Spina Bifida

Spina bifida is part of a group of birth defects called neural tube defects. The neural tube is the embryonic structure that eventually develops into the baby's brain and spinal cord and the tissues that enclose them.

Normally, the neural tube forms early in the pregnancy and closes by the 28th day after conception. In babies with spina bifida, a portion of the neural tube fails to develop or close properly, causing defects in the spinal cord and in the bones of the spine.

Spina bifida occurs in various forms of severity. When treatment for spina bifida is necessary, it's done surgically, although such treatment doesn't always completely resolve the problem.

Doctors aren't certain what causes spina bifida. As with many other problems, it appears to result from a combination of genetic and environmental risk factors, such as a family history of neural tube defects and folic acid deficiency.

Although doctors and researchers don't know for sure why spina bifida occurs, they have identified a few risk factors:

Race. Spina bifida is more common among whites and Hispanics.

Sex. Girls are affected more often.

Family history of neural tube defects. Couples who've had one child with a neural tube defect have a slightly higher chance of having another baby with the same defect. That risk increases if two previous children have been affected by the condition.

In addition, a woman who was born with a neural tube defect, or who has a close relative with one, has a greater chance of giving birth to a child with spina bifida. However, most babies with spina bifida are born to parents with no known family history of the condition.

Folate deficiency. Folate (vitamin B-9) is important to the healthy development of a baby. Folate is the natural form of vitamin B-9. The synthetic form, found in supplements and fortified foods, is called folic acid. A folate deficiency increases the risk of spina bifida and other neural tube defects.

Some medications. Anti-seizure medications, such as valproic acid (Depakene), seem to cause neural tube defects when taken during pregnancy, perhaps because they interfere with the body's ability to use folate and folic acid.

Diabetes. Women with diabetes who don't control their blood sugar well have a higher risk of having a baby with spina bifida.

Obesity. Pre-pregnancy obesity is associated with an increased risk of neural tube birth defects, including spina bifida.

Increased body temperature. Some evidence suggests that increased body temperature (hyperthermia) in the early weeks of pregnancy may increase the risk of spina bifida. Elevating your core body temperature, due to fever or the use of saunas or hot tubs, has been associated with increased risk of spina bifida.

If you have known risk factors for spina bifida, talk with your doctor to determine if you need a larger dose or prescription dose of folic acid, even before a pregnancy begins.

If you take medications, tell your doctor. Some medications can be adjusted to diminish the potential risk of spina bifida, if plans are made ahead of time.

Spina bifida may cause no symptoms or only minor physical disabilities. Frequently, it leads to severe physical and mental disabilities.

Factors that affect severity

The severity of the condition is affected by:

  • The size and location of the neural tube defect
  • Whether skin covers the affected area
  • Which spinal nerves come out of the affected area of the spinal cord
  • Range of complications

Complications may include:

Physical and neurological problems. This may include lack of normal bowel and bladder control and partial or complete paralysis of the legs. Children and adults with this form of spina bifida might need crutches, braces or wheelchairs to help them get around, depending on the size of the opening in the spine and the care received after birth.

Accumulation of fluid in the brain (hydrocephalus). Babies born with myelomeningocele also commonly experience accumulation of fluid in the brain, a condition known as hydrocephalus.

Most babies with myelomeningocele will need a ventricular shunt — a surgically placed tube that allows fluid in the brain to drain as needed 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.

Infection in the tissues surrounding the brain (meningitis). Some babies with myelomeningocele may develop meningitis, an infection in the tissues surrounding the brain, which may cause brain injury and can be life-threatening.

Other complications. Additional problems may arise as children with spina bifida get older. Children with myelomeningocele may develop learning disabilities, including difficulty paying attention, problems with language and reading comprehension, and trouble learning math.

Children with spina bifida may also experience latex allergies, skin problems, urinary tract infections, gastrointestinal disorders and depression.

Spina bifida treatment depends on the severity of the condition. Spina bifida occulta often doesn't require treatment at all, but other types of spina bifida do.

Surgery

Meningocele involves surgery to put the meninges back in place and close the opening in the vertebrae. Myelomeningocele also requires surgery, usually within 24 to 48 hours after birth.

Performing the surgery early can help minimize risk of infection that's associated with the exposed nerves and may also help protect the spinal cord from additional trauma.

