Vascular Malformation: Causes, Symptoms, Diagnosis, and Treatment Methods

If in the process of embryonic development the formation of the circulatory system is disturbed, then vascular malformation is formed. This is an incorrect combination of veins and arteries, which manifests itself at pubertal age. It all starts with headaches, migraines and seizures. In severe cases, children experience epileptic seizures.

Definition

The structure of the vessels is different throughout the circulatory system. Some of them are thicker, some have a muscular wall, some have valves, but they are all connected in a certain sequence. If for some reason the sequence established by nature is violated, then conglomerates of convoluted vessels, called malformations, are formed.

As a rule, this is an innate pathology, the causes of which are unknown. It occurs in nineteen out of a hundred thousand newborns every year. Malformations can cause the syndrome of stealing, squeeze the tissues of the spinal cord and brain, form aneurysms and cause strokes and hemorrhages in the meninges. More often complications occur already in adulthood, after forty years.

Classification

There are several forms that vascular malformation can take. For their systematization is adopted in 1996, the American classification of ISSVA. Its fundamental difference from other classifications is the division of all anomalies into tumors and malformations.

1. Vascular tumors:
   - infantile hemangioma (appears in childhood);
   - congenital hemangioma;
   - pessiform hemangioma;
   Spindle-like hemangiendothelioma;
   - Kaposiiform hemangiendothelioma;
   - Acquired tumors.
2. Malformations:
   - capillary (telangiectasias, angoikeratomas);
   - venous (sporadic, glomangiomas, Mafucci syndrome);
   - lymphatic;
   - arterial;
   - arteriovenous;
   - combined.

Malformation of venous vessels

Venous malformation is an abnormal development of the veins with subsequent pathological expansion. It is the most common among all kinds of malformations. This disease is congenital, but it can manifest itself both in childhood and in adulthood. The location of the altered vessels can be anything: nervous system, internal organs, skin, bones or muscles.

Venous malformations can be on the surface or lie in the thickness of the organ, be isolated or stretch into several parts of the body. In addition, the closer they are to the surface of the skin, the more saturated they are.

Because of the unusual shape and color, they can be confused with hemangiomas. For differential diagnosis, it is enough to apply pressure to the modified area. Malformations are soft and easily change their color. In the case of the occurrence of abnormal vessels deep in the human body, pathology may not appear.

Along with the growth of the child, malformation also increases, but rapid expansive growth of the vessels is observed under the influence of trigger factors, such as surgical interventions, traumas, infections, hormonal preparations, childbearing or the period of menopause.

Chiari Malformation

This is vascular malformation, which is characterized by a low location of the tonsils of the cerebellum. The disease was described in the late nineteenth century by the Austrian doctor Hans Chiari, in whose honor the phenomenon was named. He identified several of the most common species of this anomaly. Because of the low location of the tonsils make it difficult to drain the fluid from the brain to the spinal cord, this creates increased intracranial pressure and provokes the development of hydrocephalus.

Chiari malformation of the first type describes the displacement of the tonsils of the cerebellum downward and pushing them through the large occipital foramen. This arrangement causes an expansion of the canal of the spinal cord, which clinically manifests itself during puberty. The most frequent symptoms are headache, tinnitus, instability in walking, diplopia, articulation, problems with swallowing, sometimes vomiting. For teenagers, a reduction in pain and temperature sensitivity in the upper half of the body and in the limbs is characteristic.

Chiari malformation of the second type develops if the size of the occipital foramen is enlarged. In this case, the tonsils of the cerebellum do not fall, but fall into it. This leads to compression of the spinal cord and the cerebellum respectively. Simultaneously, symptoms of stagnant fluid in the brain, heart defects, embryogenesis of the digestive canal and the genitourinary system are possible.

Spinal cord

Malformation of the spinal cord is a rare disease leading to progressive myelopathy. Arteriovenous malformations prefer to lie between sheets of a hard shell or lie on the surface of the spinal cord in the thoracic or lumbar regions. More often, pathology occurs in adult men.

Symptomatic disease resembles multiple sclerosis and may mislead the neurologist. A sharp deterioration of the condition occurs after the rupture of blood vessels and hemorrhages in the subarachnoid space. In patients, there are disorders of the sensory and motor area, disorders in the work of the pelvic organs. If the cortical symptomatology joins, the disease becomes more similar to ALS (amyotrophic lateral sclerosis).

In the event that the patient presents a complaint typical of two different neurological disorders, the doctor must suspect malformation of the vessels and conduct a visual examination of the spine. Signs of the presence of the altered vessels will be lipomas and areas of increased pigmentation. Such a person should be sent to magnetic resonance imaging or computer myelography. This will verify the diagnosis.

Symptoms

Vascular malformation is similar to a time-bomb or a pistol with a triggered trigger in the child's hands - no one knows when the catastrophe will begin. Despite the fact that the disease is a congenital anomaly, it begins to manifest itself much later. There are two types of vascular malformation:

- hemorrhagic (in 70% of cases);
- Torpid (in the remaining 30%).

