method for detecting pathology. MRI requires immobilization of the patient, who must lie quietly on the machine table for some time. Children may have significant difficulties with this, so the study is carried out in a state of medicated sleep. To search for combined defects of the spinal cord and spine, these parts of the spinal column are also examined.

When the diagnosis is established, the patient is referred to a neurosurgeon or neurologist to determine a treatment plan, indications for surgery, and its type.

Arnold-Chiari malformation, which is asymptomatic, does not require treatment. Moreover, the carrier of the pathology himself may not realize that something is wrong in the body. When clinical signs conservative or surgical treatment is indicated for the disease. If manifestations are limited to headaches, it is prescribed drug therapy , including anti-inflammatory drugs (Nise, ibuprofen, diclofenac), analgesics (ketorol) and drugs that relieve muscle spasms (mydocalm). In the presence of neurological disorders, signs of compression of parts of the brain, nerve trunks, in the absence of effect from drug treatment for 3 months, the patient needs surgical correction. The operation is necessary to eliminate compression of the nerve tissue and normalize the circulation of cerebrospinal fluid. The most popular operation for Chiari disease is considered to be craniovertebral decompression, which is aimed at increasing the size of the PCF. During decompression, the surgeon removes sections of the occipital bone, resects the cerebellar tonsils, and, if necessary, excises the posterior sections of the first cervical vertebrae. To prevent prolapse rear parts brain, special synthetic patches are applied to the dura mater into the resulting hole.

example of removal of parts of the occipital bone and cervical vertebrae. Decompression of the cervical vertebrae is considered both traumatic and risky. The statistics are that complications occur in at least every tenth patient, while without surgery the mortality rate is much lower. Due to high risk For surgical treatment, neurosurgeons resort to it only in the case of really serious indications - clinical signs of compression of areas of the brain. Another option for surgical treatment is shunting, which ensures the outflow of cerebrospinal fluid from the cranial cavity into the chest or abdominal cavity. By implanting special tubes, cerebrospinal fluid flows and intracranial pressure decreases. In severe forms of pathology, hospitalization and preventive measures are indicated infectious complications and correction of neurological disorders. The increase in cerebral edema due to wedging of its parts into the foramen magnum requires treatment in intensive care, including the fight against edema (magnesium, furosemide, diacarb), establishment artificial ventilation lungs in case of breathing problems, etc.

Life expectancy and prognosis for Arnold-Chiari malformation depend on the type of pathology. In type I, the prognosis can be considered favorable; in some cases, the clinic does not occur at all or is provoked only by strong traumatic factors. At asymptomatic carriers of the anomaly live as long as all other people. For anomalies of the second and first types with clinical manifestations the prognosis is somewhat worse, since a neurological deficit manifests itself, which is difficult to eliminate even with active treatment Therefore, timely surgery is of great importance for such patients. The sooner the patient is provided surgical care, the less pronounced neurological changes await him. Malformations of the third and fourth types are the most severe forms pathology. The prognosis is unfavorable, since many brain structures are involved, there are often combined defects of other organs, and severe dysfunction of the brain stem that is incompatible with life.

Video: presentation and other information on Arnold-Chiari malformation

Step 1: pay for the consultation using the form → Step 2: after payment, ask your question in the form below ↓ Step 3: You can additionally thank the specialist with another payment for an arbitrary amount