During the procedure, a neurosurgeon places the spinal cord and exposed tissue inside the baby's body and covers them with muscle and skin. Sometimes a shunt to control hydrocephalus in the baby's brain is placed during the operation on the spinal cord.

Prenatal surgery

In this procedure — which takes place before the 26th week of pregnancy — surgeons expose a pregnant mother's uterus surgically, open the uterus and repair the baby's spinal cord.

Proponents of fetal surgery believe that nerve function in babies with spina bifida seems to worsen rapidly after birth, so it may be better to repair spina bifida defects while you're still pregnant and the baby is still in your uterus (in utero).

So far, children who received the fetal surgery need fewer shunts and are less likely to need crutches or other walking devices. But the operation poses risks to the mother and greatly increases the risk of premature delivery.

Discuss with your doctor whether this procedure may be right for you.

Ongoing care

Treatment doesn't end with the initial surgery, though. In babies with myelomeningocele, irreparable nerve damage has already occurred and ongoing care from a multidisciplinary team of surgeons, physicians and therapists is usually needed. Babies with myelomeningocele may need further operations for a variety of complications.

Paralysis and bladder and bowel problems often remain, and treatment for these conditions typically begins soon after birth. Babies with myelomeningocele may also start exercises that will prepare their legs for walking with braces or crutches when they're older.

Many have a tethered spinal cord — a condition in which the spinal cord is bound to the scar of the closure and is less able to properly grow in length as the child grows. This progressive “tethering” can cause loss of muscle function to the legs, bowel or bladder. Surgery can limit the degree of disability and may also restore some function.

Cesarean birth

Cesarean birth may be part of the treatment for spina bifida. Many babies with myelomeningocele tend to be in a feet-first (breech) position. If your baby is in this position or if your doctor has detected a large cyst, cesarean birth may be a safer way to deliver your baby.

Sacroiliac Joint Disorders

The sacroiliac (SI) joints are formed by the connection of the sacrum and the right and left iliac bones. The sacrum is the triangular-shaped bone in the lower portion of the spine, below the lumbar spine. The sacrum is made up of five vertebrae that are fused together and do not move. The iliac bones are the two large bones that make up the pelvis. As a result, the SI joints connect the spine to the pelvis. The sacrum and the iliac bones (ileum) are held together by a collection of strong ligaments. There is relatively little motion at the SI joints. There are normally less than 4 degrees of rotation and 2 mm of translation at these joints. Most of the motion in the area of the pelvis occurs either at the hips or the lumbar spine. These joints do need to support the entire weight of the upper body when we are erect, which places a large amount of stress across them. This can lead to wearing of the cartilage of the SI joints and arthritis.

What is sacroiliac joint dysfunction?

There are many different terms for sacroiliac joint problems, including SI joint dysfunction, SI joint syndrome, SI joint strain, and SI joint inflammation. Each of these terms refers to a condition that causes pain in the SI joints from a specific cause.

What are the causes of sacroiliac joint dysfunction?

As with most other joints in the body, the SI joints have a cartilage layer covering the bone. The cartilage allows for some movement and acts as a shock absorber between the bones. When this cartilage is damaged or worn away, the bones begin to rub on each other, and degenerative arthritis (osteoarthritis) occurs. This is the most common cause of SI joint dysfunction. Degenerative arthritis occurs commonly in the SI joints, just like other weight-bearing joints of the body.

Another common cause of SI joint dysfunction is pregnancy. During pregnancy, hormones are released in the woman’s body that allows ligaments to relax. This prepares the body for childbirth. Relaxation of the ligaments holding the SI joints together allows for increased motion in the joints and can lead to increased stresses and abnormal wear. The additional weight and walking pattern (altered gait) associated with pregnancy also places additional stress on the SI joints.

Any condition that alters the normal walking pattern places increased stress on the SI joints. This could include a leg length discrepancy (one leg longer than the other), or pain in the hip, knee, ankle, or foot. Patients with severe pain in the lower extremity often develop problems with either the lower back (lumbar spine) or SI joints. Usually, if the underlying problem is treated, the associated lumbar spine or SI joint dysfunction will also improve.

