Encephalocele (cranium bifidum), is a

neural tube defect characterized by sac-

like 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.