Encephalocele: types, causes, symptoms and treatment
The skull is a bone structure that protects our brain with great effectiveness from various blows and injuries, in addition to allowing the brain to have a defined structure. The formation of the neural tube assembly It is carried out 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 finish closing properly, which can generate that part of the material that should be protected is visible and accessible from the outside or protruding from what should be its protective cover. Is about problems such as spina bifida or, in the case of the brain, encephalocele .
- Related article: "Spina bifida: types, causes and treatment"
What is an encephalocele?
Encephalocele is a congenital malformation in which the skull does not close completely during fetal development, which causes part of the material and substances that would generally be inside the cranial cavity to protrude through the opening, forming a bulge or protrusion in the form of sack that contains it on the 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 its 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 that are outside the skull and the area in which the opening in question is located.
Generally the encephalocele has a cognitive deterioration important and developmental delay, hydrocephalus and microcephaly (since part of the neural material expands on the outside), motor coordination in the voluntary muscles. It can also cause vision, hearing and smell problems, or even seizures.
Types of encephalocele
The encephalocele can be subdivided into different types according to the zone in which the protrusion appears or according to the type of material that forms part of it.
Types according to the location of the protrusion
The protrusion may be located in different parts of the skull, although it is common in the three areas indicated below.
1. Front region
In this area the opening can be found at different heights, including the nose, the orbit of the eyes or the forehead. Mostly the sac usually does not include brain tissue , so that it is one of the subtypes that has a lower 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 zones the encephalocele tends to be linked to other malformations and neurological disorders , presenting almost half of the cases intellectual disability. In addition, speech may be affected.
3. Occipital region
It is the most common type of encephalocele .
In this case a large part of those affected have normal intelligence or a slight disability, although it is much more likely that neuronal material protrudes than in the case of a frontal opening. Visual problems often occur . 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 protruding from the skull. In this sense we can find different types, such as the following.
1. Meningocele
In this encephalocele subtype, only the meninges stand out, so that the risk is much lower than in other cases .
2. Hydrocephalus
The protruding material is made up of meninges and cerebral ventricles, which is more serious than in the previous case and usually linked to the presence of hydrocephalus .
3. Encephalomeningocele
In this case in addition to the meninges it also protrudes brain matter , which causes a greater risk of neuronal damage and the presence of different and more severe symptoms.
4. Hydroencephaloeningocele
Part of the brain including ventricles protrude outside the cranial cavity next to the meninges , being an affectation that can have great severity.
What causes it?
The causes of this alteration are, as with spina bifida, a bad closure or the absence of it in the neural tube during the first month of development. In fact, the encephalocele sometimes It has been called "skull bifido" , considering the same type of malformation as spina bifida although at cranial level.And just as with this other disorder, the specific causes that cause the neural tube to fail to close properly 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 in this period or the consumption of substances. Although there is also the possibility that there is genetic influence due to the fact that families in which it has previously occurred may present a greater risk, this is not a determining factor.
- Related article: "How to take care during the first month of pregnancy: 9 tips"
Treatment of encephalocele
Although the neurological consequences that the state of the baby has brought during its development are not curable, prevention and early treatment They can help improve their situation and prevent further neuronal damage.
For this, surgery is used in order to reposition the protruded material inside the skull and seal the part that has not been properly closed throughout the fetal development. It is especially important to perform this surgery if there is an open lesion (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 that is outside the skull, even the protuberances can be amputated. In the case of hydrocephalus, a surgical intervention will also be necessary to solve it.
In addition to that, the specific symptomatology 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 carried out until the minor dies.
Psychologically, it is very useful the presence of psychoeducation and advice to the child's environment , because it is a condition that can generate painful feelings, fear and even sometimes feelings of guilt in the parents.
At the educational level, special educational needs that may be present in those individuals with cognitive impairments such as intellectual disability must 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 to help avoid this and other types of malformation.
As with spina bifida, the administration of folic acid during pregnancy, the maintenance of a balanced diet and the cessation of consumption of substances such as alcohol, tobacco and other drugs greatly reduces the risk of the fetus in development suffer encephalocele.
Bibliographic references:
- Siffel, C .; Wong, L.C .; Olney, R.S. & Correa, A. (2003). Survival of infants diagnosed with encephalocele in Atlanta, 1978-98. Paediatr Perinat Epidemiol.; 17: 40-8.
- Spacca, B .; Amasio, M.E .; Giordano, F .; Mussa, F .; Busca, G .; Donati, P. & Genitori, L. (2009). Surgical management of congenital median perisellar transsphenoidal encephaloceles with an extracranial approach: a series of 6 cases. Neurosurgery; 65 (6): 1140-6.
