Vascular malformations of the brain and spinal cord

Vascular abnormalities of the brain are rare. There are 100 cases of pathology per 19 thousand people. The reason for its occurrence is still unknown. It is assumed that injuries and intrauterine malformations of the fetus have a negative effect on the structure of blood vessels.

Specialists identify two main risk groups:

  1. Male gender (more often the disease occurs in men).
  2. Genetic mutations.

This problem remains unexplored to the end, so there are a variety of hypotheses about the nature of its occurrence. Some experts talk about the negative factors that the expectant mother was exposed to during pregnancy (alcohol, drugs, smoking, taking illegal drugs, chronic diseases).

As a rule, vascular malformation of the brain occurs when arteries and veins in the back of the hemispheres are affected, but the formation of pathology in any other part of it is not ruled out. The larger the pathological node and the deeper it is, the more pronounced the neurological symptoms.

Pathology is divided depending on which vessels are affected:

  • With an abnormal connection of veins, venous malformation of the vessels of the brain is formed.
  • If the problem lies in arteries that directly pass into the veins, then this is the arteriovenous malformation of the brain (AVM), which is most often found.

With AVM pathology, the thin vessels connecting the veins with arteries twist and pass into each other, forming a vascular interlacing. In this plexus there is no protective wall consisting of capillaries, therefore, in the pathological region, blood flow is noticeably accelerated. As a result, arterial walls hypertrophy and expand, and arterial blood enters the veins.

Weave take blood and nutrients to themselves, robbing neurons. As a result of forced starvation, brain cell functions and metabolic processes are disrupted. The resulting malformations are quite vulnerable, since their vessels are in constant tension, which is dangerous due to their sudden rupture and the occurrence of hemorrhage.

Arteriovenous changes are:

Brain malformation is a congenital pathology in which an abnormal connection of vessels, veins and arteries occurs. Such a problem is not inherited. Disorders in the body in the initial stages are asymptomatic, so the disease is sometimes detected in the patient’s mature age.

In this pathological condition, an additional formation of blood vessels occurs in the organ that connect the arterial, venous and lymphatic links of the blood supply system. The result of this deformation is the discharge of blood and malnutrition of the area located just below the lesion area.

One of the most common diagnoses in medicine is AVM – arteriovenous malformation of cerebral vessels. This disease is characterized by a violation of the connections between arteries and veins, and the result is a violation of blood flow. Additional compounds look like saccular nodes, and the capillaries are completely absent, which leads to an acceleration of blood supply.

This anomaly proceeds with a continuous expansion, and subsequently a possible damage to the vessels. When they reach critically large sizes, there is a threat to the normal functioning of the brain.

The disease has the appearance of large pulsating vessels. The sizes of vascular malformation can be different in each case.

Most often, this pathology is manifested in men having an age of 30 years.

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Forms of the disease

Modern medicine divides brain malformation into several forms:

  1. Arteriovenous malformation is a type of disease characterized by the complete absence of capillaries connecting the venous and arterial networks with each other.
  2. Lymphatic pathologies are disorders in which the patient has problems with the lymph nodes. A feature of this form is the frequent absence of a tumor.
  3. The Arnold-Chiari anomaly is characterized by a displacement of the cerebellum to the opening of the occipital lobe, resulting in compression of the medulla oblongata.

Cerebral arteriovenous malformations can arise and develop in any part of the brain, both on its surface and in deep areas. In addition, the onset of pathology can occur between the pulmonary trunk and the aorta.

Spinal cord AVM

Such a disease most often originates in the lower parts of the spine. In the initial stages, it is asymptomatic, and after a while it can be expressed in a strong loss of sensitivity in the back. It can lead to hemorrhage in the spinal cord.

The pulmonary trunk is one of the largest human blood vessels starting from the right ventricle, and the aorta is a large unpaired arterial vessel of a large circle of blood circulation.

The embryo should have connections between the heart and blood vessels – arterial flow. After birth, it begins to overgrow, and if this does not happen, then the overgrown arterial flow is considered a congenital heart disease.

The disease, which originated and develops in this part of the body, provokes the most painful manifestations and complications.

The term “malformation” means any anomaly in development that entails physical changes in the structure or function of the organ and its tissues. The violation can be either congenital or acquired.

Brain vascular malformations often occur in patients at a young age of 10 to 30 years. The ailment consists in the improper connection of the cerebral veins, arteries and small capillaries.

The main symptoms of the disorder are throbbing headaches, sometimes leading to seizures.

