Dealing With Spina Bifida in Children

Spina bifida is a birth defect that occurs when the spine and spinal cord don’t form properly. It falls under the broader category of neural tube defects. The neural tube is the embryonic structure that eventually develops into the baby’s brain and spinal cord and the tissues that enclose them. Normally, the neural tube forms early in pregnancy, and it closes by the 28th day after conception. In babies with spina bifida, a portion of the neural tube fails to develop or close properly, causing defects in the spinal cord and in the bones of the spine.

Spina bifida can range from mild to severe, depending on the type of defect, size, location and complications. When early treatment for spina bifida is necessary, it’s done surgically, although such treatment doesn’t always completely resolve the problem.

 

 

There are four main types of which include the following:

  • Occulta: This is the mildest form. Most patients have no neurological signs or symptoms. There may be a small birthmark, dimple or tuft of hair on the skin where the spinal defect is. The person may never know they have spina bifida unless a test for another condition reveals it by chance.
  • Closed neural tube defects: In this version, there can be a variety of potential defects in the spinal cord’s fat, bone, or meninges. In many cases, there are no symptoms; however, in some, there is partial paralysis and bowel and urinary incontinence.
  • Meningocele: The spinal cord develops normally, but the meninges, or protective membranes around the spinal cord, push through the opening in the vertebrae. The membranes are surgically removed, usually with little or no damage to nerve pathways.
  • Myelomeningocele: Myelomeningocele is the most severe form of spina bifida. In this condition, the spinal cord is exposed, causing partial or complete paralysis of the body below the opening. The symptoms are outlined in detail below.

Symptoms of Spinal Bifida

Signs and symptoms vary by type and severity. Symptoms can also differ for each person. The most obvious sign for spina bifida occulta might be a tuft of hair or a small dimple or a birthmark at the site of the defect. Meningocele and myelomeningocele, may show the sac poking through the infant’s back. In the case of meningocele, the membranes around the spinal cord push out through an opening in the vertebrae, forming a sac filled with fluid, but this sac doesn’t include the spinal cord.

In myelomeningocele,

  • there’s usually no skin covering, and the spinal cord tissue is out in the open.
  • There may be a thin layer of skin over the sac.
  • The spinal canal remains open along several vertebrae in the lower or middle back.
  • Both the membranes and the spinal cord or nerves protrude at birth, forming a sac
  • Tissues and nerves usually are exposed, though sometimes skin covers the sac.

Other symptoms of myelomeningocele include:

  • Weak leg muscles (in some cases, the infant can’t move them at all).
  • Unusually shaped feet, uneven hips, or a curved spine (scoliosis).
  • Seizures.
  • Bowel or bladder problems.

The nervous system will also be more prone to infections, some of which can be life-threatening.

What Causes Spinal Bifida?

Doctors aren’t certain what causes spina bifida. As with many other problems, it appears to result from lack of folic acid (a type of vitamin B) in the mother’s body or a combination of genetic and environmental risk factors, such as a family history of neural tube defects and folate deficiency.

Also, women who have diabetes that isn’t managed well or who are obese may be more likely to have a child with spina bifida.

Risk factors

Spina bifida is more common among whites and Hispanics, and females are affected more often than males. Although doctors and researchers don’t know for sure why it occurs, they have identified some risk factors:

  • Folic acid: Spina bifida is more likely if a mother does not have enough folic acid during the pregnancy, although the reason is unclear. All women of reproductive age should make sure their folic acid intake is adequate. Since the introduction of folic acid recommendations by doctors, the number of births involving neural tube defects has fallen.
  • Plant proteins, iron, magnesium, and niacin: A low intake of these nutrients before conception may be associated with an increased risk of neural tube defects by two to five times.
  • Family history: If one infant is born with spina bifida, there is a 4 percent chance that a future sibling will have the same condition.
  • Medications: Drugs such as valproate, used to treat epilepsy or bipolar disorder, have been associated with a higher risk of giving birth to babies with congenital defects, such as spina bifida.
  • Diabetes: Women with diabetes who don’t control their blood sugar well have a higher risk of having a baby with spina bifida.
  • Obesity: A woman whose body mass index (BMI) is 30 or above with an increased risk of neural tube birth defects, including spina bifida, has a higher risk of having a baby with spina bifida.
  • Increased body temperature: Some evidence suggests that increased body temperature (hyperthermia) in the early weeks of pregnancy may increase the risk of spina bifida. Elevating your core body temperature, due to fever or the use of saunas or hot tubs, has been associated with a possible slight increased risk of spina bifida.

