A cavernoma is a malformation of a blood vessel. The medical term is haemangioma cavernosum. Basically, a cavernoma represents a so-called hemangioma. The malformation of vessels is in principle possible in all types of tissue. Of primary importance for medicine are those cavernomas that affect the central nervous system of the human organism. The central nervous system primarily includes the spinal cord as well as the brain.
What is a cavernoma?
Malformations of vessels are usually benign. However, with regard to the frequency of occurrence of cavernomas in the population, no definite statements can be made at this time. The reason for this is that no sufficient epidemiological surveys have been conducted to date to investigate the frequency of cavernomas. However, the results of autopsies indicate that corresponding malformations of the vessels in the central nervous system are found in approximately 0.3 to one percent of all cases. However, the detectable cavernomas usually do not show any symptoms and for this reason remain completely unnoticed in many cases. Frequently, the malformations of the vessels are discovered and diagnosed merely by chance, for example in the course of other clinical examinations. Cavernomas can be detected by magnetic resonance imaging, among other methods, and are often found by chance during the procedure. In the majority of cases, a distinction is made between two types of cavernomas, namely cerebral cavernomas and those located in the spinal cord. The latter are also called spinal cavernomas. Cerebral cavernomas are usually benign arteriovenous malformations of the vessels affecting the brain. Only about 50 percent of all cavernomas are conspicuous from a clinical point of view. Cerebral cavernomas are characterized by their appearance as special capillary vessels. These vessels are close together and arranged in a cluster. In addition, no visible vessels supply the capillaries. Such dilated capillary vessels are also called caverns in some cases in medicine. The malformation of the capillary vessels is enclosed by a special connective tissue. There is no nerve tissue inside this tissue.
Causes
According to the current state of medical research, the causes of cavernoma formation are mostly unexplained. From a histologic perspective, the abnormal vessels are characterized by thin walls that are fibrosed. Unlike arteriovenous malformations, no differentiation into venous or arterial cavernomas is possible. In numerous cases, the area of cavernomas shows deposits of substances that are formed during the degradation of blood. These substances are also called hemosiderins. They serve as evidence for hemorrhages that occurred a long time ago. In the majority of cases, genetic causes for the formation of cavernomas are not detectable. However, in some patients suffering from the malformations on the vessels, mutations on the corresponding genes are shown. The reasons for the formation of cavernomas in the brain are also not yet known. However, it is suspected that the malformations are to a certain extent genetically determined. In this case, they are passed on in an autosomal-dominant manner.
Symptoms, complaints, and signs
Cavernomas are associated with a number of typical symptoms and complaints that facilitate diagnosis. For example, epileptic seizures are possible due to the malformations on the vessels. Sometimes there are also neurological deficits, such as sensory disturbances or paralysis. A dangerous complication is cerebral hemorrhage. However, there are no reliable data for the likelihood of hemorrhage. It is estimated that brain hemorrhage occurs in 0.5 to 10 percent of cases. Basically, the individual symptoms of a cavernoma depend primarily on the exact location. They are most often discovered due to epileptic seizures. Other possible symptoms include dysesthesias or paresis. Approximately 80 percent of all cavernomas of the central nervous system are located in a cerebral hemisphere. Only 15 percent develop in the brainstem or cerebellum. Five percent of cavernomas form in the medulla of the spinal cord. It should be noted that approximately half of all cavernomas that occur in the brain do not show any symptoms.In numerous cases, these malformations of the vessels are discovered only by chance.
Diagnosis and course of the disease
In principle, cavernomas can be diagnosed by means of various examination techniques. As a rule, imaging methods play the most important role. For example, magnetic resonance imaging or computed tomography are possible. However, since numerous cavernomas do not cause any symptoms, they are found by chance in many cases during other examinations.
Complications
Cavernoma usually results in various malformations and deformities of the affected person’s vessels. These malformations can thereby lead to various complaints, which, however, depend on the severity. In most cases, there are disturbances of the nervous system or the spinal cord, so that they can cause paralysis and other disturbances of sensibility. The quality of life is considerably limited and reduced by the cavernoma. It is not uncommon for epileptic seizures to occur, which may also be associated with pain. If not treated, in the worst case a cerebral hemorrhage occurs, which can be life-threatening for the affected person. Mental or speech complaints can also occur, so that patients suffer from speech disorders or motor function restrictions. It is not uncommon for them to be dependent on the help of other people in everyday life and unable to carry out many activities independently. As a rule, the cavernoma can be removed with the help of a surgical procedure. There are no particular complications. If the cavernoma can be removed well, the symptoms usually disappear again and there is no reduction in life expectancy.
