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Infant Toddler Development Training
Module 6, Lesson 4

Neural Tube Defects (NTDs)

Neural tube defects or spina bifida are another of the neurological conditions.

Please refer to NICHCY Spina Bifida for required reading before going further in this section. For very specific information about spina bifida and hydrocephalus, including treatments and supports, you may visit the following two websites:

Neural tube defects (NTDs) are defects of the spine that occur as a result of the neural tube failing to close. This is called spina bifida or myelomeningocele. A spina bifida may be an open defect or a closed (occulta) defect. In spina bifida occulta the spinal cord is not open to the outside. It is estimated that 5 - 10% of the population has a spina bifida occulta that is undetected and causes no problems.

Open defects are referred to as spina bifida cystica (cyst or sac like). Open defects mean that part of the tissues or the spinal cord itself may be outside of the spinal column. The defect is exposed to the outside world and the risk of infection and complications is great unless treated shortly after birth. The treatment is surgical intervention to close the opening.

There are two types of spina bifida cystica: meningocele and myelomeningocele or meningomyelocele. Meningocele is the least common type and results in very little disability. The sac in a meningocele contains tissues, but does not contain nerves or spinal cord making the surgical repair relatively simple.

The second type of spina bifida cystica, myelomeningocele or meningomyelocele, may cause significant disability because the spinal cord itself is outside the spinal column. It is so named as the meninges or the protective covering of the brain and spinal cord, as well as nerves and part of the spinal cord, are outside the body. There is risk of infection and increased intra-cranial pressure. The defect results in movement disorders and bowel and bladder involvement.

These neural tube defects occur from failure of closure of the neural plate to become a closed tube. The open defect allows the spinal cord to be in direct contact with amniotic fluid and eventually to the outside world after delivery. The defect can be isolated to the spinal cord or it can involve bony malformations.

Neural tube defects may be a result of chromosomal deletions and/or a deficiency of folic acid in the mother's diet. Therefore, women, prior to and during child-bearing years, are encouraged to eat foods such as bread fortified with folic acid, green leafy vegetables and liver.

baby in bath tubPrognosis in spina bifida is associated with the location of the defect. If the defect is low around the S-2 or S-3 of the sacral spine, the child is usually able to walk without adaptive equipment. If the defect occurs around the L-4 or L-5 of the lumbar spine, the child will usually be able to walk with crutches or support. If the defect is higher around L-1 or L-2, the child will generally need a wheelchair for mobility. Many specialists are involved in the life of the child and family including orthopedic doctors, therapists, neurologists, urologists, and special educators.

The higher the lesion or defect, the more likely is the associated presence of an Arnold-Chiari malformation. An Arnold-Chiari Malformation is a congenital deformity at the base of the brain in which cerebellar tissue extends into the fourth ventricle of the brain. It is often associated with spina bifida. If an Arnold-Chiari malformation is present, then seizures, learning disabilities, and shunt issues are associated concerns.

Higher lumbar defects are also associated with scoliosis or curvatures of the spine such as kyphosis and lordosis. Kyphosis is a posterior curvature of the thoracic spine usually the result of a disease (lung disease), or a congenital problem. Lordosis is a curvature or bending backward at the lower spine. These are generally accompanied by hip/knee/foot problems, skin problems from immobility, and bowel and bladder incontinence. Seizures, learning disabilities, and shunt issues may also be associated with higher lumbar defects.

Lower lumbar defects do not have the associated scoliosis and hip problems that are associated with the higher lumbar defects. The defects are generally only associated with bowel and bladder problems.

Role of the ITDS

If hydrocephalus is present, the child may require a shunt to drain the cerebral spinal fluid. The ITDS can be alert to infections in children with shunts by noting changes in behavior such as irritability, changes in somnolence, or loss of developmental skills. Many persons who have shunts are allergic to latex.

Think and reflect on what the ITDS should do with a child who has a shunt and may be allergic to latex. What about the child care provider?

What should the ITDS remember about the cognition of children with Spina Bifida?

Consider some ideas of how the ITDS can help the family whose child has Spina Bifida.

 

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