The disease is congenital, so abnormal vascular blockage in certain areas of the brain occurs during the prenatal period. Reliable reasons for the development of pathology to the current moment have not been established. But, according to experts, negative factors during pregnancy can presumably contribute to the abnormal structure of the vascular system of the GM in the fetus:

  • getting the mother’s body high doses of radiation;
  • intrauterine infections transmitted in the prenatal period from mother to child;
  • chronic or acute intoxication;
  • smoking and drinking alcohol;
  • narcotic drugs, including from a number of medicines;
  • drugs with teratogenic effects;
  • history of chronic diseases in a pregnant woman (glomerular nephritis, diabetes, bronchial asthma, etc.).
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Experts also believe that a genetic factor may play a role in the formation of a defect. Until recently, heredity was not taken seriously as a cause of pathology. Today, more and more reports are received about the involvement of this factor. So, in a number of cases, vascular malformations of a similar type are determined in the patient’s blood relatives. Presumably, they are caused by a hereditary gene mutation affecting chromosome 5q, the CMC1 locus and RASA1.

As mentioned earlier, the disease is characterized by a prolonged “mute” course, which can last for decades. The diagnosis is either accidentally recognized at the time of a diagnostic examination of the brain structures, or after the break of the malformation. In a few cases, the disease can make itself felt before the vessel bursts. Then the clinic of pathology is more often manifested by such symptoms as:

  • tinnitus (ringing, hum, hissing, etc.);
  • frequent headaches;
  • convulsive syndrome, which is similar to epileptic seizures;
  • neurological symptoms (paresthesia, numbness, tingling, lethargy and apathy, etc.).

The clinical picture of an AVM rupture is similar to all types of intracranial hemorrhage:

  • a sharp appearance of a severe headache, rapidly progressing;
  • dizziness, confusion;
  • fainting, up to the development of coma;
  • nausea, vomiting;
  • loss of sensitivity of half the body;
  • impaired vision, hearing;
  • expressive aphasia, dysarthria (pronunciation disorders);
  • rapidly growing neurological deficit.

In what part of the head are brain AVMs located?

A common localization of arteriovenous abnormalities is the supratentorial space (upper parts of the brain) passing over the tent of the cerebellum. To make it clearer, we’ll explain more simply: in about 85% of cases, a vascular defect is found in the cerebral hemispheres. Lesions of the vascular links of the parietal, frontal, occipital, temporal lobe of the cerebral hemispheres predominate.

In general, AVMs can be located at any pole of the brain, both in the surface parts and in the deeper layers (thalamus, etc.). It is possible to reliably determine the exact localization of the focus only after passing a hardware study with the ability to visualize soft tissues. The basic principles of diagnosis include MRI and angiography.

How is the disease manifested

The size of the malformation can be very different: small, medium, large. The largest foci are capable of causing massive hemorrhages and epileptic seizures. If the affected areas are small, then in such cases, the disease does not make itself felt for a long time and is detected by chance in the diagnosis of other ailments. More pronounced manifestations of the pathology are often found after head injuries, stress, during pregnancy.

According to the clinical picture, there are:

  • The torpid nature of the course, accompanied by headaches, dizziness, nausea. Similar symptoms are inherent in many diseases, so it is impossible to suspect a pathology for them. The focus of interweaving is usually located in the cortical layers of the brain, and by signs simulates oncology.
  • Hemorrhagic character. It occurs in 70% of victims. If the hemorrhage is slight, severe symptoms will not be noticeable. With extensive hemorrhage, vital brain centers are affected. With dystrophic changes in the arteries and their subsequent rupture, a stroke is associated. As a result, the victim may have: impaired speech, vision, hearing, coordination problems. In the worst case, the patient will die.
  • Neurological in nature with spinal cord injuries. It manifests itself as pain in the back and lower back due to the fact that, expanding, the vessels compress the nerve roots, after which numbness of the extremities and violation of the sensitivity of the skin join the pain syndrome.

Symptoms of vascular malformation have their own characteristics and are manifested in:

  • Bouts of headache.
  • Convulsions with impaired consciousness.
  • Muscle weakness.
  • Paresis of the limbs.
  • Violation of stability.
  • Loss of vision (more often with proliferation of the vascular plexus in the frontal lobe).
  • Speech problems.

If malformation is found in older people, then often it no longer progresses.