There are many disorders that affect the joints of the body that can also cause inflammation in the SI joints. These include gout, rheumatoid arthritis, psoriatic arthritis, reactive arthritis, and ankylosing spondylitis. These are all various forms of arthritis that can affect all joints. Ankylosing spondylitis is an inflammatory arthritis that always affects the SI joints. It can lead to stiffness and severe pain in the SI joints, due to inflammation in the sacroiliac joints (sacroiliitis). As the disease process continues, the SI joints can fuse together and have no range of motion. Once this occurs, there is no further pain associated with the SI joints. Rarely, bacterial infection can involve the sacroiliac joints.

What are sacroiliac joint dysfunction symptoms?

The most common symptom of SI joint dysfunction is sacroiliac joint pain. Patients often experience pain in the lower back or the back of the hips. Pain may also be present in the groin and thighs. In many cases, it can be difficult to determine the exact source of the pain. Your physician can perform specific tests to help isolate the source of the pain. The pain is typically worse with standing and walking and improved when lying down. Inflammation and arthritis in the SI joint can also cause stiffness and a burning sensation in the pelvis.

How is sacroiliac joint dysfunction diagnosed?

The first step in diagnosis is typically a thorough history and physical examination by a physician. Certain signs can also help differentiate pain coming from the SI joints, lumbar spine, or hips. There are various tests a physician can perform during the physical examination that can help isolate the source of the pain. By placing the patient’s hips and legs in certain positions and applying pressure, the SI joints can be moved or compressed to identify them as a source of pain. Other portions of the examination are to exclude certain possibilities that could mimic sacroiliac disease.

The next step in diagnosis is often plain radiographs (X-rays). The patient may have X-rays of the pelvis, hips, or lumbar spine depending on what the physician finds on the history and physical examination. A computed tomography (CAT or CT) scan may also help in the diagnosis. A CT scan gives a more detailed look at the joint and bones. Both X-rays and CT scans can help identify sacroiliitis. This can appear as sclerosis in the joints. More severe wear in the joints can appear as erosion of the bone around the SI joints. These tests can also look for fusion of the SI joints. A magnetic resonance imaging (MRI) scan can also be helpful. This provides a better evaluation of the soft tissues, including muscles and ligaments. It can also identify subtle fractures that may not be visible on an X-ray. The MRI can identify inflammation in the SI joint by the presence of excessive fluid in the joint. A bone scan can be obtained to help isolate the source of the pain and can be used to identify bony abnormalities. The bone scan can identify areas of increased activity in the bone. This is a nonspecific test and can be positive in cases of arthritis, infection, fracture, or tumors of bone.

A helpful method of diagnosing SI joint dysfunction is by performing an injection that can numb the irritated area, thereby helping to pinpoint the pain source. An anesthetic material (for example, lidocaine [Xylocaine]) can be injected along with a steroid (cortisone) directly into the SI joint. This is usually performed with the aid of an X-ray machine to verify the injection is in the SI joint. The anesthetic and steroid can help relieve the pain from inflammation that is common with SI joint dysfunction. The duration of pain relief is variable, but this is very useful to determine that the SI joint is the source of the pain.

What is the treatment for sacroiliac joint dysfunction?

As stated above, injections into the SI joint can provide both diagnosis and treatment. The duration of pain relief from injection can last from one day to much more long term. The injections can be repeated each month up to three each year. Oral antiinflammatory medications (NSAIDs, ibuprofen [Motrin], naproxen [Naprosyn]) are often effective in pain relief as well. These can be taken long term if the patient does not have any other medical problems that prevent them from taking these medications. Oral steroids (prednisone) are used as well for short periods of time occasionally to treat the inflammation.

Physical therapy can be very helpful. Pain in the SI joint is often related to either too much motion or not enough motion in the joint. A physical therapist can teach various stretching or stabilizing exercises that can help reduce the pain. A sacroiliac belt is a device that wraps around the hips to help stabilize the SI joints, which can also help the SI joint pain. Other options to stabilize the SI joints include yoga, manual therapy, and Pilates.

If other treatments fail and pain continues to interfere with normal activities, surgery might be an option. Surgery for SI dysfunction typically involves a fusion of the SI joints. In this surgery, the cartilage covering the surfaces of the SI joints is removed and the bones are held together with plates and screws until they grow together (fuse). This eliminates all motion at the SI joints and typically relieves the pain. This should be considered only if other less invasive treatments have not been successful.

What is the prognosis of sacroiliac joint dysfunction?

