Myelomeningocele (Spina Bifida)

What is spina bifida?

Spina bifida means that the bones that protect the spinal cord have not formed completely while a baby is developing in the mother’s womb. This happens very early in pregnancy.

Spina bifida can happen anywhere along a baby’s back between the head and the hips. It happens most often in the lower back. This area is called the lumbar spine.

Children with spina bifida may have health problems because of the condition. They may be paralyzed (not able to move their legs) or have decreased movement of their legs, changes or loss of feeling in their legs. They may have problems with their bladder and bowel function.

About 1 baby in every 2,500 babies has some form of spina bifida.

What causes spina bifida is not known

Doctors believe the following things are linked to spina bifida:

  • Some spina bifida is found in families, meaning there is a genetic link.
  • What you eat during pregnancy may have an impact on healthy growth of the spinal cord.

Recent research has shown that spina bifida and other neural tube defects are less likely to occur when women get enough folic acid. These defects occur in early pregnancy, often before many women even know that they are pregnant. If there is any possibility of becoming pregnant, it is important to have a well balanced diet rich in folic acid. If you are planning to become pregnant, it is essential that you begin taking folic acid daily, at least 3 to 4 months before you start trying. Talk with your doctor about the right dosage for you. If you have had a pregnancy affected by spina bifida, or a family history of spina bifida, you may require a higher dose of folic acid.

Spina bifida means the spinal cord is not protected

The spinal cord is a thick bundle of nerves that carries messages between the brain and the rest of the body. It floats in a liquid called cerebrospinal fluid (CSF). The CSF is covered by a lining made of 3 thin layers called the meninges (say: meh-NIN-jees). This lining is normally protected by the bones of the back (the vertebrae). In spina bifida, these bones do not form completely. This means that the lining, the CSF, and the spinal cord are not protected.

There are 3 main types of spina bifida

There are 3 main types of spina bifida: spina bifida occulta, meningocele, and myelomeningocele. “Meningo” refers to the lining of the spinal cord. “Myelo” refers to the spinal cord itself. “Cele” means something bulging out.

Spina bifida occulta

Spina bifida occulta occurs when the outer part of the vertebrae (the bones of the spine) have not completely joined, leaving an opening. In this type of spina bifida, the spinal cord and its coverings (the meninges) are not damaged and they do not protrude or bulge through the opening. There may be a dimple, tuft of hair, birthmark, or fatty bulge at the site of the defect. This type of spina bifida is not detected before birth.

Meningocele

A meningocele (say: meh-NING-go-seal) occurs when the outer part of the vertebrae have not completely closed, leaving an opening. The spinal cord itself may not be affected, but its coverings (the meninges) are damaged and pushed through the opening.

With a meningocele, the spinal cord stays inside the back where it belongs. This means that most children with a meningocele will have normal movement and normal feeling in their legs and feet.

Myelomeningocele

A myelomeningocele (say: MY-ell-oh-meh-NING-go-seal) occurs when the outer part of the vertebrae have not completely closed, creating an abnormal opening. With a myelomeningocele, both the covering of the spinal cord (the meninges) and the spinal cord itself are pushed out the opening. Usually they protrude into a covered, fluid-filled sac that has a very thin membrane and can easily split, exposing its delicate contents. This is the most serious form of spina bifida.

Because part of the spinal cord bulges into the sac, most children with a myelomeningocele will have some difficulty with movement and feeling in their legs and feet.

Meningocele and myelomeningocele are usually repaired with surgery

The sac cannot stay outside of the body for a long time. It may tear or get infected. Your baby will have surgery (an operation) to repair the sac within 2 or 3 days of birth. A neurosurgeon will do the operation. A neurosurgeon is a doctor who operates on the brain and spinal cord.

Your baby may need an MRI before her operation, to help the surgeons to better see the spinal cord. An MRI is a machine that takes special pictures of the inside of the body. MRI scan requires a person to be still while the pictures are being taken, and some children need to be given sedation medicine to help them keep still during the tests.

The aim of the operation is to do the following things:

  • Close the skin over the sac.
  • Prevent infection and further damage to the spinal cord.

The operation will NOT bring back movement and feeling to the baby’s legs and feet.

What happens after the surgery

The surgery takes about 4 hours. After the operation, your baby will spend 3 to 4 hours in the Post Anaesthetic Care Unit (PACU) to recover from the anaesthetic (sleep medicine). Then your baby may go back to the neurosurgical unit, or she may need to be briefly monitored in the NICU (neonatal intensive care unit).

Your child will have a bandage on her back for 2 to 3 days. The nurse will check the bandage often. The nurse will also check your child’s temperature, heart rate, blood pressure, breathing, leg movements, and pain.

Your child will have a thin tube in her arm. This is called an intravenous (IV) tube. It lets us give fluids and medicines directly into your child’s bloodstream. Your child will have this IV until she is drinking well.

For the first 2 to 3 days after the operation, your child needs to lie flat in bed. This is to prevent leakage of fluid from around the spinal cord. Do not get your child up until the doctor says this is OK.

The nurse will turn your child from side to side about every 2 to 4 hours. This will help prevent sores that may develop from lying in bed and help prevent any chest problems after surgery.

Your child may feel pain at the operative site. Your child will be given medicine to control the pain. If it does not control the pain, speak to your child’s nurse.