Types of cerebral malformations of the venous arterial bed

Pathological formations are usually distinguished by topographic and anatomical characteristics, hemodynamic activity, size. The first parameter characterizes the location of malformation in the brain, hence their names come from:

  • surface AVMs are concentrated in the cerebral cortex of the brain (on the surface of the brain) and adjacent structures of white matter;
  • deep AVMs are localized in the depths of the brain gyrus, basal ganglia, inside the ventricles, in the structures of the trunk of the GM.

According to hemodynamic activity, malformations are distinguished:

  • active – they include a mixed type of AVM GM (the most common variety, in which partial destruction of the capillaries is detected) and the fistulous type (the artery passes directly into the vein, the capillary network is completely destroyed);
  • inactive – capillary (telangiectasias), venous, arteriovenous cavernous.

The lesion is also evaluated by size, only the diameter of the malformation coil is taken into account. When diagnosing sizes, the following AVM names are used:

  • micromalformations – less than 10 mm;
  • small – from 10 mm to 20 mm;
  • medium – 20-40 mm
  • large – 40-60 mm
  • giant – more than 6 cm in diameter.

It must be understood that the vessels of the AVM are too prone to breakthroughs, since they are seriously depleted on the basis of an abnormal structure and impaired blood flow. At the same time, large malformations compress and damage the surrounding brain tissue, which poses an additional threat to the viability of the central nervous system. Therefore, if the diagnosis is clinically confirmed, it is impossible to delay the treatment with treatment.

Diagnosis of the disease

If there is a suspicion of a vascular disease in the brain or spinal cord, the neurologist directs the patient for diagnosis, which will determine the location of the focus, its size and characteristic changes in the vessels. Common diagnostic methods are:

  • Magnetic resonance imaging. Shows exactly where the changes occurred.
  • CT scan. Allows doctors to identify the focus of the disease.
  • Angiography of vessels with contrast. Helps identify stranded vessels.
  • Duplex scanning. It registers the speed of blood flow in the vessels and helps to quickly find the pathological area.
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Patients must be referred to:

  • General blood and urine tests.
  • Biochemistry of blood.
  • Coagulogram.
  • Echoencephalogram.

An optometrist consultation is also required.

Treatment of the disease

The consequences of pathology are ruptures of the vascular wall with hemorrhages, ischemia, aneurysms. Treatment is only surgical, aimed at the removal, embolization or obliteration of malformation.

What’s this

Normally, veins with arteries are connected by an extensive network of small capillaries. The function of the latter is the transfer of nutrients from the blood to the tissues of organs. Diffusion is possible due to the single-layer structure of the capillary wall.

In malformation, part of the capillary network is replaced by larger convoluted vessels. Most often, this tangle is a combination of veins with arteries – in this case we are talking about arteriovenous malformation of cerebral vessels (abbreviated AVM).

Sometimes anomalies of only one type are observed.

What is the danger of this pathology? With a small size of conglomerate, it may not manifest itself for a long time, but large formations are very dangerous. First of all, the risk of rupture, since the vascular wall of malformation is deformed, thinned.

If, in addition to malformation, there are aneurysms, mortality increases.

Another likely consequence is ischemia of brain tissue. The discharge of blood directly from arteries into veins leads to the fact that portions of the brain substance located lower in the direction of blood flow lose oxygen with nutrients. The result of prolonged “starvation” is dysfunction and tissue death by the type of ischemic stroke.

Despite the fact that the disease is quite rare – 2 cases per 100 – due to negative consequences and high mortality, special attention is paid to him in neurology.

Classification

Vascular malformation located in the brain can consist only of veins, arteries or be combined. The last option – arteriovenous – is most common.

According to the type of structure, cerebral AVMs are divided into:

  • Racemous (make up ¾ of the total) – a branched vascular conglomerate.
  • Fistulous – is a massive shunt between large vessels.
  • Cavernous – an accumulation of thin-walled cavities that resembles mulberry berries (diagnosed in 11% of cases of AVM).
  • Micromalformation is a small formation.

Among isolated isolated venous malformation, arterial, telangiectasia. Anomalies are also distinguished by size. The diameter of small ones does not exceed 30 mm, medium ones – 60 mm, and large ones are tangles larger than 6 cm.

For the diagnosis and treatment, the localization of the defect is important: in mild cases, they are located outside functionally significant areas, which include the brain stem, temporal and occipital lobes, thalamus, sensorimotor cortex, speech region, Broca center.

The nature of the drainage is also important, that is, the presence of access to large veins.

