What is Anencephaly?

What is Anencephaly?

• Anencephaly: A birth defect where most or all of the brain is missing - most die before birth. Usually the associated portions of skull and other tissue are also missing.
• Anencephaly: A malformation of the nervous system caused by failure of the anterior neuropore to close. Infants are born with intact spinal cords, cerebellums, and brainstems, but lack formation of neural structures above this level. The skull is only partially formed but the eyes are usually normal. This condition may be associated with folate deficiency. Affected infants are only capable of primitive (brain stem) reflexes and usually do not survive for more than two weeks. (From Menkes, Textbook of Child Neurology, 5th ed, p247)
  Source - Diseases Database
• Anencephaly: a defect in brain development resulting in small or missing brain hemispheres.
  Source - WordNet 2.1

Anencephaly is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Anencephaly, or a subtype of Anencephaly, affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)

Ophanet, a consortium of European partners, currently defines a condition rare when it affects 1 person per 2,000. They list Anencephaly as a "rare disease".
Source - Orphanet

Name and Aliases of Anencephaly

Main name of condition: Anencephaly

Anencephalus, Absence of a large part of the brain and the skull

Anencephalia
Source - WordNet 2.1

Absence of a large part of the brain and the skull, Anencephalus
Source - Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)

Types of Anencephaly:

Parent types of Anencephaly: Congenital conditions, Brain conditions, Cephalic disorders, Neural tube defect, Spinal Cord Disorders

Organs Affected by Anencephaly:

brain, spinal cord

How many people get Anencephaly?

Incidence (annual) of Anencephaly: less than 4,000 cases (the rate for spina bifida and anencephaly, NWHIC)
Incidence Rate of Anencephaly: approx 1 in 68,000 or 0.00% or 4,000 people in USA [about data]
Incidence of Anencephaly: Each year in the U.S., 4,000 pregnancies are affected by birth defects of the spine and brain, called spina bifida and anencephaly. (Source: excerpt from Folic Acid: NWHIC)

Who gets Anencephaly?

Patient Profile for Anencephaly: Newborns.

How serious is Anencephaly?

Prognosis of Anencephaly: Poor. Newborn usually dies within days.
Prognosis of Anencephaly: 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. (Source: excerpt from NINDS Anencephaly Information Page: NINDS)
Complications of Anencephaly: see complications of Anencephaly

What causes Anencephaly?

Causes of Anencephaly: 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. (Source: excerpt from NINDS Anencephaly Information Page: NINDS)
Class of Condition for Anencephaly: congen.defect
Causes of Anencephaly: see causes of Anencephaly

How is it treated?

Treatments for Anencephaly: see treatments for Anencephaly
Prevention of Anencephaly: see prevention of Anencephaly
Research for Anencephaly: see research for Anencephaly

Society issues for Anencephaly

Hospitalization statistics for Anencephaly: The following are statistics from various sources about hospitalizations and Anencephaly:

• 0.0002% (24) of hospital consultant episodes were for anencephaly and similar malformatins in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
• 100% of hospital consultant episodes for anencephaly and similar malformatins required hospital admission in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
• 29% of hospital consultant episodes for anencephaly and similar malformatins were for men in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
• 71% of hospital consultant episodes for anencephaly and similar malformatins were for women in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
• 8% of hospital consultant episodes for anencephaly and similar malformatins required emergency hospital admission in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
• 1 days was the mean length of stay in hospitals for anencephaly and similar malformatins in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
• 0 days was the median length of stay in hospitals for anencephaly and similar malformatins in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
• 4 was the mean age of patients hospitalised for anencephaly and similar malformatins in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
• 17% of hospital consultant episodes for anencephaly and similar malformatins occurred in 15-59 year olds in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
• 0% of hospital consultant episodes for anencephaly and similar malformatins occurred in people over 75 in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
• 8% of hospital consultant episodes for anencephaly and similar malformatins were single day episodes in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
• 0.00004% (22) of hospital bed days were for anencephaly and similar malformatins in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)