The prognosis of sacroiliac joint dysfunction varies depending on the cause of the dysfunction. When the problem is caused by pregnancy, the prognosis is excellent, as the condition usually improves after pregnancy during the postpartum period. Conditions affecting the sacroiliac joints such as ankylosing spondylitis and psoriatic arthritis are chronic, but excellent treatments are available. These treatments can minimize the SI joint pain and prevent destruction of the joints. Degenerative arthritis affecting the SI joint is also a chronic condition and cannot be reversed, but treatments are generally very effective in improving symptoms.

Is it possible to prevent sacroiliac joint dysfunction

Unfortunately, SI joint dysfunction is not preventable in some people. For many, it is an unfortunate part of the normal aging process. However, the severity can be reduced through treatment with medication, injections, or physical therapy. Maintaining a healthy body weight and good conditioning can reduce the chances of developing SI joint dysfunction and other forms of arthritis. By reducing the loads on the joints, there is less chance for cartilage damage and subsequent arthritis.

Degenerative Spinal Disorders

Degenerative spinal disorders are a group of conditions that involve a loss of normal structure and function in the spine. These common disorders are associated with the normal effects of aging, but also may be caused by infection, tumors, muscle strains, or arthritis.

Pressure on the spinal cord and nerve roots associated with spinal degeneration may be caused by disc displacement or herniation; spinal stenosis, a narrowing of the spinal canal; or osteoarthritis, cartilage breakdown at spinal joints.

Causes

The discs between the bones of the spine are made up of cartilage, fibrous tissue, and water. With age these discs can weaken and may flatten, bulge, break down, or herniate. Disc herniation, a common cause of pain associated spinal degeneration, occurs when the fibrous portion of a disc weakens and the disc nucleus pushes through and puts pressure on nearby nerves. In addition, a degenerating disc also may cause bony growths that can place additional pressure on the spinal cord.

Spinal stenosis, or narrowing of the spinal canal, is a condition potentially more serious than degenerative disc disease. As the spinal canal tightens, the spine and nerves may be significantly compressed and irritated, causing both back pain and pain that radiates to other parts of the body, depending on the location of the pressure on the nerves.

Symptoms

The primary symptoms of degenerative spinal disorders are sharp and/or chronic pain in the back and legs, weakness, limited motion, and sensory loss. If spinal degeneration leads to compression or injury of the spinal cord, weakness and limited motion may increase significantly. Loss of bladder and bowel function and problems with sexual function also may occur as the problem worsens. The specific symptoms often depend on the location of the structural problem in the spine.

Diagnosis

Diagnosis often begins with a spinal x-ray, which does not show the discs but can show other bony changes in the spine. Magnetic resonance imaging (MRI) scans are the primary means of diagnosis because they can show the disc in detail and allow physicians to see the nerves and spinal canal space and how they are affected by the disease. Computed tomography (CT) scans also may be used. However, the diagnosis of back problems, even with MRI, can be difficult by the presence of findings that do not correlate well with a patients symptoms.

Treatment

Treatment for degenerative spinal disorders depends on the severity of the condition. In most cases, the problem is not severe. The first line of treatment is activity modification, oral pain medication, and physical therapy to strengthen the muscles of the back and improve flexibility and range of motion. In addition, epidural injections of steroids or pain medication, may be used to isolate the source of pain and provide temporary pain relief making physical therapy more productive in patients with severe pain. Noninvasive and minimally invasive procedures will provide pain relief for the vast majority of patients.

Surgery may eventually be necessary as a condition progresses. Surgery is indicated in patients with chronic severe pain, nerve deficits, and loss of bladder and bowel control. Also, surgery may be considered in patients who have not responded to less invasive treatment and in patients who have an identifiable structural abnormality that can be effectively corrected. Surgical procedures used vary depending on the type of condition and its severity. In some patients, a herniated disc can be surgically repaired to restore the normal anatomic structure. In other patients the disc that is causing pain or the bone placing pressure on the spinal cord must be removed. In patients with spinal stenosis for example, only surgery to alleviate the pressure on the spinal cord can provide relief of notable duration. The vertebrae may also need to be stabilized through a process called spinal fusion. Metallic devices are used to stabilize the spine, and then bone taken from another part of the body or from a bone bank is implanted to encourage bone to grow across the span. Bone growth may be encouraged with bone morphogenetic protein, a biologic product that stimulates the creation of new bone. The results of surgery usually are excellent and most patients return to normal function in a matter of weeks.