If you have any of these known risk factors for spina bifida, talk with your doctor to determine if you need a larger dose or prescription dose of folic acid, even before a pregnancy begins. In addition, if you take medications, tell your doctor. Some medications can be adjusted to diminish the potential risk of spina bifida, if plans are made ahead of time.

What are the Complications?

Spina bifida may cause minimal symptoms or only minor physical disabilities. If the spina bifida is severe, sometimes it leads to more significant physical disabilities. Severity is affected by:

  • The size and location of the neural tube defect.
  • Whether skin covers the affected area.
  • Which spinal nerves come out of the affected area of the spinal cord.

This list of possible complications may seem overwhelming, but not all children with spina bifida get all these complications. And these conditions can be treated, they include:

  • Walking and mobility problems.
  • Orthopedic complications.
  • Bowel and bladder problems.
  • Accumulation of fluid in the brain (hydrocephalus).
  • Shunt malfunction.
  • Chiari malformation type II.
  • Infection in the tissues surrounding the brain (meningitis).
  • Tethered spinal cord.
  • Sleep-disordered breathing.
  • Skin problems.
  • Latex allergy.

Other complications may arise as children with spina bifida get older, such as urinary tract infections, gastrointestinal (GI) disorders and depression. Children with myelomeningocele may develop learning disabilities, such as problems paying attention, and difficulty learning reading and math.

How Spina Bifida is Detected

Three tests can check for spina bifida and other birth defects while the baby is still in the womb:

  • Blood test: A sample of the mother’s blood is tested to see if it has a certain protein the baby makes called AFP. If the level of AFP is very high, it could mean the baby has spina bifida or another neural tube defect.
  • Ultrasound: High frequency sound waves bounce off tissues in your body to make black-and-white pictures of the baby on a computer monitor. If your baby has spina bifida, you may see an open spine or a sac poking out of the spine.
  • Amniocentesis: If the blood test shows a high level of AFP but the ultrasound looks normal, your doctor may recommend amniocentesis. This is when your doctor uses a needle to take a small amount of fluid from the amniotic sac around the baby. If there’s a high level of AFP in that fluid, that means the skin around the baby’s sac is missing and AFP has leaked into the amniotic sac.

Sometimes, spina bifida is diagnosed after a baby is born, usually if the mother didn’t get prenatal care or the ultrasound didn’t show anything wrong. The doctor probably will want to get X-rays of the baby’s body and do a magnetic resonance imaging (MRI) scan, which uses strong magnets and radio waves to get more detailed images.

How You Can Prevent Spina Bifida?

The only known way to prevent spina bifida and other neural tube defects is for the mother to have adequate folic acid levels before and during early pregnancy. This does not work in all cases, but studies have shown cases of severe spina bifida could be prevented by adequate folic acid intake.

Folic acid is essential for life. The need for folic acid increases during periods of rapid growth, such as development of a fetus in the womb. Good sources of folic acid include dark green leafs vegetables, spinach, egg yolks, beans, whole grains, orange juice, and citrus fruits.

Treatment Available

Treatment depends on several factors, mainly how severe the signs and symptoms are.

Surgical options includes the following:

  • Surgery to repair the spine: This can be done within 2 days of birth. The surgeon replaces the spinal cord and any exposed tissues or nerves back into the newborn’s body. The gap in the vertebrae is then closed and the spinal cord sealed with muscle and skin.

If bone development problems occur later, such as scoliosis or dislocated joints, further corrective surgery may be needed. A back brace can help correct scoliosis.

  • Prenatal surgery: The surgeon opens the uterus and repairs the spinal cord of the fetus, usually during week 19 to 25 of pregnancy. This type of surgery may be recommended to reduce the risk of spina bifida worsening after delivery.
  • Cesarian-section birth: If spina bifida is present in the fetus, delivery will probably be by cesarean section. This is safer for the exposed nerves.

Surgery can treat a buildup of cerebrospinal fluid in the brain (hydrocephalus). The surgeon implants a thin tube, or shunt, in the baby’s brain. The shunt drains away excess fluid, usually to the abdomen. A permanent shunt is usually necessary.

Physical and occupational therapy includes:

  • Physical therapy: This is vital, as it helps the individual become more independent and prevents the lower limb muscles from weakening. Special leg braces may help keep the muscles strong.
  • Assistive technologies: A patient with total paralysis of the legs will need a wheelchair. Electric wheelchairs are convenient, but manual ones help maintain upper-body strength and general fitness.
  • Leg braces can help those with partial paralysis.
  • Computers and specialized software may help those with learning problems.
  • Occupational therapy: This can help the child perform everyday activities more effectively, such as getting dressed. It can encourage self-esteem and independence.

A child with spina bifida may need help from a number of specialists, including a pediatrician, a neurosurgeon, a urologist, and an orthopedist.

 

Reference

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