Spine University s Guide to Congenital Cervical Spine Disorders
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Introduction A disorder or disease that someone is born with is called a congenital disorder. In the case of cervical spine disorders, a child may be born with the problem but it may not be noticed for many years, maybe until adulthood. This is possible if the disorder hasn t caused any obvious problems any signs or symptoms of being there. The disorder could be found by accident as some other medical problem is being investigated or because a problem finally does develop. Anatomy What parts make up the spine and neck? Your neck, the cervical spine, is the connection between your skull and brain with the rest of your body. If you have problems with your neck, the effect can be mild and annoying or you can end up with devastating disabilities that affect your body's function. Your neck isn't fully developed when you are born. In fact, it isn't mature until you re about eight years old. You can see this when you look at a newborn. Newborns have little control over their neck. Injuries can happen to a baby's neck when adults are too rough with them and don t support the baby's neck, like with shaken baby syndrome. As your neck develops, it not only becomes more able to protect your spinal cord and support your skull, but it allows you to turn your head from side to side and to bend it forward and backward. The cervical spine is made up of many parts and injuries can happen to any of them, which is why it can seem like sometimes it is very easy to injure your neck. Of course this is even easier with children whose necks aren t fully developed. When doctors refer to the spine, they refer to the location in the spine and the specific vertebrae. The lower part of the spine is the L spine (lumbar spine). The middle part of the spine (thoracic) is the T spine. The neck (cervical spine) is the C spine. There are seven vertebrae in the cervical spine, they are called by number from C1 to C7. The vertebrae that connects to the skull is C1 and the numbers go up as the spine goes down to where C7 joins with the thoracic spine. You may hear the term, upper cervical spine, and this usually means the part of the neck that runs from the very base of the skull to the C2-C3 disc space. In between each vertebrae are two joints that help give the neck its side-to-side flexibility. These joints are called facet joints. The hollow tube formed by the bony ring on the back of the spinal column surrounds the spinal cord as it passes through the spine. The spinal cord is a similar to a long wire made up of millions of nerve fibers. Just as the skull protects the brain, the bones of the spinal column protect the spinal cord. These nerves then branch off each vertebrae, to the left and right. These leave the spinal area through a small tunnel, called the neural foramen. If you imagine the nerves as a telephone electricity wire bringing service into the community, this is the wire that goes through the spinal cord, but the wires that go to each individual home are the ones that run through the neural foramen. Each vertebral level is responsible for a certain set of nerves and nerve function. If the cervical spine injured at a specific level, the nerves at that level will be affected: C1 and C2: Head and neck C3: Diaphragm (which helps control breathing C4: Upper body muscles (including upper arms) C5 and C6: Wrist extensors C7: Triceps: (back of upper arm) C8: Hands 3
Soft tissue in the body is anything that helps build up the foundation of the body that isn't bone. This means the ligaments (strong, fibrous tissue that connect bones and joints), tendons (strong tissue that connects muscle to bone), muscle, and others. The back also is made up of these soft tissues. Diagnosis How will my doctor identify the condition? A disorder or disease that someone is born with is called a congenital disorder. This is different from a disorder or disease that develops later on in life. In the case of cervical spine disorders, a child may be born with the problem but it may not be noticed for many years, maybe until adulthood. This is possible if the disorder hasn't caused any obvious problems any signs or symptoms of being there. The disorder could be found by accident as some other medical problem is being investigated or because a problem finally does develop. An example of a congenital disorder is congenital torticollis, which can happen at birth or just after. This is sometimes called wry neck. Acquired torticollis can happen after a trauma, or accident, so there is a distinction between the two when doctors discuss it. Congenital torticollis is caused by the muscle on one side of the neck pulling or contracting harder than on the other side. If the right side is too tight, the baby s chin will be tilting to the left, and if the muscle pulls on the left, the baby's chin will tilt to the right. The torticollis can be caused by other problems with the cervical spine or it can be caused by problems with the birth. Why a delay in diagnosis? The delay in finding a disorder depends on when the particular part of neck that has the defect matures and if there are any symptoms. However, it's important to know that these problems exist, even without symptoms, because they can be the sign of other problems in the body, such as the kidneys or the heart. This works the other way around as well. There are several syndromes or disorders that often involve cervical spine problems. If a child is diagnosed with one of these problems, he or she should be evaluated for neck problems. Some of these problems include: Down's syndrome Marfan syndrome Osteogenesis imperfecta (brittle bones) Rickets Juvenile rheumatoid arthritis (JRA) Neurofibromatosis An example of a congenital problem that may not be diagnosed right away is Arnold-Chiari malformation, a malformation at the top of the cervical spine that can press on the nerves. People born with spina bifida (a "hole" in the spine) often have this malformation even if their spina bifida is very low on the back. People without spina bifida may have Arnold- Chiari malformation as well. Unless you have a syndrome like spina bifida that will draw attention to the possibility that you might have the malformation, you could go through life well into adulthood without ever knowing. Another example of such a defect is a basilar impression. The upper part of the cervical spine pushes on to the brain stem and spinal cord. The symptoms may not appear until you are in your teens or twenties. Even at that point, it may not be diagnosed right away because it's often mistaken for other disorders like multiple sclerosis or even tumors. Occasionally, problems with the upper cervical spine can be caused by medical conditions that cause inflammation (swelling) of the soft tissues around the spinal cord, such as juvenile 4
