CM is a boney malformation at the opening of skull that can cause increased pressure on the lower part of the brain and the spine.  In some cases, this causes the spinal column to curve and create a Scoliosis.

The surgical treatment for this problem aims to remove this pressure.  Bone is taken from the back of the skull and neck.  It is also standard for surgeons to open up the dura to relieve pressure.  Dura, short for dura mater, is a thick lining that surrounds the brain and spinal cord creating a closed system.  Coursing through this system is spinal fluid that regulates pressure and cushions these vital structures.

Though it is patched afterward, opening the dura is risky because it makes this protected system vulnerable to contamination such as meningitis or leaks that can create a dangerous disruption of the pressure system.  Their research indicates that this part of the procedure may not always be worth the risk.

They looked at patients with Scoliosis and CM who had surgery at this institution since 1995.  The patients treated until 2003 had their dura opened and after that year the dura was not opened unless the scoliosis progression was unusually rapid.

The degree of scoliosis and neurological symptoms like limb weakness, hyperactive reflexes, and difficulty swallowing were assessed before and after surgery and compared between these two groups.  Their results showed that improvements following surgery were more or less the same with or without dural opening.

There was a slightly higher chance that a second surgery would be needed in the non-dural-opening group but this group also had significantly fewer complications.

This was the first study of it’s kind and the authors encourage other neurosurgeons to compare their results with and without dural opening.  It may be that this part of the surgery isn’t always necessary and they can expose fewer children to these risks.

*Doctors from the Pediatric Neurosurgery Center involved in this study were: TC Hankinson, J Wade, C Shannon, S Ghatan, RCE Anderson, and NA Feldstein

The Annual Pediatric Neurosurgery Section Meeting is a joint venture of the American Association of Neurological Surgeons and the Congress of Neurological Surgeons. (See the full agenda from the December 2009 meeting).

See a related blog about Chiari 1 Malformations: Pediatrics Added to this Year’s IML at the Congress of Neurosurgery Meeting

Degenerative Lumbar Scoliosis (curvature of the spine)

When most people think of scoliosis they think of teenagers, particularly girls. While adolescent idiopathic scoliosis is one common form of scoliosis, there are other types. Degenerative lumbar, or de novo adult, scoliosis is a spinal deformity that typically develops in individuals over 50 years old. Although its cause is not completely understood, it seems to result from the asymmetric degeneration (breakdown) of the spine. As its name implies, degenerative lumbar scoliosis preferentially affects the lumbar (lower) spine, although occasionally the lower part of the thoracic (middle) spine is also involved.

Individuals with degenerative lumbar scoliosis may have no symptoms of the deformity, have mild complaints, or be severely disabled. Common complaints include back pain that is worst with sitting or standing and that usually goes away when the person lies down, electric shock-like pain, numbness, or weakness in one or both legs, or a combination of these symptoms.

The size of the scoliosis may be relatively small or it may be quite large. Studies have shown that, in general, degenerative lumbar scoliosis is a progressive deformity and that the curve may increase in size by between 2 and 6 degrees per year. It is not unusual for patients to have a small scoliosis that is discovered incidentally while evaluating other symptoms.
The treatment of an individual with degenerative lumbar scoliosis depends on many factors and may include physical therapy, epidural or nerve root injections, a laminectomy, or a spinal fusion procedure. Bracing is occasionally recommended for a short period of time but does not play a significant role in the treatment of degenerative lumbar scoliosis.

Despite an initially normal-appearing spine, over time a spinal deformity (scoliosis) develops in about 1 percent of adolescents. AIS typically does not cause any symptoms. Health professionals including pediatricians and nurses try to detect the presence of AIS before the curve becomes large in order to institute treatment, if necessary.

The primary goal of scoliosis screening and treatment is to keep scoliotic deformities from growing so large that they endanger the health of the child, cause symptoms, or are likely to continue to progress through adulthood. Scoliosis of less than approximately 40 degrees is unlikely to cause significant problems in adulthood or to progress significantly once the skeleton has fully matured.

Many people remember being checked for a spinal curvature in middle or high school. The simplest way to detect scoliosis is the forward bending test. This clinical test, in which the subject bends forward as though she is trying to touch her toes but stops with her back parallel to the floor, makes use of the fact that there is usually a posterior chest wall deformity (“rib hump”) associated with scoliosis. Having the patient bend forward makes it easier to see if there is an asymmetry in the chest wall. Other signs of scoliosis include a shoulder imbalance (one shoulder higher than the other) or an asymmetry in the waistline.

If it is thought that an adolescent may have scoliosis the next step is usually evaluation by a spinal specialist. Usually this neurosurgeon or orthopaedic surgeon will order special x-rays that show the entire spine from the base of the skull to the pelvis. These x-rays are the definitive test to determine if, in fact, scoliosis is present.

The treatment of scoliosis is somewhat complex and depends on multiple factors including the patient’s age, skeletal maturity, and body type and the size of the scoliosis. The doctor uses information from published studies to try to estimate the potential of the scoliosis to increase in size before the patient completes her skeletal growth. Some curves, such as those of a small size or in patients very near completion of their growth, may be observed with sequential x-rays. Curves of a moderate size, or in a patient with a significant amount of growth left, may be braced with an external brace. Bracing is generally instituted to try to prevent a further increase in the size of the scoliosis, not to correct the curve and straighten the spine. Patients with curves of large magnitude (the exact size depends on multiple factors) may be recommended to undergo spinal surgery to correct the spinal deformity.

