Spina bifida (Latin: "split spine") are birth defects caused by an incomplete closure (an opening) of one or more vertebral arches (lamina) of the spine, resulting in malformations of the spinal cord. The spinal membranes and spinal cord may protrude through the absence of vertebral arches (called clefts). These malformations fall into three categories: spina bifida occulta, spina bifida cystica (myelomeningocele), and meningocele.
Types of spina bifida
Spina bifida occulta
Occulta is Latin for "hidden." This is the "mildest" form of spina bifida although the degree of disability can vary depending upon the location of the lesion and actually be very severe in some patients.
In occulta there is no opening of the back, but the outer part of some of the vertebrae are not completely closed. The split in the vertebrae is so small that the spinal cord does not protrude. The skin at the site of the lesion may be normal, or it may have some hair growing from it; there may be a dimple in the skin, or a birthmark. People with this form may have incontinence, ambulatory problems, loss of sensation, deformities of the hips, knees or feet and loss of muscle tone. Depending on the location of the lesion, intense pain may occur originating in the lower back, and continuing down the leg to the back of the knee. Other related problems that may occur include a Chiari Malformation most likely type I, scoliosis, and tethered cord. Many people with this form do not even know they have it, or symptoms do not appear until later in life.
Spina bifida cystica (myelomeningocele)
In this, the most serious form, the meningeal membranes that cover the spinal cord and part of the spinal cord protrude through a cleft, forming a sac or cyst, and are clearly visible. In developed countries, this opening is surgically repaired shortly after birth. Additionally, in the U.S. the National Institute of Child Health and Human Development (NICHD) is currently involved in a fetal intervention study. (MOMS).
The sac or cyst not only contains tissue and cerebrospinal fluid but also nerves and part of the spinal cord. The spinal cord is damaged or not properly developed. As a result, there is usually some degree of paralysis and loss of sensation below the damaged vertebrae. The amount of disability depends very much on where the spina bifida is and the amount of nerve damage involved. Many children and adults with this condition experience problems with bowel and bladder control. In approximately 90% of the people with myelomeningocele, hydrocephalus, extra fluid in the ventricles of the brain, will also occur.
Meningocele
In this, the least common form, the outer part of some of the vertebrae are split and the meninges are damaged and pushed out through the opening, appearing as a sac or cyst, which contains both the meninges and cerebrospinal fluid. The nerves are not usually badly damaged and are able to function, therefore there is often little disability present. There are usually no negative long-term effects, although problems have been known to arise.
Causes
Spina bifida is caused by the failure of the neural tube to close during embryonic development. Normally the closure of the neural tube occurs around the thirtieth day after fertilization. However, if something interferes and the tube fails to close properly, a neural tube defect will occur. Neural tube defects include the conditions of anencephaly, encephalocele, and spina bifida.
Spina bifida occurs in the first month of pregnancy, often before the woman knows that she is pregnant, and is associated with ventriculomegaly.
Spina bifida does not follow direct patterns of heredity like muscular dystrophy or haemophilia. Studies show that a woman who has had one child with a neural tube defects such as spina bifida, have about a 3% risk to have another child with a neural tube defect. This risk can be reduced to about 1% if the woman takes high doses (4 mg/day) of folic acid before and during pregnancy.
It is known that women taking certain medication for epilepsy, such as valproic acid, which is a known folate antagonist (with folate being known to reduce the incidence of spina bifida) or carbamazepine can increase the chances of having a child with spina bifida. Women with insulin dependent diabetes have a higher chance of having a child with a neural tube defect, as well. It is more common among those of Gaelic descent than any other ethnic group.
Prevention
There is no single cause of spina bifida nor any known way to prevent it entirely. However the FDA has issued guidelines to help reduce the chances. Doctors and scientists have found that lack of folic acid is a contributing factor of spina bifida. The FDA and UK recommended amount of folic acid for women of childbearing age and women planning to become pregnant is at least 0.4 mg/day of folic acid from at least one month before conception, and continued for the first 12 weeks of pregnancy. Women who have already had a baby with spina bifida or other type of neural tube defect, or are taking anticonvulsant medication should take a higher dose of 4–5 mg/day. As yet it is unknown how or why folic acid helps to prevent spina bifida. Sources of folic acid include: whole grains, fortified breakfast cereals, dried beans, leaf vegetables, fruits.
Effects
Spina bifida results in varying degrees of paralysis, absence of skin sensation, incontinence, and spine and limb problems depending on the severity and location of the lesion damage on the spine. In very rare cases, cognitive problems also result.
