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When a curvature in babies of at least 10-15 degrees is diagnosed between 2 months to 5 years old we refer to it as
Early Onset Scoliosis is the newer term but both terms are still used.
EOS is characterized by curvature of the spine that often corrects over time, although not always. Sometimes it can become quite severe. The key is vigilance. Above all make sure that we are not dealing with some other cause of scoliosis that could be more serious (such as a neurologic tumor or some congenital bony abnormality).
Fortunately Infantile Scoliosis is rare, found in only 1 or 2 people in 100,000. Oddly, it occurs more commonly in Europe than in the US. By definition, it has onset before the age of five. The reverse of adolescent distribution, it occurs more often in boys than in girls, though we don't know why. In fact, as in juvenile and adolescent scoliosis, we consider it idiopathic meaning we don’t know why some people get it and others don’t.
 The most frequent confusing condition would be congenital scoliosis. Congenital scoliosis is a term reserved for overt structural spinal anomalies - an extra piece or missing piece or parts not properly separated. Examples of congenital scoliosis include hemivertebra, bar vertebrae with fused ribs, or other bone structure problem. Simply being present at the time of birth does not land the name ‘congenital’. This term is usually reserved for actual identifiable structure abnormalities.
After congenital scoliosis it is very important to make sure the patient doesn’t have a neurologic problem. It is important to confirm that the patient is neurologically normal. Examination of the abdominal and plantar reflexes in the feet are subtle neurologic signs that may cause the doctor, in certain cases, to order a magnetic resonance imaging (MRI). The MRI scan is not easy to obtain in a very young child and sedation may be necessary to make sure the child lays still throughout the exam.  MRI’s can be used to image any part of the body but in this case are used to evaluate the spinal cord and to exclude neurologic anomalies at the base of the skull which can cause curvatures of the spine. A dimple at the base of the spine can be just a dimple or a part of incomplete formation. You have to check (ultrasound typically).
If there is no obvious bony abnormality that occurred in the development of the vertebrae, no neurologic abnormalities and no other medical condition like and infection than we can fairly safely make the diagnosis of infantile or early onset scoliosis.
An overwhelming majority of Infantile Scoliosis cases are resolved on their own. Therefore, doctors generally use the method of observation to treat this disease. X-rays of the patient are routinely taken to confirm that the spinal disease is not growing. However, certain spinal curves are more stubborn, and worsen with the child’s growth. There are certain findings on x-rays (such as certain right-left rib angle comparisons) that can more-or-less spot the ones that are apt to progress. That does not mean ignore the others. Given a demonstration of failure to correct then corrective plaster casts or braces can be used. Surgery is used as the last resort in very serious cases.
To sum it all up, Infantile Scoliosis or Early Onset Scoliosis generally rectifies itself although sometimes it can progress and cause a severe deformity as the child grows older. Therefore, it is very important to find out the nature of the spinal curve, quickly. Parental awareness leading to early medical intervention and proper early treatment can help prevent severe curvatures which become progressively more difficult to treat.
Juvenile Scoliosis
The Yin-Yang years: 69
(from 6 through 9 y/o)
By definition juvenile scoliosis develops after age 5 (earlier is called infantile or early onset) but before age 10. These curves are idiopathic meaning we don’t know why some people get it and others don’t. The majority of the curvatures are right thoracic curves.
Think of a curve as sharp...
So ) is thought of as > pointing right
& ( is thought of as < pointing left.
It isn’t simply the side to which
the head leans. This naming is required because there may be
several curves stacked one on the other.
In other words, the convex side of the curve names that curve segment as a curve right or left as the case may be. Most thoracic curves are curiously on the right, but sometimes a thoracic curve is left sided. Even though we don’t know why the right sided form is most common, a left version concerns us. It is unusual. We get extra cautious to determine that some subtle neurologic problem is not the cause of the oddity. It is therefore not unusual for a doctor to order an MRI scan of the entire spinal cord to make sure we aren’t missing something.
Clinically, juvenile scoliosis shows slow progression prior to age 10, but after age 10 (when the child starts to grow quickly) more rapid progression may be found. If the curve starts to progress more rapidly earlier the doctor may become suspicious of another cause (in other words it isn’t really idiopathic). Other conditions which can cause scoliosis in this age group are benign bone tumors, infections in either the bone or one of the discs, neurologic problems where the brain meets the spinal cord, small benign tumors of the nerves themselves or even small benign cyst that occur in the spinal cord. A good quality MRI of the Entire Spinal Cord is usually very accurate at picking up any and all of these abnormalities. Sometimes if it is a bony problem a CAT scan may be ordered as a follow-up test. As we mentioned before, a left-sided thoracic curve is a tip-off to the presence of one of these other problems.
