A Cerebral hemangioma Is a type of vascular malformation characterized by clusters of dilated capillaries.
They usually appear in Areas of the brain and spinal cord, although it may be present in the retina or the skin (Orphanet, 2014).
In the medical literature we can find several terms that refer to the same medical pathology, cerebral hemangiomas (Angioma Alliance, 2016):
- Cavernous hemangioma.
- Cavernous angioma.
- Cerebral cavernous malformation or" Cerebral Cavernous Malformation "-CCM-.
- Cavernoma.
Brain hemangiomas can range in size from just a few millimeters to several centimeters in diameter, depending on the number of vessels Involved. In some cases, those affected may present multiple lesions, while others will not experience a clinical picture (National Institute of Neurological Disorders and Stroke, 2016).
At brain and in the spinal cord , These malformations composed of capillaries of very thin walls, are very fragile and prone to hemorrhage, Causing hemorrhagic strokes, seizures, and a wide variety of neurological deficits (National Institute of Neurological Disorders and Stroke, 2016).
The signs and symptoms produced by this pathology will vary depending on the affected areas and secondary medical complications; however, Some of the most common symptoms include muscle weakness or numbness, difficulty speaking, difficulty understanding others, Severe headache, sensory changes, instability, convulsive seizures, repeated bleeding, etc. (Mayo Clinic, 2015).
Generally, cerebral hemangiomas have a congenital origin and brain imaging techniques are usually used to identify their presence. In the Symptomatic cases, the treatment usually employed is surgery, to eliminate vascular malformation (Jhons Hopkins Medicine, 2016).
What is a cerebral hemangioma?
The Angioma Alliance (2016) points out that a cerebral hemangioma or cavernous angioma is an abnormal grouping of blood vessels at the cerebral level, Spinal or other areas of the body.
In addition, it points out that commonly the angiomas present a structural configuration similar to a raspberry, composed by multiple bubbles (caves), Which contain blood in their interior and are covered with a thin layer of cells (endothelium) (Angioma Alliance, 2016).
Due to both their shape and lack of other supporting tissues, these blood vessels are prone to leakage and hemorrhage, leading to Development of the clinical picture characteristic of this pathology (Genetics Home reference, 2016).
Although cavernous malformations can occur anywhere in the body, they usually produce only significant or major symptoms When they develop in the brain or spinal cord (Genetics Home reference, 2016).
In addition, the clinical picture will vary depending on the number of vascular malformations, location, severity and size. In many cases, this Type of malformations may vary in size and number over time (Genetics Home reference, 2016).
Are brain hemangiomas frequent?
Cavernous hemangiomas or angiomas are a type of cerebral malformation that can occur in any age group and equally in Men and women (National Organization for Rare Disorders, 2016).
Statistical studies show that this pathology occurs in approximately 0.5-1% of the general population, ie approximately 100-200 people (Angioma Alliance, 2016).
As for the age of presentation of the first symptoms, it is frequent that the clinical course begins to develop between the 20 and the 30 years (Angioma Alliance, 2016).
What are the characteristic signs and symptoms of cerebral hemangiomas?
About 25% of those affected by cavernous malformations or cerebral hemangiomas usually do not experience significant signs or health problems Related to this pathology (Genetics Home reference, 2016).
However, in a good part of diagnosed cases, this medical condition can lead to several serious signs and symptoms (Genetics Home Reference, 2016): Convulsive episodes 30% , Neurological deficit 25% , Cerebral hemorrhage 15% , Severe headache 5% (Angioma Alliance, 2016).
- Convulsive Episodes: Epileptic discharges are one of the most frequent symptoms of cavernous malformations. It is common for those affected to come to the Urgencies and after the control of the convulsive episode, the presence of a cerebral hemangioma is discovered. (The Toronto Brain Vascular Malformation Study Group, 2016). Approximately 30% of cases of cavernous malformations present seizures as one of the main symptoms (Angioma Alliance, 2016).