The classification parameters listed are important for determining the risk in the event of surgery. Each of them (localization, type of drainage, size) is evaluated on a three-point scale, and depending on the amount of points scored, operational risk is determined. Low is 1, and five means increased technical complexity of the intervention, a high probability of disability or death.

Causes

In most cases, arteriovenous malformation of the brain is a consequence of a violation of the formation of the cerebral vasculature in the prenatal period. The genetic factor has not been proven, therefore, heredity does not seem to play a role.

Chronic diseases of the expectant mother, intrauterine infections, and an increase in radiation background have a negative effect on the development of the fetal cerebral blood circulation system.

The use of certain drugs, intoxication, the presence of bad habits in a pregnant woman (alcoholism, drug addiction, smoking) also give a teratogenic effect.

The frequency of hemorrhages in children with cerebrovascular pathology of this type is small. Usually, the disease first appears after reaching the age of 20.

As a patient grows older, the risk of a break in education increases. Acquired malformations are very rare, manifesting to 50 years.

Among the causes of the development of the disease, atherosclerotic, sclerotic changes in the vascular wall, as well as traumatic brain injuries, are distinguished.

Symptoms

The symptomatology of the disease depends on the type of its course. The first, hemorrhagic, is observed in more than half of cases (according to statistics, up to 70%). It is characteristic of small-sized vascular malformations. The second variant of clinical manifestations – torpid – is found in large and secondary formations.

Small conglomerates characteristic of this type of course may not occur for years. Often the first sign of a disease is the rupture of a defective vessel wall with subsequent hemorrhage.

If an arteriovenous abnormality is deployed in the posterior cranial fossa and has drainage veins, a clinical symptom such as arterial hypertension appears.

In the event of a rupture, the symptoms of hemorrhagic stroke develop:

  • intense headache;
  • nausea with bouts of vomiting;
  • paresthesia, paralysis, muscle weakness;
  • confusion and loss of consciousness;
  • disorders of brain activity (coordination, vision, speech).

The listed symptoms are characteristic of subarachnoid hemorrhage (bleeding occurs between the meninges). It accounts for almost half of the total number of cases.

In the rest, blood is poured directly into the brain with the formation of hematomas of various localization. The most dangerous of them are intraventricular.

Specific symptoms are attached to the general symptomatology with intracerebral hemorrhage, by which it is possible to determine the affected area of ​​the brain.

Unlike hemorrhagic, the presence of vascular malformation can be judged even before its rupture. Large and medium-sized anomalies are manifested by regular cluster headaches. Attacks can last for 3 hours, against their background convulsive syndrome often develops. Another characteristic feature may be a neurological deficit inherent in brain tumors.

In this case, cerebral symptoms develop: diffuse headaches of a bursting nature, mental and visual disturbances, vomiting, epiprotic. By the nature of epileptic seizures and the aura preceding them, the location of malformation can be reliably determined.

Depending on the localization, cognitive dysfunctions, paresis of the facial nerves, paralysis of the extremities and other neurological disorders can be observed.

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AVM veins Galena

A separate type of congenital cerebrovascular pathology in children, characterized by a complex of defects in the development of the large cerebral vein, including bypass surgery. It is quite rare, but in most cases it leads to death.

The only treatment is neurosurgery in the first year of life. The main symptoms are determined immediately after birth in half of the babies with this type of AVM: heart failure, hydrocephalus. Subsequently, there is a lag in mental and physical development.

Diagnostics

Prior to rupture, vascular malformation of the hemorrhagic type often does not appear in any way and can be detected randomly. In the torpid course of the disease, headaches, the appearance of a convulsive syndrome, and focal signs are the reason for contacting a neurologist. Based on complaints, the doctor appoints a consultation of a neurosurgeon who conducts a comprehensive examination:

  • electroencephalography;
  • echoencephalography;
  • rheoencephalography;
  • CT and MRI;
  • cerebral angiography.

Computed and magnetic resonance imaging in the case of pathology of the torpid type may be uninformative. Angiography using a contrast agent is preferred to confirm the diagnosis. At break, the whole complex of diagnostic measures is carried out urgently, while the most informative is MRI.

Treatment of the anomaly before rupture is reduced to the exclusion of conglomerate from the bloodstream. This task is performed by one of three methods: embolization of the AVM of the brain, transcranial or radiosurgical removal. The methodology is selected taking into account all operational risks, the size and localization of education.

The operation is performed according to indications also after rupture, when the patient’s condition stabilizes. In the acute stage, only removal of the hematoma is possible. Combined intervention (clot extraction and excision of an AVM) is indicated for small education.