If surgery is necessary for the correction of a scoliotic curve it may be performed from either an anterior (through an incision over the ribcage) or posterior (through an incision in the middle of the back) approach. The decision making and surgical planning are complex and are discussed in detail by the surgeon with the patient and her family.
Depending on the procedure and individual patient factors, most normal activities can be resumed between 3 and 6 months after surgery and competitive sports, except highly physical contact sports, can be resumed within one year of surgery.

Scoliosis is a type of spinal deformity and should not be confused with poor posture.

Four common types of curve patterns seen in scoliosis include the following:

• Thoracic – 90 percent of the curves occur on the right side.
• Lumbar – 70 percent of the curves occur on the left side.
• Thoracolumbar – 80 percent of the curves occur on the right side.
• Double major – curves that occur on the right and left side.

Causes

In most (80 to 85 percent) cases, the cause of scoliosis is unknown – a condition called idiopathic scoliosis. In other cases, scoliosis may develop as a result of degeneration of the spinal disks or as a hereditary condition that tends to run in families.

The abnormal curves of the spine are classified according to their cause, including the following:

• Nonstructural scoliosis (also called functional scoliosis)In this condition, a structurally normal spine appears curved due to one or more underlying conditions (i.e., difference in leg length, an inflammatory condition, etc.). This type of scoliosis is generally temporary and is often relieved when the underlying condition is treated.
• Structural scoliosisThe possible causes of structural scoliosis are numerous, including the following:
  

  Of unknown origin (idiopathic structural scoliosis)

  Disease (i.e., neuromuscular, metabolic, connective tissue, or rheumatoid disease)

  Congenital malformation of one or more vertebrae

  Injury

  Infection

  Abnormal growth or tumor

Symptoms

The following are the most common symptoms of scoliosis. However, each individual may experience symptoms differently. Symptoms may include:

• Difference in shoulder height
• The head is not centered with the rest of the body
• Difference in hip height or position
• Difference in shoulder blade height or position
• When standing straight, difference in the way the arms hang beside the body
• When bending forward, the sides of the back appear different in height

Back pain, leg pain, and changes in bowel and bladder habits are not commonly associated withidiopathic scoliosis. A person experiencing these types of symptoms requires further medical evaluation by a physician.

The symptoms of scoliosis may resemble other spinal conditions or deformities, or may be a result of an injury or infection. Always consult your physician for a diagnosis.

Diagnosis

In addition to a complete medical history and physical examination, x-rays (a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film) are the primary diagnostic tool for scoliosis. In establishing a diagnosis of scoliosis, the physician measures the degree of spinal curvature on the x-ray.

The following other diagnostic procedures may be performed for non-idiopathic curvatures, atypical curve patterns, or congenital scoliosis:

Magnetic resonance imaging (MRI) – a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.

Computed tomography scan (CT or CAT scan) – a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.

Early detection of scoliosis is most important for successful treatment.

Treatment

Specific treatment of scoliosis will be determined by your physician based on:

• Your age, overall health, and medical history
• Extent of the condition
• Your tolerance for specific medications, procedures, or therapies
• Expectations for the course of the condition
• Your opinion or preference

The goal of treatment is to stop the progression of the curve and prevent deformity. Treatment may include:

• Physical therapy
• Instruction in stretching and strengthening exercises to address muscle imbalances.
• Observation and repeated examinations may be necessary to determine if the spine is continuing to curve.
• Bracing may be used when the curve measures between 25 to 40 degrees on an x-ray, but skeletal growth remains. The type of brace and the amount of time spent in the brace will depend on the severity of the condition. When scoliosis is diagnosed at birth or in early childhood, treatment with braces or a body jacket may be required to prevent progression – and careful monitoring is important to discern whether surgery becomes advisable.
• Surgery
• Surgery may be recommended when the curve measures 50 degrees or more on an x-ray and bracing is not successful in slowing down the progression of the curve.

According to the Scoliosis Research Society, there is no scientific evidence to show that other methods for treating scoliosis (i.e., manipulation, electrical stimulation, and corrective exercise) prevent the progression of the disease.

Dr. Angevine’s unusual training reflects his belief that a surgeon with the most comprehensive training is best able to treat spinal disorders effectively.  After completing his residency in neurological surgery at The Neurological Institute of New York, Dr. Angevine became the first neurosurgeon to be admitted to and complete the prestigious fellowship in spine surgery at the Department of Orthopaedic Surgery at Washington University in St. Louis emphasizing the operative treatment of complex spinal deformities.  As a result of this training he brings to each patient the expertise necessary to evaluate and, if required, treat disorders of spinal alignment such as scoliosis or sagittal imbalance.

Dr. Angevine’s special interests include Adult & Pediatric Spinal Deformities, Neuromuscular Spinal Deformities, Complex Revision Spinal Surgery and Global & Regional Sagittal Imbalance.