Conditions Caused by Spina Bifida

Conditions caused by Spina Bifida can range from minor physical problems to severe physical disabilities. The severity of the conditions depend upon the size and location of the defect, whether or not skin covers it, whether or not spinal nerves are exposed, and which spinal nerves are involved. In general, the majority of the nerves located below the defect are affected. This means that the higher the defect on the child’s back, the greater the amount of nerve damage and loss of muscle function and sensation.

The chart below gives a good general description of nerve and muscle function by vertebrae.

 

Vertebrae Muscle & Funtion
CERVICAL
C1-C2
C3 Diaphragm, Breathing
C4 Diaphragm & Shoulder, Breathing & Shoulder Shrug
C5 Deltoid & Biceps, Lift Arms, Sideways, Bend Elbow
C6 Wrist Extensors, Lift Wrist Back
C7 Triceps, Straighten Elbow
C8 Hands & Fingers, Grip Object
THORACIC
T1 Hands & Fingers, Splay Fingers Apart
T2-T5 Chest (intercostal), Allow Ribcage to Move & Breathe
T6-T8 Chest & Abdomen, Ribcage Move &Cough
T9-T12 Abdomen, Cough & Balance
LUMBAR
L1 Hips, Bends Hips
L2 Hips, Bends, Flex Hip Joints
L3 Quadricep & Hip Adductors, Straighten Leg at Knee
L4 Knee & Ankle, Bend Ankle, Draw Foot Back (dorsiflexion)
L5 Ankle & Toe, Lift Ankle & Lift Big Toe
SACRAL
S1 Ankle & Toe, Bend Ankle, Point Toe (plantarflexion)
S2 Toes & Anal, Bladder Sphincter
S3-S5 Anal, Bladder Sphincter

The following conditions frequently accompany the diagnosis of Spina Bifida:

 

Arnold Chiari II Malformation is a condition commonly associated with Spina Bifida. In Arnold Chiari II Malformation, the cerebellum and brain stem are positioned further down into the spinal column than normal, compressing the tissue. Though the percentage of children with myelomeningocele who have the presence of Arnold Chiari II is quite high, the percentage who are symptomatic is actually quite small. The symptoms of Arnold Chiari II Malformation are stridor (noisy breathing), difficulty swallowing, gagging, sleep apnea and muscle weakness. If symptoms are problematic, a decompression surgery may be required to remove part of the skull and relieve the pressure on the cerebellum.

normal vs sb

Hydrocephalus can result from the Arnold Chiari II Malformation blocking the flow of cerebrospinal fluid surrounding the brain and spinal cord. It occurs in about 80% of individuals diagnosed with myelomeningocele. Although the word literally means “water on the brain,” it is actually a build-up of cerebrospinal fluid in and around the ventricular spaces of the brain, causing pressure on the brain itself.  Children with pronounced hydrocephalus usually require a special device called a “shunt” to by-pass this blockage and drain away the excess fluid. The shunt relieves pressure on the brain by removing the excess fluid from the brain and draining it into the abdomen, where it can be eliminated easily. This surgery is often performed before a newborn goes home from the hospital.

Shunts can be very reliable, but blockages or malfunctions can occur which will require replacement or revision. In fact, 30% of shunts fail in the first year and almost 100% fail within 10 years. Headaches, vomiting, seizures, sleepiness, poor performance at work/school or emotional changes can be signs that a shunt has failed.

A fairly new surgical technique for treating hydrocephalus in infants, the ETV/CPC, was developed by Dr. Benjamin Warf to give Ugandan children a treatment option that required less intervention and dependency on the shunt. During this innovative new surgery, neurosurgeons create a pathway to allow cerebrospinal fluid (CSF) to reach the areas around the brain where it is absorbed. Then they perform Choroid Plexus Cauterization (CPC), applying heat to the tissue in the brain that makes the CSF and reducing the amount that is produced. This procedure opens up the possibility that a large portion of patients with hydrocephalus may be able to live life free from shunts, and the associated fear of their malfunction.

Neurogenic Bladder and Bowel occurs when the nerves that carry messages to the brain telling the body to hold or release urine and stool are impaired at or below the lesion site. Incontinence, high bladder pressure, bladder infections, urine reflux into the kidneys, stool leakage and chronic constipation are some of the problems that can result from incomplete nerve messages to the brain. Inability to empty the bladder effectively can result in infections and kidney damage. Management of the bladder is critical since poor management can lead to kidney failure impacting health and lifespan. Inability to effectively empty the bowels can result in chronic severe constipation. Bowel and bladder programs, which can include medication, catheterization, irrigation, and sometimes surgery, are often necessary to achieve a successful continence program.

Tethered Cord occurs when the spinal cord attaches to the scar from the earlier surgery to close the opening in the spine. Because it is attached to something that does not move, the spinal cord is not able to grow in length and keep up with the growth of the child. As a consequence, it stretches abnormally. Tethered cord syndrome may result in a loss of nerve and muscle function in the legs, feet, bowel, and bladder. In addition to producing deformities of the feet and legs, this syndrome also may produce incontinence and paralysis. Another surgery to detach the scar tissue and release the end of the spinal cord may allow the child to regain their prior level functioning and prevent further nerve deterioration.

Lower Limb Paralysis can occur when the nerves are damaged at or below the lesion site. The location of the defect on the spine often indicates the type of devices needed. Generally, the higher the lesion, the greater the level of paralysis to the lower limbs.  Children with a defect high up on the spine and more paralysis will often require a wheelchair, while those with a defect lower on the spine may use crutches or walkers for mobility. Treatment for paralysis usually begins soon after birth. The goal of treatment is to increase mobility, strength and independence. Working with a physical therapist, parents can learn how to exercise the baby’s legs. When the child is old enough to walk, leg braces may be required. Braces can help to support and protect weak muscles or joints and assist with proper alignment of the bones. Various orthopedic surgeries may also be necessary to facilitate correct positioning.

Orthopedic Abnormalities may include dislocated hips, spinal curvatures, clubfeet and tightness of the knee, hip and/or ankle. Along with the physical therapist, the orthopedic surgeon will decide what kinds of surgery, equipment, or braces the child may require as he or she grows. Some orthopedic problems develop over time as a child grows. Spinal curvatures, progressive foot deformities, and contractures are among the most common. Any rapid changes should be referred to the neurosurgeon to make sure there is no underlying neurological cause, like tethered cord. The overall goal of orthopedic treatment is to provide support for normal development and function. It is important for children to maintain as much mobility as possible.

Clubfoot is a deformity in which an infant’s foot is turned inward, often so severely that the bottom of the foot faces sideways or even upward. The goal of treatment is to obtain a functional, pain-free foot that enables standing and walking with the sole of the foot flat on the ground. The most widely used technique in North America and throughout the world is the Ponseti method, which uses gentle stretching and casting to gradually correct the deformity. In the Ponseti method, long plaster casts are used to progressively correct the foot deformity. After the manipulation and casting period, most babies will require a minor procedure (called a tenotomy) to release continued tightness in the Achilles tendon (heel cord), followed by a 3-week casting. By the time the cast is removed, the Achilles tendon has regrown to a longer length and the clubfoot has been fully corrected. Even after successful correction with casting, clubfeet have a natural tendency to recur. To ensure that the foot will permanently stay in the correct position, the child will need to wear a brace (commonly called “boots and bar”) full-time for 3-months, then just at night for 2-3 years.


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