On examination:
the doctor asks the patient to bend over in front of him (the Adam’s Bend test). This brings out the appearance of the curves and makes subtle curves easier to see directly. If there is enough of a suspicion on the bend test, x-rays are obtained. X-rays are usually the first test ordered and confirms the presence of a curve or curves! One curve looks “C-shaped” and two curves are often described as “S-shaped”. The doctor can measure the x-ray and come up with a numerical value called the Cobb Angle measured in degrees.
Bracing:
When the curvature either shows clear signs of getting worse or reaches between 25-30 degrees the doctor may suggest bracing. There are two different goals to consider when bracing a child. The first desire is to “cure” the curve! By cure we mean prevent it from getting worse. While the initial brace may improve the Cobb measurement the ultimate success will be measured by having the child grow to full adult height and have a mild curve of less than 40-45 degrees in the thoracic region and 35-40 degrees in the lumbar region.
If curvature progression looks to be unrelenting, then at some point the treating doctor is going to consider surgery. I consider brace treatment not a complete success but a “partial” success” if we keep a curve under control until the child has gotten older and bigger nearer to adult height before we are forced to perform surgery. There is a fine balance between waiting too long and letting the curve get worse making the ultimate surgery difficult and operating too soon when some ultimate loss of adult height might result.
Surgical Treatment Options:
It has been said that up to 25% to 50% of children with juvenile scoliosis will require surgery. But these statistics can be viewed as batting averages. You can improve your odds or worsen them by the decisions you make.
The goals of surgery are to straighten the spine (usually 40-60%) and fuse the spine in the new straightened position so the curvature won’t return. The word fusion often confuses patients. Basically we are taking normally mobile individual mobile vertebrae and placing additional bone (bone graft ) connecting them causing them to grow solid into one solid vertebral bone joining from two vertebra together to 15 or more vertebra together (in severe cases).
Fusions have been done in the spine for over 100 years. Fusions can be done from different approaches. The classical approach is a posterior spinal fusion. The spine is relatively close to the skin at the “back of the back”. Another approach to the spine is the anterior aspect of the spine or the “front of the back”. Just because we are going to the front of the spine the incision for front or anterior surgery is not necessarily the front of the patient. Most approaches to the front of the spine are done through the side or flank of the patient. In some cases we approach both the front and the back of the spine which is known as a “Front-Back” or “Anterior-Posterior”.
The last 50 years however has lead to the era of spinal instrumentation. Spinal Instrumentation are metal implants (customarily either stainless steel or titanium) that are used to straighten the spine and hold the spine still until fusion can take place. 50 years ago in scoliosis the Harrington rod was a major advance in the surgical correction of scoliosis. The instrumentation is useful to help correct a spinal deformity (to take a curved spine and bring into a more normal position). Today we have a wide variety of rods, hooks, wires, screws, plates and other devices to aid in fusion. The instrumentation is not only useful in correcting a curve but also holding the spine still so the fusion can take place more easily. Think of a healing spine fusion as similar to water that has to freeze solid into ice. We know that moving water is harder to freeze than stationary water. This is why rivers usually freeze from the calmer water at the sides and progresses towards the middle more rapidly moving water. The same is true of fusing bone. The more still the bone environment is the more quickly and reliably the bones fuse or “freeze” together.
Before, metal implants, spinal fusion required total body casts and many months in specialized rotating beds. Long after these have been abandoned, horror films revel in their use. Hollywood must own the entire historical collection of these spinal beds. Metal has been truly liberating.
One of the problems with Posterior fusion of the spine in very young individuals (with huge growth force) is that the anterior spine can sustain growth despite the posterior metal. Thus a tight nasty curve may reappear. This is known as a “Crankshaft Phenomenon” . The crankshaft of a automobile engine has a series of tight curves. The spine with “Crankshaft Phenomenon” resembles this. One of the ways to prevent this in a growing child is to add an anterior fusion at the middle part of the posterior fusion. Recently the use of pedicle screw instrumentation has seemed to be powerful enough fixation to prevent the “crankshaft phenomenon”. This may need more studies to pin down the actual batting averages.
In summary juvenile scoliosis has similarities to both Adolescent Scoliosis and Infantile or Early Onset Scoliosis. The young age and immaturity of the patients present special challenges.
As always, the
“ Art of Medicine ” is as important as the “ Science of Medicine ”.
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