None of the options can not be called attractive for a person with this diagnosis. In the case of hemorrhagic flow, the patient has high blood pressure, and the vascular node itself is small and located in the nape of the neck. A half of people with hemorrhagic type of malformation have a stroke, which leads to disability or death. The risk of hemorrhage increases with age, and for women an additional factor is pregnancy and childbirth.

If a person successfully transferred the first stroke, then with a probability of 1: 3 within a year there will be a second hemorrhage. And then the third. Unfortunately, after three episodes, few survive. Approximately half of patients have complex forms of hemorrhage with the formation of intracranial, intrathecal or mixed hematomas and tamponades of the ventricles of the brain.

The second version of the flow causes a large, deep vascular malformation in the cerebral cortex. Symptoms of it are quite typical:

- convulsive alertness or the presence of epileptiform seizures;
- severe headaches;
- the presence of deficit symptoms, similar to brain tumors.

Syncopal condition

Virtually all people with vascular malformations located in the brain, sooner or later appear syncope (that is, faints). This is due to a temporary decrease in the volume of cerebral circulation. During the syncopal condition, the patient is pale, covered with a cold sweat, his hands and feet are cold, his pulse is weak, and his breathing is superficial. The attack lasts about twenty seconds, and upon completion of it the patient does not remember anything.

Each year, there are more than half a million new cases of syncope, and only a small part of them have a clear cause-and-effect relationship. Syncope can be caused by excessive irritation of the carotid sinus, neuralgia of the trigeminal or glossopharyngeal nerve, inadequate regulation of the autonomic nervous system, disturbances in the heart rhythm and, of course, vascular malformations. Abnormal arteries and veins provide shunting of blood and acceleration of its blood flow. Due to this, the brain does not receive enough nutrition, which is manifested by impaired consciousness.

Complications

Even before the malformation manifests itself clinically, the person will conceal irreversible pathomorphological phenomena. This is due to the hypoxia of the area of the brain tissue, its dystrophy and death. Depending on the location of the affected area, there is a characteristic focal symptomatology (speech, gait, voluntary movements, intelligence, etc.), there may be epileptic seizures.

Severe complications begin already in adulthood. Abnormal vessels have a thin wall and are more prone to rupture, so patients with malformations are more likely to have ischemic strokes. Large conglomerates of arteries and veins squeeze tissues around them, causing hydrocephalus. The most dangerous is a hemorrhage due to the rupture of several vessels at once. It can both have lethal consequences, and end almost without consequences. Everything depends on the amount of blood spilled out. Hemorrhagic stroke has a much less perspective prognosis and may recur with time.

Diagnostics

Primary neurologic examination may not reveal any deviations in people with vascular malformations. As a rule, an accurate and very thorough examination is required to detect abnormalities. If the patient complains of frequent severe headaches, tremors, periodic disturbances of consciousness and visual impairment or gait, then this is an occasion to send him to perform neuroimaging. Simply put, a computer or magnetic resonance imaging of the brain.

The most detailed and accurate structure of the vessels shows contrast angiography. To do this, a radiocontrast fluid is injected into the common carotid artery and a series of images are taken. This method has a number of complications and side effects, therefore it is used only in case of difficulties in diagnosing.

In order to see the speed, direction and blood flow level at the altered site, Doppler ultrasound is used. This method allows you to see the discharge of blood between the arteries and veins, determine the type of vessels, determine the presence of aneurysms and other complications.

Treatment

Can vascular malformation be amenable to correction? Methods of treatment depend on the type of anomaly, its location, the size of the focus and the presence of a history of strokes.

There are three main methods of therapy:

- open surgical operation;
- minimally invasive embolization;
- non-invasive radiosurgery treatment.

For each of them there are indications, contraindications and a list of possible complications.

The most traumatic is open surgery. In order to reach the hearth, they perform an autopsy of the cranium, clip the vessels and cross them. This option is possible if the malformation is located on the surface of the brain and has small dimensions. Attempts to reach a deep-seated focus can result in damage to vital centers and death.

What can be done if the patient has a deep malformation? Treatment consists in carrying out endovascular embolization. This is a fairly gentle procedure, during which a large vessel feeding an abnormal conglomerate is injected with a thin catheter and under the control of the X-ray the doctor gets to the malformation. Then a hypoallergenic drug is injected into the lumen of the vessels, which fills all available space and blocks the flow of blood in this area. Unfortunately, this technique does not provide an absolute guarantee that the vessel is obliterated completely. Therefore, most often it is used as an additional treatment.

The most advanced method of treatment of vascular malformations is considered to be operations using a cyber knife (radiosurgery). The essence of the method is to treat the anomalous focus from different points with narrow radioactive rays. This allows you to quickly destroy the altered vessels without damaging the healthy tissues. The process of sclerosing vessels on average takes several months. The advantage is the complete absence of complications from the nervous system. But there are limitations to the application of this method:

1. The total diameter of the receptacles shall not exceed three centimeters.
2. There should be no stroke or other hemorrhage in the history. Since the thin wall can not withstand and break in the gap between the procedure and the final sclerosis of the malformation.