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What is AN ENCEPHALOCELE?

An Encephalocele is an outpouching of brain tissue that originates from a hole in the skull. It is present at birth as a balloon like projection of tissue. While most cases occur in the back of the head, some originate on the face or forehead. When it occurs between the eyes, this can result in hypertelorism or an increase in the distance between the orbits. Other associated brain abnormalities are possible, most notably hydrocephalus (a buildup of cerebrospinal in the brain).

Symptoms SYMPTOMS

An Encephalocele is present at birth and appears as a sac-like protrusion of the brain and membranes through a hole in the skull. Encephaloceles typically form on the face and can result in hypertelorism or large protrusions from the forehead.

The cause of these conditions remains unknown but is not thought to be genetic in most cases. Associated anomalies include microcephaly, hydrocephalus, macrophthalmia, agenesis of the corpus callosum, cortical atrophy and ventricular dilatation. These masses may appear anywhere in the skull and are most common on the back of the head. Those originating in the face account for 15 percent of cases and are classified by their location.

Treatment TREATMENT & MANAGEMENT

Treatment requires cooperation between a craniofacial plastic surgeon and neurosurgeon and involves removal or reduction of herniated tissue and reconstruction of the associated deformities, usually at an early age to prevent further deformity. This often involves reconstruction of a skull defect with bone. In the case of a nasal encephalocele, surgery may involve moving the two orbital cavities closer together with bone grafting to achieve normal interorbital distance and gain adequate nasal projection.

Surgery is performed early in life to prevent progression of the process and prevent damage to the herniated tissue.

Prognosis is dependent upon the amount of tissue in the sac that needs to be removed and the character of the residual brain tissue which remains.