Many people will help look after your baby

There is a big team of people who will be caring for your baby:

  • a neurosurgeon who will do your baby’s surgery
  • a paediatrician, a doctor who will give healthy baby care
  • a urologist, a doctor who will care for your baby’s kidneys and bladder
  • an orthopaedic surgeon who will care for your baby’s bones
  • a nurse who will care for your baby day to day
  • a nurse practitioner who will be involved in the coordination of care and services
  • a physiotherapist who will give your baby exercises to strengthen the legs and feet
  • an occupational therapist who will help with feeding and your baby’s development
  • a social worker who will help you cope with spina bifida, hospitalization, and being a new parent

Going home

Before you take your child home, the surgeon or the nurse will explain how to take care of your child at home. The instructions will include the following:

  • how to take care of your child’s incision (surgical cut)
  • what to do with your child’s stitches or staples
  • how to tell if your child’s incision becomes infected
  • other things to watch for
  • what activities your child is able to do

Taking care of your child’s incision

Watch your child’s incision to make sure it is healing well. It is all right if the incision gets wet in the bath. Your surgeon and a nurse will discuss with you how long to wait before your child can have a bath.

Taking care of your child’s stitches

How your child’s staples or stitches are taken out depends on the type of staple or stitch that was used. Your surgeon or nurse will tell you what type of stitches were used.

  • If your child has staples or stitches that need to be taken out, your family doctor will need to do this. The stitches should be removed about 7 to 10 days after the operation. Staples should be removed about 10 days after the operation. Your doctor will tell you when they need to come out.
  • If staples were used, you will be given a special remover to take to your doctor.
  • If your child has the kind of stitches that dissolve on their own, you can see your family doctor to have the incision line checked, but the stitches will not need to be taken out.

After your child goes home

Tell your child’s doctor if you notice any of the following things. These mean that your child’s incision could be infected:

  • fluid leaking out of the incision
  • fluid collection (bulging) at the incision
  • redness
  • swelling
  • smelly discharge from the incision
  • fever

Following up after the operation

Your surgeon will see your child in a follow-up clinic visit about 6 weeks after you go home. The appointment may be made for you when your child is discharged. If not, call the neurosurgery clinic to make an appointment yourself.

Your baby may have health problems associated with or caused by spina bifida

As your child grows, you should watch for any changes in bladder or bowel function, back pain, trouble walking, changes in movement of limbs, and irritability. If you have any concerns, seek medical attention.

Some health issues are common for children with spina bifida. The most common ones are described here.

Leg function (movement) and sensation (feeling)

In children with spina bifida, the nerves in the spinal canal are often damaged on improperly formed, and therefore they may not able to control the muscles properly or sometimes feel properly.

Bladder problems

With spina bifida, the nerves that tell the bladder to empty and release urine (pee) are often weak or not working. This means you may have to help your baby to pee and empty her bladder.

When your baby is born, we will put a tube inside her bladder through the urethra every few hours to see if your baby can pee on her own and empty the bladder. The urethra is the tube inside the body that carries urine from the bladder to the outside of the body. If your baby is unable to fully empty her bladder, she is at risk for an infection and possibly damage to her kidneys. You may need to learn how to empty your baby’s bladder before you can take your baby home. A urologist will talk to you about this.

Bowel problems

Sometimes the nerves that make the bowels move are weak or not working. The nurse will be assessing how well your baby’s bowels move. The nurse can teach you ideas to help your baby’s bowels to move better.

Hydrocephalus

After their back surgery, babies with spina bifida often have extra cerebrospinal fluid (CSF), a liquid that nourishes and protects the brain, built up around their brain and spinal cord. This is called hydrocephalus. Hydrocephalus is treated with an operation.

If needed, a tube called a shunt is put into the brain during a second surgery. This tube helps to drain CSF from your baby’s head into an area where the CSF is better absorbed, such as the lining of the abdomen (tummy).

For more information, please see  Hydrocephalus and Treatment,  Shunt Revisions, and  Shunt Infections.

Problems with the leg muscles and bones

Sometimes the leg muscles and bones are affected by spina bifida. If your child’s bones have not developed normally, an orthopaedic surgeon will speak to you about correcting this in the future.

Tethered cord

In children with spina bifida, sometimes the spinal cord gets stuck at the defect site. This is called a tethered cord.

Chiari malformation

Nearly all children born with myelomeningocele have a Chiari 2 malformation. This is when the lower part of the brain sits too low in your child’s upper spine (neck area).

There are many resources to help you

There are many resources available to help you and your baby to cope with spina bifida. Please talk to your nurse, nurse practitioner, or social worker to get this information.

Key points

  • Spina bifida means that the bones that protect the spinal cord have not formed completely.
  • In babies with spina bifida, the CSF and the lining of the spinal cord often bulge out of the baby’s back. Sometimes the spinal cord bulges out as well.
  • Your baby will need an operation.
  • Many health care professionals will help to look after your baby. They will talk to you about other problems your baby may have

 

View the original article here, by:

Mary Douglas, RN, KRN, BScN, MN
James Drake, BSE, MB, BCh, MSc, FRCSC
Patricia Rowe, RN, BScN, MN, NP Peds
Sheila Rowed, RN, BScN

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