Encephalocele: Types, Causes, Symptoms And Treatment
The skull is a bone structure that protects our brain very effectively from blows and various injuries, in addition to allowing the brain to have a defined structure. Formation of the neural tube assembly It takes place during the first month of fetal development, closing between the third and fourth week and allowing the brain to be protected by said bone cover.
However, in some cases the neural tube does not close correctly, which can cause part of the material that should be protected to become visible and accessible from the outside or protrude from what should be its protective cover. Is about problems such as spina bifida or, in the case of the brain, encephalocele.
Encephalocele is understood as a congenital malformation in which the skull does not close completely during fetal development, which causes part of the matter and substances that would generally be inside the cranial cavity to protrude through the opening, forming a lump or protrusion in the shape of a bag that contains it outside.
This means that the individual with encephalocele may have part of the brain displaced to the outside of the skull, which implies a high level of danger for their survival due to possible injuries at the neuronal level. In fact, in many cases the fetus does not survive this situation, dying during the development process.
The symptoms that this problem will cause in the baby will vary enormously depending on the type of tissues found outside the skull and the area in which the opening in question is located.
Encephalocele generally presents with cognitive impairment important and developmental delay, hydrocephalus and microcephaly (since part of the neural material expands outside), motor incoordination in voluntary muscles. It can also cause vision, hearing and smell problems, or even seizures.
Encephalocele can be subdivided into different typologies depending on the area in which the protrusion appears or according to the type of material that is part of it.
Types according to the location of the protrusion
The protrusion can be located in different parts of the skull, although it is common in the three areas indicated below.
1. Frontal region
In this area the opening can be found at different heights, including the nose, the eye socket or the forehead. Mostly the sac usually does not include brain tissue, so it is one of the subtypes that has the lowest risk of nerve damage. However, it can cause significant visual and respiratory difficulties that can endanger the life of the child.
2. Parietal region
In the parietal areas the encephalocele tends to be linked to other malformations and neurological disorders, with almost half of the cases presenting intellectual disability. Additionally, speech may be affected.
3. Occipital region
This is the most common type of encephalocele.
In this case, a large part of those affected have normal intelligence or a mild disability, although it is much more likely that neural material will protrude than in the case of a frontal opening. Visual problems are common. It can also occur in the basal part of the skull.
Types according to the protruded material
Another classification could be made taking into account the type of material that protrudes from the skull. In this sense we can find different types, such as the following.
1. Meningocele
In this subtype of encephalocele, only the meninges protrude, so the risk is much lower than in other cases.
The material that protrudes is made up of the meninges and cerebral ventricles, which is more serious than in the previous case and It is usually associated with the presence of hydrocephalus.
3. Encephalomeningocele
In this case, in addition to the meninges also protrudes brain matter which causes a greater risk of neuronal damage and the presence of diverse and more serious symptoms.
4. Hydroencephalomeningocele
Part of the brain including ventricles They protrude outside the cranial cavity next to the meninges being an affectation that can be very severe.
What causes it?
The causes of this alteration are, as occurs with spina bifida, poor closure or the absence of this in the neural tube during the first month of development. In fact, encephalocele sometimes It has been called “bifid skull”, considered the same type of malformation as spina bifida, although at the cranial level. And as with this other disorder, the specific causes that cause the neural tube to fail to close correctly during fetal development are largely unknown.
Despite this, it is considered that there is a link with low levels of folic acid during pregnancy (this being one of the factors most related to this disease), serious infections in the mother or the presence of high fever during this period or substance consumption. Although the possibility of genetic influence is also alleged because families in which it has occurred previously may present a greater risk, this is not decisive.
Encephalocele treatment
Although the neurological consequences that the baby’s condition has caused during its development are not curable, prevention and early treatment They can help improve your situation and prevent further neuronal damage.
For this, surgery is used in order to reposition the protruded matter inside the skull and seal the part that has not closed properly throughout fetal development. It is especially important to perform this surgery if there is an open injury (that is, if there is no skin covering the sac) or if the sac greatly hinders the baby’s breathing.
In some cases, although it depends on the type of material left outside the skull, the protuberances may even be amputated. In the event that hydrocephalus exists, a surgical intervention will also be necessary to solve it.
In addition to that, specific symptoms should be treated that the problem may have caused. Encephalocele is a condition that does not have to be fatal. However, in some cases this affectation can lead to the death of the minor, in which case palliative care will be provided until the minor dies.
Psychologically, it is very useful the presence of psychoeducation and advice to the minor’s environment since it is a condition that can generate painful sensations, fear and sometimes even feelings of guilt in parents.
At the educational level, the special educational needs that those individuals with cognitive impairments such as intellectual disability may present will have to be taken into account.
Prevention
Taking into account that the causes of this type of malformations are unknown It is advisable to adopt preventive strategies that help avoid this and other types of malformation.
As with spina bifida, administering folic acid during pregnancy, maintaining a balanced diet, and ceasing the use of substances such as alcohol, tobacco, and other drugs greatly reduces the risk of the fetus developing suffers from encephalocele.
