Description
Anencephaly is a neural tube defect (a disorder involving incomplete
development of the brain, spinal cord, and/or their protective coverings). The neural tube
is a narrow sheath that folds and closes between the 3rd and 4th weeks of pregnancy to
form the brain and spinal cord of the embryo. Anencephaly occurs when the
"cephalic" or head end of the neural tube fails to close, resulting in the
absence of a major portion of the brain, skull, and scalp. Infants with this disorder are
born without both a forebrain (the front part of the brain) and a cerebrum (the thinking
and coordinating area of the brain). The remaining brain tissue is often exposed--not
covered by bone or skin. The infant is usually blind, deaf, unconscious, and unable to
feel pain. Although some individuals with anencephaly may be born with a rudimentary brain
stem, the lack of a functioning cerebrum permanently rules out the possibility of ever
gaining consciousness. Reflex actions such as respiration (breathing) and responses to
sound or touch may occur. The cause of anencephaly is unknown. Although it is believed
that the mother’s diet and vitamin intake may play a role, scientists believe that
many other factors are also involved.
Treatment
There is no cure or standard treatment for anencephaly. Treatment is
supportive.
Prevention
Recent studies have shown that the addition of folic acid to the diet of
women of child-bearing age may significantly reduce the incidence of neural tube defects.
Therefore it is recommended that all women of child-bearing age consume 0.4 mg of folic
acid daily.
Prognosis
The prognosis for individuals with anencephaly is extremely poor. If the
infant is not stillborn, then he or she will usually die within a few hours or days after
birth.