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Anencephaly
Background
Anencephaly is a condition affecting the development of the brain and often the spinal cord with a multitude of associated congenital problems. It is a condition that is non-survivable and the baby is either born dead or dies within a few hours to days of birth.
Anencephaly is what is known as an open neural tube defect in the cephalic region. This basically means that the skull and overlying scalp are absent and a severely abnormal brain structure is open to the outside. There are also significant abnormalities of the face and the neck associated with it. There are various sub-groups depending on the involvement of the neck and associated spina bifida. These abnormal deficits in the coverings of the brain are present very early in gestation, being present by the fourth week after fertilisation. The incidence varies from 1 in 1,000 to just under 7 in 1,000 in some ethnic groups. The incidences in the Western world are reducing in line with increased uptake of folic acid. Females are more prone to this than males. Twins have a higher prevalence than single births.
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