If in the process of embryonic development the formation of the circulatory system is disturbed, then vascular malformation is formed. This is an improper connection of veins and arteries, which manifests itself at puberty. It all starts with a headache, migraine and cramps. In severe cases, children have epileptic seizures.
Definition
The structure of blood vessels differs throughout the circulatory system. Some of them are thicker, some have a muscle wall, some have valves, but they are all connected in a certain sequence. If for some reason the sequence established by nature is violated, conglomerates of convoluted vessels, called malformations, are formed.
As a rule, this is a congenital pathology, the causes of which are unknown. It occurs in nineteen of one hundred thousand newborns every year. Malformations can cause robbery syndrome, squeeze the tissue of the spinal cord and brain, form aneurysms and cause strokes and hemorrhages under the meninges. Most often, complications occur already in adulthood, after forty years.
Classification
There are several forms that vascular malformation can take. For their systematization, the American classification ISSVA, adopted in 1996, is used. Its fundamental difference from other classifications is the separation of all anomalies into tumors and malformations.
- Vascular tumors:
- infantile hemangioma (appears in childhood);
- congenital hemangioma;
- bundle hemangioma;
- spindle-shaped hemangioendothelioma;
- kaposhiformny hemangioendothelioma;
- acquired tumors. - Malformations:
- capillary (telangiectasias, angiokeratomas);
- venous (sporadic, glomangiomas, Mafucci syndrome);
- lymphatic;
- arterial;
- arteriovenous;
- combined.
Venous malformation
Venous malformation is an abnormal development of veins with their subsequent pathological expansion. It is the most common among all types of malformations. This disease is congenital, but can manifest itself both in childhood and in adulthood. The location of the altered vessels can be any: 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 to several parts of the body. In addition, the closer they are to the surface of the skin, the more saturated the color they acquire.
Due to their unusual shape and color, they can be confused with hemangiomas. For differential diagnosis, it is enough to slightly press on the changed area. Malformations are soft and easily change color. In the case of abnormal vessels occurring deep in the human body, outwardly pathology may not manifest itself.
Along with the growth of the child, malformation also increases, but under the influence of trigger factors, such as surgical interventions, injuries, infections, taking hormonal drugs, bearing a child or the period of menopause, rapid expansive vascular growth is observed.
Chiari malformation
This is a vascular malformation, which is characterized by a low location of the tonsils of the cerebellum. The disease was described at the end of the nineteenth century by the Austrian physician Hans Chiari, in whose honor the phenomenon was named. He identified several of the most common types of this anomaly. Due to the low location of the tonsils, it is difficult to drain 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 down and pushing them through the large occipital foramen. This arrangement causes the expansion of the spinal cord channel, which clinically manifests itself during puberty. The most common symptoms are headache, tinnitus, instability when walking, diplopia, impaired articulation, problems with swallowing, and sometimes vomiting. Teenagers are characterized by a decrease in pain and temperature sensitivity in the upper half of the body and limbs.
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 cerebellum, respectively. At the same time, symptoms of fluid stagnation in the brain, heart defects, disturbances in the embryogenesis of the digestive canal and urogenital system are possible.
Spinal cord
Spinal cord malformation is a rare disease that leads to progressive myelopathy. Arteriovenous malformations prefer to be located between the sheets of the hard shell or lie on the surface of the spinal cord in the chest or lumbar regions. More often, pathology occurs in adult men.
The symptomatic disease resembles multiple sclerosis and can lead the neuropathologist astray. A sharp deterioration occurs after rupture of blood vessels and hemorrhages in the subarachnoid space. Patients have disorders of the sensory and motor sphere, disturbances in the work of the pelvic organs. If cortical symptoms join, then the disease becomes more like ALS (amyotrophic lateral sclerosis).
If the patient presents complaints characteristic of two different neurological disorders, the doctor must suspect vascular malformation and conduct a visual examination of the spine. Signs of the presence of altered vessels will be lipomas and areas of increased pigmentation. Such a person should be sent for magnetic resonance imaging or computed myelography. This will verify the diagnosis.
Symptoms
Vascular malformation is like a time bomb or a pistol with a cock in the hands of a child - no one knows when the disaster will begin. Despite the fact that the disease is a congenital anomaly, it begins to manifest itself much later. Two types of the course of vascular malformation are distinguished:
- hemorrhagic (in 70% of cases);
- torpid (in the remaining 30%).
