Encephalocele is a birth defect in which the brain, its covering, and protective fluid are found outside of the skull. The root cause of an encephalocele is the failure of surface ectoderm to separate from the neuroectoderm early in embryonic development. In the calvarium, induction of bone formation may be defective, or pressure erosion from an intracranial mass may occur. An encephalocele results from failure of the surface ectoderm to separate from the neuroectoderm. This leads to a bony defect in the skull table, which allows herniation of the meninges or of brain tissue. Neural tube defects appear to be associated with maternal lack of folic acid, and supplementing the diets of all women of childbearing age with folic acid is decreasing the rate of these disorders. The presence of an encephalocele is associated with an increased incidence of death in utero.
Encephaloceles happen more usually in females than in males. Encephalocele is a neural pipe flaw characterized by sac-like protrusions of the mind and the membranes that wrap it through openings in the skull. These defects are caused by bankruptcy of the neural pipe to end totally during fetal growth. Cranial defects that are associated with encephaloceles usually occur in the midline of the skull and appear anywhere from the base of the nose to the base of the occipital bone. Many children with an encephalocele may also have or develop hydrocephalus. Encephaloceles located on the front of the skull are more likely not to contain brain tissue and have better outcomes than those at the back of the head. Occasionally, an encephalocele may go undetected because of its size and location, but typically it is an obvious malformation.
The prognosis for patients with an encephalocele depends on the encephalocele's size, position, and contents, as easily as on associated hereditary abnormalities. A prenatal diagnosis of an encephalocele should inspire an exhaustive hunt for new abnormalities. The hardship of encephalocele varies, depending on the position. Long term treatment depends on extent of child's condition. Currently, the only effective treatments are reparative surgeries following birth. Meningitis is a post-operative concern and antibiotics will be given before and after surgery. Hydrocephalus and shunting will typically be necessary to prevent build-up of cerebral spinal fluid. The timing of the surgery depends on the size, location, associated anomalies and whether the defect is skin covered. Surgery must be performed more quickly if there is no skin covering over the defect or if there is hemorrhage, airway obstruction or impairment of vision.
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