Understanding Encephaloceles: A Rare Neural Defect

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Encephaloceles

Encephaloceles are rare disorders that are caused by a neural tube defect in which the bones of the skull fail to close completely. This failure to close allows cerebral spinal fluid, brain tissue, and the meninges to push through the gap and create a sac-like formation protruding from the skull. Encephaloceles differ from meningoceles in that meningoceles contain only the meninges and cerebral spinal fluid, whereas encephaloceles involve brain tissue being pushed through the sac as well. Encephaloceles will often co-occur with other cranial and/or facial abnormalities and may be associated with a syndrome. This result of a neural tube defect most commonly occurs at the midline of the upper part of the skull, at the area …show more content…

The neural tube is the tissue of an embryo that becomes the brain, spinal cord, and the bones that surround them. It is usually an obvious malformation, but at times it can go undetected depending on the size and location of it. They can be diagnosed by doctors in routine ultrasounds and recent research suggests that fetal MRIs may give a more detailed picture of the CNS. A Fetal MRIs usefulness will depend on the mother’s body, the surrounding fluid, the position of the fetus, and how still the fetus is at the time. 1-4 of every 10,000 live births have an encephalocele in the United States, and this anomaly is more common in females than in males. Encephaloceles increase the likelihood of death in utero and only approximately half of the children with encephaloceles survive birth. Unfortunately, the cause is not yet known, however it is believed that it is not due to anything the mother did or did not do during pregnancy. Many researchers believe that multiple genetic and environmental factors cause the development of an encephalocele. Some individuals may have a genetic predisposition to neural tube defects, as encephalocelese have been noted to be more common when there is a family history of these defects. While researchers speculate that certain infections and toxins may be involved in the cause of encephaloceles, there have not been any …show more content…

The surgery will usually take place once the baby has had a chance to adjust to its new environment, and can take place anywhere from within a few days to a few months after birth. The timing of the surgery is dependent upon the size and location of the encephalocele, the associated anomalies, and whether the deficit is skin—covered. The surgery will be performed sooner if there is: no skin covering over the defect, bleeding, airway obstruction, and/or vision impairment. The operation performed is called a ‘craniotomy’ and it involves the neurosurgeon cutting and removing a piece of bone from the child’s skull and then cutting through the membrane that protects the brain. This is necessary because the neurosurgeon has to replace the brain tissue, membranes, and/or fluids that have protruded from the gap in the skull, and remove the sac that was surrounding it. The neurosurgeon will finish the surgery by closing the dura mater and closing the skull with the same piece of bone that they had initially removed. Sometimes it may be necessary for a neurosurgeon to use an artificial plate to repair the skull if there is still a large

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