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Straight Talk | Understanding Scoliosis and Thoracic Insufficiency Syndrome
Transcript: What are early onset scoliosis and thoracic insufficiency syndrome?
Robert Campbell, MD: The term scoliosis dates back 2,500 years to Hippocrates. It's Greek for "lateral curvature."
John Dormans, MD: The side-to-side curvature of the spine.
Robert Campbell, MD: You can see this lateral curvature on an X-ray taken from the front.
John Flynn, MD: Early onset scoliosis is the initiation of a significant spinal deformity in a very young child.
John Dormans, MD: It begins or becomes a problem usually prior to three to five years of age.
John Flynn, MD: Many of the real, true early onset scoliosis children that approach us are infants and toddlers.
John Dormans, MD: The incidence of regular or all comers scoliosis is about 1 in 1,000. For early onset scoliosis, it's much less common than that, maybe about 1 in 100,000. So they're very rare disorders.
Robert Campbell, MD: Most scoliosis being the teenage type which we term idiopathic adolescent scoliosis. But the early onset scoliosis is much more severe, it can cause more problems with pulmonary disease later in life.
John Dormans, MD: There are patients with chest wall deformity and that may or may not be syndromic, in that those patients will have different issues or problems to address. Some of the patients have malformations of vertebra.
Robert Campbell, MD: What's called "failure of formation" or "failure of segmentation." This is looking just at the spine. But this translates also to a chest problem. So, if you have missing ribs, you have failure of formation. Ribs that are fused, that part of the spine is usually fused adjacent to it, that's failure of segmentation. You can have a birth defect where the thorax is too narrow. That's also a volume depletion deformity, the most common being Jeune's Asphyxiating Thoracic Dystrophy.
Raymond Kleposki, MSN: The complexity can be as simple as you got a 10-degree curve, to something significant, you have a 100-degree curve and your lung doesn't move on the left side.
Robert Campbell, MD: Early curvature of the spine is a simple spine problem. Once deformity progresses and starts to warp the chest, then it starts to provoke early thoracic insufficiency syndrome.
Michael Nance, MD: Thoracic insufficiency is the lack of enough pulmonary function, or lung function, to support the patient.
Robert Campbell, MD: The inability to the thorax, which is the spine, rib cage, and the breathing muscles to support normal respiration or lung growth.
John Dormans, MD: So the patients have difficulty breathing and functioning in a normal way.
Michael Nance, MD: Many of the patients already are well into thoracic insufficiency and need support. Others are clearly headed that way.
Robert Campbell, MD: So the chest may do justice to lung growth the first year or so, but gradually, the lack of growth is a serious irreversible problem.
You look at an X-ray, and your spine is curved like this. But to look down inside the child, the thorax looks like a big oval. And then, as the spine curves, it shifts. But it shifts into the chest, and it starts to push on the lungs leaving it no room. And then the rib cage on the side, the spine rotates into, stops moving. So you not only lose volume, you also lose the chest's ability to aid respiration. And once we understand it better, and pioneer treatments to reverse it and pull it out to normal shape, normal volume, and normal function, then we'll have much better treatment for these children with early onset scoliosis and thoracic insufficiency syndrome.
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