When should you go to the doctor?
Cavernoma can take a fatal course without medical attention. Therefore, a doctor should be consulted immediately at the first signs, irregularities and disorders. If there is a feeling of illness, internal weakness or loss of performance, there is already cause for concern. If there are signs of failure or various dysfunctions of the organism, a doctor must be consulted. If epileptic seizures occur, an emergency physician is required. In addition, first aid measures must be initiated by persons present to ensure survival. Subsequently, various medical examinations are needed to determine the cause of the seizure. If circulatory disturbances are noticed, this indication should be discussed with a physician. If there is a racing heart, a feeling of pressure in the organism, unsteadiness of gait or a collapse, the affected person needs help. A sudden loss of existing muscle strength or twitching of the muscles should be investigated and treated. If disturbances or loss of consciousness occur, an ambulance service must be alerted. Intensive medical care must be provided as soon as possible, as this is a life-threatening medical condition for the affected person. If untraceable bleeding occurs, increased bruising under the skin or bruising is noticed, a visit to the doctor is necessary. In the case of a cavernoma, increased discomfort occurs in the area of the back. Therefore, a doctor should be consulted as soon as irregularities are noticed in this region of the body.
Treatment and therapy
The type of treatment for cavernomas depends primarily on their location. If the cavernoma triggers bleeding or epileptic seizures, the malformations on the vessels are often removed during surgery. If cavernomas remain asymptomatic and are discovered more by chance, surgical intervention is often delayed. Symptomatic vascular malformations are removed by surgery in most cases if they are easily accessible. If the cavernomas are located in the brain and are asymptomatic, a wait-and-see approach is often used.
Outlook and prognosis
The prognosis and outlook of cavernomas vary widely depending on their precise location. Of particular interest are cavernomas of the brain, so-called cerebral cavernomas. Again, superficial from deep vascular malformations are distinguished. While superficial cavernomas are located at the cerebral cortex, where they may occasionally trigger epileptic seizures, deep cavernomas are less advantageously located and sometimes near the brain stem.Such cavernomas near the brainstem can be very dangerous, as they can affect the respiratory center if enlarged or hemorrhaged. Most cerebral cavernomas are “silent” incidental findings on imaging of the head, so they are asymptomatic. Thus, prognosis is determined by the exact number, anatomic location, and risk of bleeding into the cavernomas. Deep brainstem cavernomas require surgical treatment in specialized centers, and sometimes they are completely inoperable. Thus, the prognosis for superficial cavernomas, which are located on the cerebral cortex, is generally good because they rarely bleed and thus rarely cause symptoms. For deep-seated cavernomas, the outlook is more mixed, as they generally bleed more frequently and usually occur near anatomically sensitive brain regions. The indication and surgical options ultimately determine the prognosis for the patient.
Prevention
Currently, there are no known measures to prevent cavernomas because their causes are unclear.
Follow-up
There are usually very few aftercare measures available to the affected person with cavernoma. First and foremost, early detection of the disease is important in this condition to prevent further complications and other discomfort for the affected individual. The sooner a doctor is consulted in the case of a cavernoma, the better the further course of the disease usually is. Most patients require surgical intervention, with the number of procedures depending heavily on the symptoms. After such an operation, the affected person should in any case rest and take care of his body. Efforts or physical and stressful activities should be refrained from in order not to put unnecessary strain on the body. As a rule, no further aftercare measures are necessary afterwards. In the case of a cavernoma in the brain, however, regular checks by a doctor are very useful. It is not uncommon that the psychological support of the affected person by his or her own family is also very important, so that depression or other psychological upsets are not prevented. Possibly, a cavernoma also limits the life expectancy of the affected person.
What you can do yourself
After a cavernoma is diagnosed, people can take some steps themselves to help recovery and avoid complications. Depending on the type of treatment, for example, general measures such as rest, bed rest and a change in lifestyle are recommended. It makes sense to lead a healthy lifestyle with a balanced diet, exercise and avoid stress. However, if serious complications occur, such as convulsions or epileptic seizures, an emergency physician must be called. From then on, the affected person should take measures to avoid accidents and resulting injuries. The doctor in charge can best answer which steps to take in detail. He or she may also refer the patient to a specialized clinic. In addition, the affected vessel should be checked regularly in order to be able to react quickly in the event of any complications. In some cases, those affected suffer from psychological problems. Anxiety and upset are best addressed with the support of a therapist. If the cavernoma occurs in conjunction with another disease, further measures must be taken, such as a comprehensive physical examination.