- Deficit and neurological disorders: Many affected can present various neurological alterations resulting from different lesions at the brain and spinal cord. The alterations Neurological disorders include double vision, muscle weakness, and even paralysis. Clinical symptoms are generally related to the Where the vascular malformation is located (The Toronto Brain Vascular Malformation Study Group, 2016). The Neurological deficits HE Present in approximately 25% of cases of cerebral hemangioma (Angioma Alliance, 2016).
- Cerebral haemorrhage: 15% of those affected by a cavernous angioma will experience bleeding or cerebral hemorrhage. In particular, Cerebral hemorrhages Constitute the More severe symptom of this type of pathology. When the bleeding begins, it is usually accompanied by a sudden headache followed by Nausea, altered level of consciousness or development of spontaneous neurological deficits. In these cases, it is essential to Urgency as the life of the affected person is at serious risk if the volume of bleeding is high (The Toronto Brain Vascular Malformation Study Group, 2016).
- Severe headache: About 5% of people diagnosed with cerebral hemangioma go to informal suffering from severe headache or
(The Toronto Brain Vascular Malformation Study Group, 2016).
What is the most serious medical complication of cerebral hemangiomas?
As we have pointed out above, cerebral haemorrhages are the most serious and urgent symptom, since from this one can derive important Transient or chronic neurological deficits.
Due to the absence of support tissues and the fragility of the capillaries that make up the cavernous malformation, they present a high Probability of bleeding.
The Angioma Alliance (2016) notes that a cerebral hemangioma or cavernous angioma can bleed in different ways:
- Mild bleeding : It is possible that the bleeding to occur progressively and slowly within the walls of the same angioma cerebral. Bleeding of small magnitude that does not usually require surgery, but its recurrence can generate important brain injuries and Medullar
- Heavy bleeding : It is also possible that bleeding occurs abundantly within the walls of the cerebral angioma. Bleeds of a high magnitude develop which cause angioma size to increase and exert pressure against adjacent nerve tissues. It usually requires urgent medical intervention as it can generate important neurological deficits.
- Hemorrhage adjacent tissues : The bleeding can break the walls of the angioma and therefore, the blood can Reach the nerve tissues surrounding the angioma.
Although the risk of bleeding depends on the size and severity of the malformation, all cavernous angiomas have a high Probability of bleeding (Angioma Alliance, 2016).
What are the causes of cerebral hemangiomas?
In relation to cerebral hemangiomas, two different forms of presentation of the pathology have been identified: familial and sporadic (Genetics Home Reference, 2016).
Family form
It is a hereditary form of cerebral hemangiomas and is frequently transmitted from father to son. Typically those affected usually have multiple Cerebral cavernous malformations (Genetics Home reference, 2016).
The familial form of cerebral hemangioma represents approximately 20% of the total cases diagnosed and follows a dominant autonomic inheritance. The condition of this form has been associated with a genetic mutation in any of the following genes: CCM1, CCM2 or CCM3 (National Organization for Rare Disorders, 2016).
Specifically, mutations of the CCM3 gene, lead to the development of the most severe form of cerebral hemangioma. Those affected are usually Diagnosed in an early stage of life and present the first hemorrhages in childhood, may also present cognitive alterations, Benign brain tumors, skin lesions, etc. (National Organization for Rare Disorders, 2016).
Sporadic form
Those affected by the sporadic form do not have a family history of the pathology and usually present only an isolated cerebral malformation (Genetics Home reference, 2016).
Experimental investigations have also identified genetic factors related to the development of the sporadic form of hemangiomas Cerebral Genetic mutations that are not heritable have been identified (National Organization for Rare Disorders, 2016).
Therefore, people with an isolated cerebral cavernous malformation have a high probability of sporadic form, while That people with multiple cavernous marlfomations are more likely to suffer from familial form.
Are there pathologies that increase the likelihood of having a cerebral hemangioma?
It should be noted that up to approximately 40% of cases diagnosed with cerebral angiomas sporadically develop in parallel to Another vascular anomaly, namely the Venous angioma (Angioma Alliance, 2016).