rheumatoid arthritis, or an infection after surgery in the skull, such as an adenotonsillectomy (removal of the adenoids and tonsils). Doctors who diagnose neck problems in children need special skills that help them know what to look for and how to look for it. If a child is born with a disorder that's known to cause neck issues, the doctors know to be watchful for signs or symptoms of any known problem. They may take a watch and wait approach, which allows them to keep an eye out without actively doing anything, but they're ready to step in if needed. If watchful waiting isn't an option, doctors can do tests to see if there are any deformities or disabilities. Some children who may fall into a watchful waiting category could be children with Down's syndrome, who have a higher than average risk of having instability of the cervical spine, or neck problems. But, most don't show any symptoms. In one study of 236 children with Down's syndrome, doctors only found instability at the C1-C2 level in 17 percent of the children and only 18 percent of this small group had any symptoms. Some obvious signs and symptoms of neck difficulties are seen with some disabilities. For example, with congenital torticollis, the baby's head would be tilted, with his or her face turning to one side and upwards. With other cervical neck instability, there may be some neurological (nerve) signs, such as uneven use of face muscles, uneven use of arms or legs, inability to walk or be toilet trained, and so on. This would depend on what level is affected between the C1 and C7 vertebrae. X-rays Your doctor will likely order x-rays of your child's neck. X-rays may be needed from several angles to see all the aspects of the vertebrae. Your child may be placed on the side, the back, and the front. While lying on the back, your child's mouth may be opened, so the neck can be seen through the mouth. Computed tomography scans (CT scans). CT scans are special x-rays that allow radiologists to see a cross-section image of the spine, by layer. This gives a closer look to the spine and any malformation that might be there. Other tests The following are other types of tests your doctor may need to do to be sure of their diagnosis. Magnetic resonance imaging (MRI), a scan that uses magnets to take images may be suggested. A myelogram is a study where the doctor injects a special dye into the spinal canal. A CT scan is then done to see where the dye has traveled, allowing the doctors to see the spinal cord and nerve roots An Electromyelogram (EMG) is performed using small needles placed into muscles to measure the electrical activity as they tense and relax. Treatment What can be done for the problem? Treatment of congenital cervical spine disorders in children depends on several things: Diagnosis Effect it has on the child Other medical issues If a watchful waiting approach is taken, it's important for you and your child s doctor to be on the same page about what to watch for and what your child is allowed to do. If your child is older and wants to participate in sports, for example, this should be discussed with your doctor. Many doctors do encourage sports activities, but usually discourage any heavy pressure on the neck. These sports would include diving and gymnastics. Heavy contact sports should be looked at with caution too. 5
Nonsurgical Treatment Medications If the cervical neck instability is caused by inflammation, medications called anti-inflammatories or corticosteroids, may be prescribed to reduce the inflammation. If your child is receiving other treatments, including surgery, sometimes these medications are given before the treatments to make them more successful. Physical Therapy Some conditions, such as congenital torticollis, are usually treated by physical therapy to stretch the neck. Unless your child is showing deterioration with physical therapy, this can be tried for a few months. If there are set backs or there is no improvement after a set amount of time, your doctor will want to do further tests to see if something else may be causing the problem. Some conditions require that the neck be kept as stable as possible. This means your child may need to wear some sort of brace to keep his or her neck from moving too much. Traction pulls on the neck, keeping muscles stretched and might keep vertebrae from fusing together as they are more separated than they would be without the traction. Surgery Occasionally, a situation comes up that needs a halo immobilization. This halo is a metal ring that is fastened to your child's skull. Your child wears a special vest to which the halo is connected and this keeps the head steady. This type of treatment involves a surgery to implant the rods that will keep the halo in place, but once this is done, usually children are discharged home. There is special care to be done where ever the rods enter the skull, to keep the area clean and free from infection, but the hospital staff should teach you how to care for that or help to arrange for nursing care, if it's required. Some conditions must be managed through surgery to keep improve your child's condition or to keep him or her from getting worse. The type of surgery depends on what needs to be corrected. If the problem is one such as Arnold-Chiari malformation, this can be corrected. The surgeon makes a small incision in the back of your child's head to access the skull. Then, he or she removes a small portion of the skull bone and maybe from the top of the vertebrae, to make a larger opening. This relieves any pressure on the nerves. If the problem is due to the spinal cord needing more support from the spine, perhaps the surgeon may fuse, or join together, the vertebrae to make them stronger. The graft is used to support the spine as the spine heals over the graft itself. A fusion can be done by bone graft or with metal. If the fusion is to be done by bone, the bone may be taken from your child. The bone is harvested from the pelvic bones or maybe a rib. If this isn't possible, bone may be taken from a bone bank. Sometimes, using a bone graft isn't possible so metal screws or plates are used. They are fastened to the spine so the bone can grow and fill around them, making the spine stronger. Some surgeries have to be repeated as the child grows or may be needed to make repairs if something happens to the hardware (metal pins, screws, or plates). How long it takes to recover from surgery depends on the type of surgery and if there were any complications. After some surgeries, children may have to wear a brace or collar to help keep their neck supported and stable. Physical therapy may be needed as well. 6
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