None of the options can be called attractive to a person with this diagnosis. In the case of hemorrhagic course, the patient has high blood pressure, and the vascular node itself is small and is located in the back of the head. Half of people with a 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 has successfully suffered a first stroke, then with a probability of 1: 3 there will be repeated hemorrhage within a year. And then the third. Unfortunately, after three episodes, few survive. Approximately half of patients experience complex forms of hemorrhage with the formation of intracranial, subshell, or mixed hematomas and tamponades of the ventricles of the brain.
The second variant of the course causes a large, lying deep in the cerebral cortex vascular malformation. Her symptoms are quite characteristic:
- convulsive readiness or the presence of epileptiform attacks;
- severe headaches;
- the presence of deficiency symptoms similar to brain tumors.
Syncope
In almost all people with vascular malformations located in the brain, sooner or later syncope (i.e., fainting) occurs. This is due to a temporary decrease in cerebral circulation. During the syncope, the patient is pale, covered with cold sweat, his hands and feet are cold, his pulse is weak, and his breathing is shallow. The attack lasts about twenty seconds, and upon its completion the patient does not remember anything.
Each year, more than half a million new cases of syncope appear, and only a small part of them have a clear causal relationship. Syncope can be caused by excessive irritation of the carotid sinus, trigeminal or glossopharyngeal neuralgia, inadequate regulation of the autonomic nervous system, heart rhythm disturbances, and, of course, vascular malformations. Abnormal arteries and veins provide bypass blood and accelerate 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, a person will secretly develop irreversible pathomorphological phenomena. This is due to hypoxia in the area of the brain tissue, its degeneration and dying. Depending on the location of the affected area, there is a characteristic focal symptomatology (impaired 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, therefore, patients with malformations are more likely to have ischemic strokes. Large conglomerates of arteries and veins squeeze the tissue around them, causing hydrocephalus. The most dangerous is hemorrhage as a result of rupture of several vessels at once. This can both have fatal consequences and end almost without consequences. It all depends on the amount of spilled blood. Hemorrhagic stroke has a much less promising prognosis and may recur over time.
Diagnostics
Initial neurological examination may not reveal any abnormalities in people with vascular malformations. As a rule, an aimed and very thorough examination is required to identify violations. If the patient complains of frequent severe headaches, tremors, periodic impaired consciousness and impaired vision or gait, then this is an occasion to send him for neuroimaging. Simply put, on a computer or magnetic resonance imaging of the brain.
The most detailed and accurate structure of the vessels is shown by contrast angiography. For this, the patient is injected with a radiopaque fluid in the common carotid artery and a series of images is taken. This method has a number of complications and side effects, therefore it is used only in case of difficulties in making a diagnosis.
In order to see the speed, direction and level of blood flow in the changed area, Doppler ultrasound is used. This method allows you to see the discharge of blood between arteries and veins, determine the type of blood vessels, establish the presence of aneurysms and other complications.
Treatment
Is vascular malformation correctable? The 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 surgery;
- minimally invasive embolization;
- non-invasive radiosurgical 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 focus, the cranium is opened, the vessels are clipped and crossed. This option is possible if the malformation is located on the surface of the brain and has a small size. Attempts to reach a deeply lying hearth may result in damage to vital centers and death.
What can be done if the patient has a deep malformation? Treatment consists of endovascular embolization. This is a rather gentle procedure, during which a thin catheter is inserted into a large vessel that feeds an abnormal conglomerate and, under the control of an x-ray, the doctor gets to malformation. Then, a hypoallergenic drug is introduced into the lumen of the vessels, which fills all the available space and blocks the blood flow in this area. Unfortunately, this technique does not provide an absolute guarantee that the vessel is completely obliterated. 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 altered vessels without damaging healthy tissue. The process of sclerosis of blood vessels on average takes several months. The advantage is the complete absence of complications from the nervous system. But there are limitations to using this method:
1. The total diameter of the vessels should not exceed three centimeters.
2. There should be no history of strokes or other hemorrhages. Since the thin wall may not withstand and tear in the gap between the procedure and the final sclerosis of the malformation.