Encephalocele (cranium bifidum), is a neural tube defect characterized by saclike protrusions of the brain and the membranes that

cover it through openings in the skull. These defects are caused by failure of the neural tube to close completely during fetal development.

Occurrence
Encephaloceles occur rarely, at a rate of one per 5,000 live births worldwide. Encephaloceles of the back of the head are more common in Europe and North America, while encephaloceles on the front of the head more frequently occur in Southeast Asia, Africa, Malaysia, and Russia.

Classifications
Encephaloceles are generally classified as nasofrontal, nasoethmoidal, or naso-orbital, and posterior. If the bulging portion contains only cerebrospinal fluid and the overlaying membrane, it may be called a meningocele. If brain tissue is present, it may be referred to as an encephalomeningocele

Causes Although the exact cause is unknown, encephaloceles are caused by failure of the neural tube to close completely during fetal development. Research has indicated that teratogens (substances known to cause birth defects), trypan blue (a stain used to color dead tissues or cells blue), and arsenic may damage the developing fetus and cause encephaloceles.

Symptoms Encephaloceles are often accompanied by craniofacial abnormalities or other brain malformations. Symptoms may include neurologic problems, hydrocephalus (cerebrospinal fluid accumulated in the brain), spastic quadriplegia (paralysis of the limbs), microcephaly (an abnormally small head), ataxia (uncoordinated muscle movement), developmental delay, vision problems, mental and growth retardation, and seizures.

Treatment
Reparative surgery, generally performed during infancy. Surgery repositions the bulging area back into the skull, removes the protrusions, and corrects the deformities, typically relieving pressure that can delay normal brain development. Occasionally, shunts are placed to drain excess cerebrospinal fluid from the brain.

The goals of treatment include: closure of open skin defects to prevent infection and desiccation of brain tissue removal of nonfunctional extracranial cerebral tissue with water-tight closure of the dura total craniofacial reconstruction with particular emphasis on avoiding the long-nose deformity (nasal elongation that results from depression of the cribiform plate and nasal placode). Without proper management, the long-nose deformity can be more obvious after repair

Proper levels of folic acid have been shown to help prevent such defects when taken before pregnancy, and early in pregnancy. It is recommended that women who may become pregnant take 400 micrograms of folic acid daily.

Recovery Recovery is difficult to predict prior to surgery, and depends on the type of brain tissue involved and location of the encephaloceles. If surgery is successful, and developmental delays have not occurred, a patient can develop normally. Where neurologic and developmental damage has occurred, the specialists will focus on minimizing both mental and physical disabilities.

In general, when the bulging material consists of primarily cerebrospinal fluid, a complete recovery can occur. When a large amount of brain tissue is present in the encephaloceles, there is a higher chance of perioperative complication.