Arteriovenous malformation of cerebral vessels occurs during fetal development, but its causes are not well understood. Normally, the heart should pump oxygen-enriched (arterial) blood through the arteries, which branch into small arterioles, and then pass into the capillaries. Oxygen is used by the brain cells, after which the venous blood enters the small venules, which connect into the large veins, and the blood returns back to replenish the lungs with oxygen.
If you have arteriovenous malformation in your cerebral vessels, then blood flows directly from arteries to veins through abnormal vascular connections between them. This disrupts the normal blood supply to the brain.
• Male gender. For unknown reasons, AVM is more common in men.
• Family history. Cases of AVM have been reported in representatives of the same family, but the genetic factor of AVM remains poorly understood. It is also possible the inheritance of diseases that predispose to AVM.
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:
- Arteriovenous malformation is a type of disease characterized by the complete absence of capillaries connecting the venous and arterial networks with each other.
- 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.
- 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.
Such a pathology is congenital, and reliable and accurate causes remain unknown to modern medicine. The main assumption is that injuries and fetal malformations have a negative effect on the formation and structure of blood vessels.
Medical specialists were able to identify risk groups:
- male gender, since the vast majority of the disease affects men;
- genetic mutations;
- infectious diseases that the mother suffered during gestation;
- bad habits that a woman suffers during pregnancy, such as smoking, excessive drinking of alcohol and medications;
- fetal injury.
These are the main factors affecting the possibility of developing pathology.
The size of the malformation can be different: from small to large. The largest foci can cause a person to have large hemorrhages and epileptic seizures. If the lesions are small, then the symptomatology does not appear in any way and does not make itself felt for a long time.
The most common symptoms of this disease:
- frequent attacks of headache;
- partial or complete loss of vision;
- loss of sensation of limbs;
- muscle weakness;
- frequent cramps, loss of consciousness;
- problems with speech;
- lethargy, general malaise, disability.
Doctors distinguish the following disease groups according to the clinical picture:
- The torpid nature of the course is characterized by headaches, frequent dizziness, and nausea. Since such manifestations apply to many other diseases, it is impossible to identify problems with the vessels of the brain by them. The affected area is located in the cortical areas of the brain.
- A hemorrhagic character is inherent in 75% of patients suffering from this pathology. The hemorrhage can be small, and in this case there will be no expressed symptomatology. However, if the hemorrhage turns out to be massive, then vital brain centers will be at great risk of a malfunction. Symptoms may include the following symptoms: impaired speech and coordination, impaired hearing and vision. The worst option is death.
- Neurological in nature occurs with damage to the spinal cord. The vessels, expanding, compress the nerve roots and endings. As a result, there is pain in the back and lower back, as well as numbness of all limbs and a decrease in the sensitivity of the skin.
It is difficult to determine such a disease in young children, because at an early age it is almost asymptomatic, however, there are some signs that may indicate the development of malformation in a child:
- speech defects, loss of intelligence, unstable gait, frequent convulsions;
- impaired coordination, rolling eyes, decreased muscle tone;
- partial visual impairment.
Vascular malformation can be diagnosed at the examination by a neurologist. He prescribes several types of examinations and then makes a final diagnosis.
The following methods are used to diagnose the disease:
- Arteriography is the most common examination method. This procedure involves the introduction of a special instrument in the form of a tube containing a contrast medium into the femoral artery. The tube passes into the blood vessels of the brain. The chemical compound contained in the device allows you to determine the current state of the vessels.
- Computed tomography is often used in conjunction with arteriography, in which case the procedure is called angiography. The diagnostic picture is revealed in a similar way: by means of x-ray radiation and chemical compounds, the state of the vessels can be detected.
- When it comes to arteriovenous malformation, magnetic resonance imaging is considered the most effective. For diagnosis, it is not X-ray radiation that is used, but magnetic particles.
The method of treatment depends on the location of the pathology, its type, severity of symptoms and individual characteristics of the patient. Any of the methods is aimed at achieving complete blockage of blood vessels in order to prevent hemorrhage.
If the patient suffers from frequent and severe headaches, then painkillers should be prescribed.
If the patient is concerned about ongoing convulsive seizures, then the doctors select the appropriate anticonvulsant therapy to avoid brain edema and nerve cell death.
There are cases when it is enough for the patient to be examined by a neurologist and take the prescribed drugs, which reduce the risk of rupture of the vascular plexus. However, this option is possible only in the absence of severe symptoms and complaints of the patient.
In all other cases, doctors resort to surgery. Pathological foci are removed, the vascular walls, where the vessels were incorrectly fused, are soldered. This is done to normalize blood flow and brain circulation.
There are 3 surgical methods in total:
- Surgical resection is one of the most effective types of surgical intervention to get rid of malformation. Such a neurosurgical procedure is complex and is prescribed mainly in situations where the pathological vascular plexus is not deep and is small.
- Embolization is a procedure carried out before surgery, which is used with a deep arrangement of abnormal plexuses of blood vessels and large amounts of pathology. With this method, a special substance that blocks blood flow is injected into the vessel.
- Radiosurgery destroys damaged vessels and completely eliminates malformation. The operation does not require the use of a surgical knife, as it consists in irradiating the patient with protons. This eliminates the possible infection of the patient. The procedure is used if the pathology is small and located at great depths.
Among the complications are:
- Rupture of arterial and venous malformation, which leads to bleeding and circulatory disorders. The result is an increase in the load on the vascular walls, and this can lead to hemorrhagic stroke.
- Due to abnormal plexuses of blood vessels and impaired blood circulation, oxygen deficiency in the brain tissues can occur, and as a result, they gradually die off. This will lead to a heart attack.
- If strong compression by the large vascular plexus occurs, then the likelihood of paralysis increases.
Symptoms of arteriovenous malformation
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.
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.
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.
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.
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.
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.
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:
- 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.
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.
Complications of AVM
• Bleeding. The walls of the affected vessels can become thin and weak. With AVM, very strong pressure is exerted on these walls, as a result of which bleeding (stroke) can occur.
• Inadequate supply of oxygen to the brain. With AVM, the surrounding brain tissue cannot absorb enough oxygen from the blood, which leads to the death of cells. Problems associated with this include speech impairment, weakness and numbness of the limbs, loss of vision, etc.
Diagnostics for AVM
Diagnosis of arteriovenous malformations (AVM) of cerebral vessels involves doctors specializing in diseases of the central and peripheral nervous system – neuropathologists. Depending on the results of the examination, additional diagnostic tests may be prescribed.
• Cerebral arteriography. This test is considered the best way to diagnose cerebrovascular AVM. For the test, a catheter is inserted into the femoral artery, which reaches the vessels of the brain, a contrast medium is injected, after which a series of x-ray photographs of the vessels are made.
• Computed tomography (CT). This is a diagnostic method based on obtaining a “picture” using x-rays.
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Sometimes, to obtain a more detailed image of the affected vessels, the patient is previously injected with contrast. Then the method is called CT angiography.
• Magnetic resonance imaging. MRI is more effective than CT when it comes to detecting arteriovenous malformation. This method does not use radioactive radiation, MRI is based on the use of a magnetic field. If a special dye “illuminating” the vessels is introduced before the examination, the method is called MR angiography.