Performed transcranially after craniotomy, if the volume of vascular malformation does not exceed 100 ml. It is used for the shallow occurrence of conglomerate outside functionally significant areas of the brain. During the operation, the resulting vessels are blocked by the coagulation method, the conglomerate is secreted, the discharge veins are ligated, and the AVM is excised completely.

Classical removal in most cases guarantees complete recovery, however, nootropics with angioprotectors in the recovery period are required. Be sure to systematically monitor a doctor, since complications are likely – strokes.

Embolization AVM

Less traumatic treatment, aimed not at removal, but at the occlusion of cerebrovascular anomaly. Using a microcatheter inserted into the incision of the femoral artery, a special copolymer gluing vessels is introduced into the malformation. This method is used only when there are lead vessels available for catheterization.

In addition, embolization is carried out in several stages, and complete occlusion can be achieved in only a third of patients. Therefore, often this manipulation is carried out as preparatory before surgical excision. Pre-gluing part of the vessels reduces the risk of surgical bleeding and complications in the postoperative period.

Like the classical surgical operation, it allows you to completely remove the anomaly (however, its size should not exceed 3 cm).

This method is used when transcranial access to vascular malformation of the brain is difficult and embolization is not possible.

Where is better to be operated on and prices of operation

A well-planned algorithm of therapeutic actions will help to completely remove the vascular conglomerate without sacrificing vital tissues. The adequacy of therapy, taking into account the principle of individuality, will protect against the progression of neurological abnormalities, a possible early relapse with all the ensuing consequences.

To operate the highest organ of the central nervous system, which is responsible for many functions in the body (motor abilities, memory, thinking, speech, smell, vision, hearing, etc.), one should trust the world-class neurosurgeons. In addition, the medical facility should be equipped with a wide base of high-tech advanced intraoperative equipment.

In foreign countries with highly developed neurosurgery, services are expensive, but there, as they say, bring patients back to life. Among the popular areas equally advanced in brain surgery are the Czech Republic, Israel and Germany. In Czech clinics, the prices for medical care for arteriovenous malformations are the lowest.

Central Military Hospital of Prague.

Surgery methodRussia (rubles)Czech Republic (Euro)Germany, Israel (Euro)
Operation on CyberKnife or GammaKnifefrom 2000008000 – 12000from 20000
Endovascular interventionfrom 75000to 8000from 15000 and more
Microsurgical resection150000 – 2500007000 – 1000013000 – 18000

Why are malformations dangerous?

Symptoms

  • The possibility of rupture of arterial venous malformation with subsequent bleeding due to thinning of the vascular walls. Disturbed blood circulation increases the load on weakened walls. Due to increased pressure, hemorrhagic stroke occurs. Such a patient must be urgently taken to a hospital, where he will be provided with qualified assistance.
  • Lack of oxygen in brain tissue due to impaired blood circulation leads to their gradual death, and later to brain infarction. Terrible symptoms of the pathological process are memory loss, visual impairment, problems with speech.
  • Paralysis. May occur when squeezed by the large vascular plexus of the spinal cord.

To avoid the development of complications, treatment should not be delayed. Patients need to use all the methods of treatment of arteriovenous malformation proposed by specialists. We should not forget that cerebral hemorrhage results in disability or death of the patient.

Prevention of complications

Specialists recommend that their patients not neglect preventive measures that are aimed at preventing possible complications. It follows:

  • Eliminate heavy physical exertion requiring weight lifting.
  • Avo >

Timely competent treatment with resection of the vascular bundle gives the patient a chance for a full recovery. Modern medical technology allows you to identify the disease in the early stages and provide the patient with qualified care.

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Tatyana Jakowenko

Editor-in-chief of the Detonic online magazine, cardiologist Yakovenko-Plahotnaya Tatyana. Author of more than 950 scientific articles, including in foreign medical journals. He has been working as a cardiologist in a clinical hospital for over 12 years. He owns modern methods of diagnosis and treatment of cardiovascular diseases and implements them in his professional activities. For example, it uses methods of resuscitation of the heart, decoding of ECG, functional tests, cyclic ergometry and knows echocardiography very well.

For 10 years, she has been an active participant in numerous medical symposia and workshops for doctors - families, therapists and cardiologists. He has many publications on a healthy lifestyle, diagnosis and treatment of heart and vascular diseases.

He regularly monitors new publications of European and American cardiology journals, writes scientific articles, prepares reports at scientific conferences and participates in European cardiology congresses.

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