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Spina bifida

Spina bifida

Spina bifida

Spina bifida, which literally means “cleft spine,” is characterized by the incomplete development of the brain, spinal cord and/or meninges (the protective covering around the brain and spinal cord). It is the most common neural tube defect in the United States affecting 1,500 to 2,000 of the more than four million babies born in the country each year.

Types of spina bifida

There are four types of spina bifida:

  • Occulta is the mildest and most common form in which one or more vertebrae are malformed. The name “occulta,” which means “hidden,” indicates that a layer of skin covers the malformation, or opening in the vertebrae. This form of spina bifida, present in 10–20 percent of the general population, rarely causes disability or symptoms.
  • Closed neural tube defects are a diverse group and occur when the spinal cord is marked by malformations of fat, bone or meninges. In most instances there are few or no symptoms; in others the malformation causes incomplete paralysis with urinary and bowel dysfunction.
  • Meningocele occurs when spinal fluid and meninges protrude through an abnormal vertebral opening; the malformation contains no neural elements and may or may not be covered by a layer of skin. Some individuals with meningocele may have few or no symptoms while others may experience such symptoms as complete paralysis with bladder and bowel dysfunction.
  • Myelomeningocele is the most severe and occurs when the spinal cord/neural elements are exposed through the opening in the spine, resulting in partial or complete paralysis of the parts of the body below the spinal opening. The impairment may be so severe that the affected individual is unable to walk and may have bladder and bowel dysfunction.

Complications of spina bifida

Complications of spina bifida can range from minor physical problems with little functional impairment to severe physical and mental disabilities. It is important to note, however, that most people with spina bifida are of normal intelligence. Spina bifida’s impact is determined by the size and location of the malformation, whether it is covered, and which spinal nerves are involved. All nerves located below the malformation are affected to some degree; therefore, the higher the malformation occurs on the back, the greater the amount of nerve damage and loss of muscle function and sensation.

In addition to abnormal sensation and paralysis, another neurological complication associated with spina bifida is Chiari II malformation – a condition common in children with myelomeningocele – in which the brain stem and the cerebellum (hindbrain) protrude downward into the spinal canal or neck area. This condition can lead to compression of the spinal cord and cause a variety of symptoms, including difficulties with feeding, swallowing and breathing control; choking; and changes in upper arm function (stiffness, weakness).

Chiari II malformation may also result in a blockage of cerebrospinal fluid, causing a condition called hydrocephalus, which is an abnormal buildup of cerebrospinal fluid in and around the brain. Cerebrospinal fluid is a clear liquid that surrounds the brain and spinal cord. The buildup of fluid puts damaging pressure on these structures. Hydrocephalus is commonly treated by surgically implanting a shunt – a hollow tube – in the brain to drain the excess fluid into the abdomen.

Some children will have a tethered cord, which prevents movement of the spinal cord during growth. It can cause pain, increasing paralysis and spasm in lower extremities. In these cases, a tethered cord release may be required.

Some newborns with myelomeningocele may develop meningitis – an infection in the meninges. Meningitis may cause brain injury and can be life-threatening.

Children with both myelomeningocele and hydrocephalus may have learning disabilities, including difficulty paying attention, problems with language and reading comprehension, and trouble learning math.

Additional problems such as latex allergies, skin problems, gastrointestinal conditions and depression may occur as children with spina bifida get older.

Treatment of spina bifida

Treatment for children with spina bifida at Shriners Hospitals for Children — St. Louis is comprehensive. A full team of neurologists, urologists and other specialists are on hand during clinic days for patients with spina bifida so that families can have all the services they need under one roof and in one day.

Treatments vary with the severity of spina bifida, but the common denominator is that a high level of parent and family involvement is required.

Possible treatments for spina bifida:

  • Shunting for hydrocephalus
  • Treatment of bone deformities and muscle weakness with braces, crutches or walkers to aid ambulation; surgical correction of the hip; or orthopaedic surgery to reduce or prevent spinal deformities such as scoliosis and kyphosis
  • Bladder and bowel training programs which may include clean intermittent catheterization coupled with medication to increase bladder tone and possible urinary diversions
  • For those who have lost sensation and have resulting skin problems, our specialists teach children and families the techniques of frequent skin checks, pressure relief techniques and keeping skin dry and clean, as well as how to treat pressure sores.
  • Occupational and physical therapy are important parts of the treatment plan for children with spina bifida to help with strengthening muscles, training in activities of daily living, use of adaptive equipment, providing orthotic devices and training in use of equipment such as braces, splints, standing frames, walkers and wheelchairs.
  • Family and patients are trained to recognize the symptoms of shunt malfunction such as lethargy, nausea, vomiting, headaches, irritability and decreased strength in arms and hands.
  • Nutrition is also an important part of the treatment plan, encouraging a diet high in fiber, fluids and weight control.

* Information for this page comes from the National Institutes of Health Spina Bifida Fact Sheet.

   
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