Most babies born with the condition will need surgeries to correct spinal, foot or leg problems, shunt surgery to drain fluid from the brain, application of techniques to control bladder and bowel function (such as self-catherization or diapers), and braces or other equipment to assist in walking.
Treatment
There is no cure for spina bifida. To prevent further drying and damage of the nervous tissue and to prevent infection, doctors operate as soon as possible to close the opening on the back, but there is no operation that can fix damaged nerves. During the operation, the spinal cord and its nerve roots are put into place and covered with skin.
Doctors are now experimenting with and evaluating the efficacy of fetal surgery to correct the spina bifida lesion in-utero. In the fourth or fifth month of pregnancy, surgeons have a window of opportunity to perform fetal surgery by opening the mother's abdomen, and entering the uterus to operate on the spina bifida lesion found on the fetus. Skin grafts are used to cover the exposed spinal cord and protect the spinal cord from further damage caused by prolonged exposure to amniotic fluid. It is believed that the fetal surgery will decrease some of the damaging effects of the spina bifida lesion, but it is not a cure. The fetal surgery is currently undergoing NIH clinical trials and the results of these trials and studies (MOMs study at About.com and Official MOMs Website) will not be completed until 2008.
Children with severe spina bifida require long-term, intensive therapy that is designed to encourage development and prevent deterioration in physical abilities and kidney function. Surgery may be needed to close the opening in the spinal column and to treat the associated hydrocephalus, bladder and kidney abnormalities and physical deformities.
Occurrence rates
In Canada, spina bifida occurs in about one in every 1,000 births.
In Western Australia, up until 1996 around 2 children in every 1,000 were born with a neural tube defect. Since 1996, as a result of the folic acid campaign, the figure has dropped to 1.3 children per 1,000 births.
In the United States, spina bifida occurs in about 7 out of 10,000 live births. More children in the U.S. have spina bifida than have muscular dystrophy, multiple sclerosis, and cystic fibrosis combined.
2006-12-05 14:26:39
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answer #1
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answered by SeahawkFan37 5
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Spina bifida is a birth defect in which the bones of the spine (vertebrae) do not form properly around the spinal cord. This can occur anywhere along the spine. Spina bifida is the most common of a group of birth defects called neural tube defects.
Spina bifida develops in a fetus early in pregnancy, often before a woman knows she is pregnant. In the United States, about 1 in every 2,000 children is born with spina bifida. 1 It is one of the most common birth defects, although the rates have steadily declined in recent years.
There are two main types of spina bifida: spina bifida occulta and spina bifida manifesta.
Spina bifida occulta is the mildest and most common form. The spinal defect is hidden under the skin and does not usually cause problems or need treatment. Doctors estimate that 10% to 24% of the general population unknowingly have this spinal defect. 2, 3 In some cases, a dimple, depression, birthmark, or hairy patch forms over the skin where more than one vertebrae is affected. This is referred to as occult spinal dysraphism (OSD).
Spina bifida manifesta is the severe but rare form of this birth defect. It often is associated with nerve damage that can result in problems with walking, bladder control, and coordination. It can be separated into two classes, meningocele and myelomeningocele.
In meningocele, fluid leaks out of the spinal canal, causing a swollen area over the baby's spine.
In myelomeningocele, a segment of the spinal nerves pushes out of the spinal canal against the underside of the skin. The nerves are often damaged. In the worst cases, the skin is open and the nerves are exposed to the outside of the body.
2006-12-05 10:50:08
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answer #2
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answered by cho 1
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Spina bifida (SB) is a neural tube defect (a disorder involving incomplete development of the brain, spinal cord, and/or their protective coverings) caused by the failure of the fetus's spine to close properly during the first month of pregnancy. Infants born with SB sometimes have an open lesion on their spine where significant damage to the nerves and spinal cord has occurred. Although the spinal opening can be surgically repaired shortly after birth, the nerve damage is permanent, resulting in varying degrees of paralysis of the lower limbs. Even when there is no lesion present there may be improperly formed or missing vertebrae and accompanying nerve damage. In addition to physical and mobility difficulties, most individuals have some form of learning disability. The three most common types of SB are: myelomeningocele, the severest form, in which the spinal cord and its protective covering (the meninges) protrude from an opening in the spine; meningocele in which the spinal cord develops normally but the meninges protrude from a spinal opening; and occulta, the mildest form, in which one or more vertebrae are malformed and covered by a layer of skin. SB may also cause bowel and bladder complications, and many children with SB have hydrocephalus (excessive accumulation of cerebrospinal fluid in the brain).
Hope this helps
2006-12-05 10:48:23
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answer #4
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answered by *~*ME*~* 2
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