What Is Spina Bifida?

Myelodysplasia is a defective development of any part (especially the lower segments) of the spinal cord. They are typically classified into aperta (visible or open) lesions and occulta (hidden or not visible) lesions. It is a neural tube defect that can cause motor and sensory impairments or loss usually at the level of the spinal cord lesions and below. Spina Bifida is commonly referred to as myelomeningocele and is the most severe form of myelodysplasia which is an open spinal cord defect that usually protrudes dorsally. With a myelomeningocele:

1. The spinal canal remains open along several vertebrae in the lower or middle back

2. Both the membranes and the spinal cord or nerves protrude at birth, forming a sac

3. Tissues and nerves usually are exposed, though sometimes skin covers the sac

Doctors aren't certain what causes spina bifida. It's thought to result from a combination of genetic, nutritional and environmental risk factors, such as a family history of neural tube defects and folate (vitamin B-9) deficiency.

How Is Spina Bifida Typically Diagnosed and Treated?

Spina bifida can be screened with maternal blood tests, but typically the diagnosis is made with ultrasound.

Nerve function in babies with spina bifida can worsen after birth if it isn't treated. Prenatal surgery for spina bifida (fetal surgery) takes place before the 26th week of pregnancy. Surgeons expose the pregnant mother's uterus surgically, open the uterus and repair the baby's spinal cord. In select patients, this procedure can also be performed less invasively with a fetoscope through ports in the uterus. This specialized surgery is done at a health care facility that has experienced fetal surgery experts, a multi-specialty team approach and neonatal intensive care. Otherwise, the surgery will be performed within a few days after being born. Performing the surgery early can help minimize the risk of infection associated with the exposed nerves. It may also help protect the spinal cord from more trauma. Treatment for other complications will be started soon after birth as well.

One common complication is hydrocephalus which is the build up of fluid in the cavities (ventricles) deep within the brain. The excess fluid increases the size of the ventricles and puts pressure on the brain. A shunt may need to be surgically placed that allows fluid in the brain to drain into the abdomen. This tube might be placed just after birth, during the surgery to close the sac on the lower back or later as fluid accumulates. Other complications such as weak legs and bladder and bowel problems, — typically begins soon after birth. Chiari II malformation and tethered spinal cord are other common conditions that may occur along with Spina Bifida.

How Does Pediatric Physical Therapy Help Children With Spina Bifida?

Pediatric Physical Therapy may begin in the Intensive care unit to address infant positioning needs and provide parent and family education. Once the child is home, outpatient or early intervention physical therapy should be started to perform a thorough assessment and start a plan of care to help the child gain and maintain mobility as they develop and grow. PT will address any musculoskeletal deformities, motor paralysis, sensory deficits, and muscle tone abnormalities. Two primary orthopedic concerns during infancy are to identify and manage dislocated hips and foot deformities. A mixture of hypotonia, hypertonia, and spastic movements may be present in the limbs. Muscle testing is typically tested by observing position and movements that the infant/child is able to do during positioning and floor play.

Knowledge of Motor levels is important to assess, monitor, and help a child progress with the level of intact musculature innervated by their spinal cord. As an example, an L5 motor level is based on the presence of lateral hamstring muscles with at least grade 3 strength, and either grade 2 gluteus minimus and medius muscles (L4-S1), grade 3 posterior tibialis muscles (L5-S1), or grade 4 peroneus tertius muscles (L4-S1). Therefore, an individual with an L5 motor level has at least antigravity knee flexion and weak hip extension using the hamstrings and may have weak hip abduction, as well as weak plantar flexion with inversion, strong dorsiflexion with eversion, or both.

Weak toe movements may also be present. Hindfoot valgus deformities or calcaneal foot deformities are common as a result of muscle imbalance. Individuals with motor function through L5 should be able to progress to ambulation without orthoses, yet require them to correct foot alignment and substitute for lack of push -off. A gluteal lurch (Trendelenburg gait) is typically evident unless upper limb support is used. Bilateral upper limb support (i.e. forearm crutches) is usually recommended for community distances to decrease energy expenditure, decrease gluteal lurch and trunk sway, maintain symmetric alignment, protect lower limb joints, and improve safety.

As a child grows and progresses with their developmental motor skills, a skilled PT will help the child and parent decide if any orthotics or adaptive equipment would be beneficial based on the child’s functioning motor level.