The venous angioma or developmental venous anomaly is a venous malformation in which a radial formation of veins can be observed ending in a Central or main that is dilated. When presented in isolation, without cavernous angioma, it usually does not lead to the development of Secondary medical complications (Angioma Alliance, 2016).
In addition to venous angiomas, cerebral hemangiomas may also develop associated with a type of lesion called"malformations Occult vascular diseases,"since they are not visible in some diagnostic tests such as angiograms (Angioma Alliance, 2016).
How are brain hemangiomas diagnosed?
When signs and symptoms compatible with the presence of a cavernous malformation are detected, two diagnostic tests are commonly used (The Toronto Brain Vascular Malformation Study Group, 2016):
- Computed axial tomography (TAC)
- Magnetic Resonance Imaging (MRI)
Both techniques are able to provide us images through brain sections and thus allow medical specialists to locate the Presence of a cerebral angioma. Specifically, magnetic resonance imaging is able to provide us with a view of the malformations hidden Angiograms, providing a high diagnostic accuracy (The Toronto Brain Vascular Malformation Study Group, 2016).
Thus, Magnetic resonance imaging is the standard diagnostic technique In cavernous malformations, since they are not detected with Ease in computed tomography or cerebral angiography (National Organization for Rare Disorders, 2016).
On the other hand, the use of genetic tests allows the identification of genetic mutations related to familial and sporadic forms. The tests Genetics are recommended for those with a family history of the disease or with multiple cavernous lesions (National Organization for Rare Disorders, 2016).
Is there treatment for cerebral hemangiomas?
In the therapeutic approach of cerebral hemangiomas, it is essential to consider the following factors (The Toronto Brain Vascular Malformation Study Group, 2016):
- Presence of neurological dysfunction.
- Bleeding episodes.
- Uncontrolled convulsions.
- Other serious medical symptoms.
Therefore, depending on these factors may be used various approaches, such as the pharmacological to control convulsive episodes and attacks Of severe headaches.
Apart from this, the fundamental treatment of cavernous angiomas is limited to surgical procedures (The Toronto Brain Vascular Malformation Study Group, 2016).
Usually, cerebral hemangiomas are treated by surgical removal or resection through a craniotomy, or cranial opening.
Although this type of microsurgery is safe thanks to the technical advances that allow to locate the injury of precise form interrupting Minimally the rest of functions, also carries some risks such as: paralysis, coma and even death, although they are infrequent.
What is the prognosis of patients affected by a cerebral hemangioma?
If the affected person undergoes a surgical resection procedure, they usually leave the hospital a few days after the procedure and recover A fully functional life.
In spite of this, in cases where there was neurological dysfunction or develops later, it will be necessary to use rehabilitation Physical and / or neuropsychological to recover altered functions or generate compensatory strategies.
Bibliography
- Angioma Alliance. (2016). Venous Angioma . Obtained from Angioma Alliance.
- Angioma Alliance. (2016). Cavernous Angioma Information . Obtained from Angioma Alliance.
- Cavernous Malformations of the Brain (Cavernomas). (2016). Cavernous Malformations of the Brain (Cavernomas) . Obtained from Cavernous Malformations
Of the Brain (Cavernomas). - Hoch J. (2016). Cavernous angioma of the brainstem . Obtained from Angioma Alliance.
- Johns Hopkins Medicine. (2016). Cavernous Malformations (Cavernomas) . Retrieved from Jhons Hopkins Medicine.
- Mayo Clinic. (2015). Cavernous malformations . Obtained from Mayo Clinic.
- NIH. (2016). Cerebral cavernous malformation . Retrieved from the Genetics Home Reference.
- NIH. (2016). Cerebral Cavernous Malformation . Retrieved from the National Institute of Neurological Disorders and Stroke.
- NORD. (2016). Cavernous Malformation . Retrieved from the National Organization for Rare Disorders.
- Orphanet. (2014). Brain cavernous malformations . Obtained from Orphanet.