University Hospital Southampton NHS Foundation Trust

Initial diagnosis and management of spina bifida

The central nervous system and spine develop between the 14th and 28th day after conception. Spina Bifida occurs when there is a failure of development of the bony canal which surrounds the brain and spinal cord. The exact cause is not yet known but it is probably connected with both genetic and environmental factors.

What is Spina Bifida?

Spina Bifida is a problem within the spine in which one or more vertebrae (the bones which form the backbone) fail to form properly, leaving a gap or split which causes damage to the central nervous system.

In most babies with spina bifida there is a sac, rather like a large blister on the back, covered by a thin layer of skin.

There are two types of Spina Bifida, Meningocele and Myelomeningocele.

In the form of Myelomeningocele (meningomyelocele), the most serious and more common of the two forms of Spina Bifida, the cyst not only contains tissue and cerebro-spinal fluid but also nerves and part of the spinal cord. The spinal cord is damaged or not properly developed.

In the form of Meningocele, the sac contains tissues that cover the spinal cord and cerebro-spinal fluid. This fluid bathes and protects the brain and spinal cord. The nerves are not usually badly damaged and are able to function, therefore there is often little disability present. This is the least common form.

What happens at the delivery?

Babies with spina bifida can be born in the normal way. Some doctors have recommended caesarean section delivery but there is no evidence that this of any benefit to the baby. Immediately following delivery the lower part of the baby will be wrapped in a film to protect the sac. There is a small risk of sac infection or damage if it is not protected in this way. Later the baby will need to be taken to the Neonatal Unit for observation and assessment.

What happens next?

Your baby will be reviewed by the relevant specialists to assess the extent of nerve problems and the timing of an operation. An operation will usually be done in the first 24 to 48 hours after delivery to remove the sac and repair the skin of the baby’s back. A plastic film dressing will usually be placed over the scar. After the operation the baby will be nursed on their tummy to reduce the chance of infection getting to the wound from the contents of the nappy. Your baby will also be treated with antibiotics to help prevent infection.

What problems may affect a baby with Spina Bifida?

The problems the baby has will depend the level of the sac and which nerves are affected. The problems may affect different parts of the body.

How will my baby be assessed for nerve problems?

There may be a number of ways in which Spina Bifida can affect a child. Some of these become more important in later childhood, other problems are more important in the newborn period. These include:

Head growth and Hydrocephalus

After the operation to close the back, it is possible that the fluid surrounding the brain may build up causing the head to increase rapidly in size (Hydrocephalus). If this happens an operation may be required to drain the fluid. Head circumference will be measured daily and if necessary the Neurosurgeons will be asked for advice. Find out further information about Hydrocephalus.

Passing urine and kidney function

Most Spina Bifida babies will have some problem with bladder control. Your baby will need to be on antibiotics at least once a day to prevent urine infection. In the first few days after the delivery the baby will be seen by the Paediatric Urologists who will be responsible for managing these problems.

A bladder and kidney ultrasound scan will be done to see if the baby’s bladder empties completely. If the bladder is not being emptied pressure will build up and may damage the kidneys. It will then be necessary to pass a small tube (catheter) into the bladder several times each day (clean intermittent catheterisation – CIC). To begin with the nurses will do this, but you will soon learn how to do it.

A small number of patients for whom intermittent catheterisation is not feasible would go on to a surgical procedure of a vesicostomy, where the bladder is opened directly onto the skin of the tummy.

At around 3 months of age a test called video-urodynamics will be done to make a more accurate assessment of the way in which the bladder is working and to plan further treatment during the rest of infancy and early childhood.

Muscles and movement

The extent to which your baby’s muscles are affected depends on the level of the sac. Very low lesions may only cause problems with movement of the toes and feet. Higher lesions may result in your baby being unable to move the lower leg or even the whole leg.

Your baby will be assessed by the Orthopaedic team and a decision will be made as to whether any active treatment needs to be started before your baby goes home. Treatment in the first few weeks is likely to be confined to physiotherapy or splinting. Surgery on your baby’s legs or hips may be needed in later life.

What are the long term problems associated with Spina Bifida?

Most children who have Spina Bifida will have some long term problems. Find out more about the long term problems associated with Spina Bifida.

Are there any future risks?

Once there has been as affected pregnancy, there is an increased risk of further spina bifida pregnancies. The risk of an adult with spina bifida having a child with a similar condition is approximately 3% or 1 in 35. Risks may be reduced by taking folic acid in pregnancy and advice should be sought before conception.