Spina Bifida, Pediatric

Spina bifida occulta

• This type is also called hidden spina bifida because there may be no physical signs of the condition.
• Spina bifida occulta is usually not harmful. The spinal cord and nerves are usually normal. In some cases, however, the spinal cord cannot move freely (tethered) inside the spinal column. This can cause the spinal cord to become stretched, which can result in neurological problems.
• This condition may be diagnosed during a back X-ray that is done for a different reason.

Closed neural tube defects

• This group of defects (occult spinal dysraphism) involves problems with the bone, fat, or other tissues that surround the spinal cord.
• Infants with this type of spina bifida often have a dimple on their lower back. They may also have a red or brown birthmark, a small lump, or a small bunch (tuft) of hair on the lower back.
• In many cases, there are no symptoms. If there are symptoms, they may include bowel and bladder problems or partial inability to move or partial loss of feeling (paralysis).

Meningocele

• A meningocele is a fluid-filled sac that is pushed out of the skin. The sac is usually on the back.
• Often there is no nerve damage with this type of spina bifida.
• Many children have few or no symptoms. If there are symptoms, they include bowel or bladder problems, or paralysis.

Myelomeningocele

• This is the most severe form of spina bifida.
• This type causes the spinal nerves, fluid, and cord to stick out at birth, forming a sac on the back. This results in nerve damage and other disabilities. Children may have partial or complete paralysis, difficulty walking, and bowel and bladder problems.
• Many children with this form of spina bifida also have too much spinal fluid around their brains (hydrocephalus). The spinal fluid cannot drain like it should. This causes the fluid to build up, which causes pressure and swelling.

What are the causes?

What increases the risk?

The risk of spina bifida may be greater if:

• There is a family history of spina bifida.
• During pregnancy, the mother:
  • Did not get enough of a certain type of B vitamin (folic acid).
  • Developed an illness that caused a high fever.
  • Had a medical condition, such as uncontrolled diabetes or obesity.
  • Was exposed to certain medicines that prevent seizures.

What are the signs or symptoms?

Signs and symptoms may include:

• A visible sign on your child's back, such as:
  • A meningocele.
  • An exposed area of spinal cord tissue.
  • A dimple, a birthmark, or an unusual clump of hair.
• Partial to complete paralysis. This can cause difficulty walking.
• Bowel or bladder problems.
• Difficulty feeding, swallowing, or breathing.
• Upper arm stiffness or weakness.
• Learning disabilities or brain damage.
• Seizures.

How is this diagnosed?

In most cases, spina bifida is diagnosed by a test during a routine pregnancy exam before a baby is born, such as:

• An ultrasound.
• A blood test that measures levels of alpha-fetoprotein (AFP). High levels of this protein may mean that your baby has spina bifida.
• A test to screen for certain conditions using a sample of fluid from the sac that surrounds the unborn baby (amniocentesis).

After your child is born, his or her health care provider can diagnose spina bifida based on your child's signs and symptoms. At that time, your child may also have imaging tests such as:

• X-rays.
• CT scan.
• MRI.

How is this treated?

Treatment depends on what type of spina bifida your child has and how severe the condition is. Treatment may include:

• Surgery.
  • Surgery to correct the spinal defect usually takes place during the first few days of life to preserve as much function as possible.
  • More surgeries may be done to manage complications of the disease, such as problems with the brain, hips, feet, or spine. Your child may need a tube (shunt) placed in the skull to drain excess fluid from the brain.
• Devices to help your child move around (assistive devices). Children with spina bifida often need help with moving around (mobility). This help may come from the use of:
  • Crutches.
  • Braces.
  • Wheelchairs.
• A variety of medicines to prevent infections related to spina bifida and to treat complications.
• Physical therapy. This can help to strengthen muscles and reduce pain and other symptoms.
• Bladder and bowel management. Children with spina bifida who cannot control their bowels or bladder may need:
  • A thin, flexible tube placed in the bladder to drain urine (catheterization).
  • A system to manage bowel movements.

Follow these instructions at home:

• Give your child over-the-counter and prescription medicines only as told by your child's health care provider.
• Have your child use assistive devices and do strength and mobility exercises as directed.
• Monitor your child's symptoms. Tell your child's health care provider about any changes in your child's health.
• Keep all follow-up visits as told by your child's health care provider or physical therapist. This is important.

Contact a health care provider if:

• Your child's ability to move around gets worse.
• Your child's bowel and bladder symptoms get worse.
• Your child’s pain gets worse.

Get help right away if:

• Your child develops a bulge on a soft spot (fontanelle) on his or her head.
• Your child has a seizure.

Summary

• Spina bifida is a problem that happens as the spine develops in the womb during early pregnancy (neural tube birth defect).
• Some children have no symptoms at all. Others may have severe paralysis and brain damage. Symptoms depend on the type of spina bifida that your child has.
• Treatment depends on what type of spina bifida your child has and how severe the condition is.