BACKGROUND: Scoliosis is a sideways curvature of the spine that occurs most often during the growth spurt just before puberty. While scoliosis can be caused by conditions such as cerebral palsy and muscular dystrophy, the cause of most scoliosis is unknown. Most cases of scoliosis are mild, but severe scoliosis can be disabling. An especially severe spinal curve can reduce the amount of space within the chest, making it difficult for the lungs to function properly. (www.mayoclinic.com)
TREATMENT: While there are guidelines for mild, moderate and severe curves, the decision to begin treatment is always made on an individual basis. Factors to be considered include: sex, severity of curve, curve pattern, location of curve and bone maturity. Some treatments include: braces, surgery, and physical therapy. Spinal fusion surgery connects two or more of the bones in your spine (vertebrae) together with new bone. Surgeons may use metal rods, hooks, screws or wires to hold that part of the spine straight and still while the bone heals. The process is similar to what occurs when a broken bone heals. (www.mayclinic.com)
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Dr. Acosta: Frank L. Acosta, Jr., MD is the Director of Spine Deformity in the Department of Neurosurgery at Cedars-Sinai. His clinical practice focuses on conditions affecting the spine.
Dr. Acosta's research, which concentrates on the diagnosis and treatment of spine disorders, has been funded by the National Institutes of Health, Harvard Medical School and Howard Hughes Medical Institute grants and fellowships. He has published more than 40 papers in peer-reviewed journals and other publications, including Spine, Neurosurgery, Journal of Neurosurgery: Pediatrics, Journal of Neurosurgery: Spine, Neurosurgical Focus, Neurosurgery Clinics of North America, Surgical Neurology, Cancer Gene Therapy, Chest and Journal of the American Geriatric Society.
He is a member of AOSpine North America, the American Association of Neurological Surgeons and the Congress of Neurological Surgeons.Dr. Acosta earned his bachelor's degree in chemistry magna cum laude from Harvard College and his medical degree from Harvard Medical School. He completed an internship and neurosurgical residency at the University of California, San Francisco, and a fellowship in complex and reconstructive spine surgery at Northwestern University. (www.cedars-sinai.edu)
Dr. Frank Acosta, Director of Spinal Deformity at Cedar Sinai, discusses spinal surgery for patients with scoliosis.
What was your first impression of Julie Flores when you first saw her?
Dr. Acosta: My heart went out to her I thought she was such a sweet girl but she wasn't doing too well and I felt really bad for her.
What was wrong with her medically?
Dr. Acosta: She had severe scoliosis which is a curvature of the spine. Hers was an extreme case where her spine was essentially shaped like and S. She was really bent over both in terms of not being able to stand up straight and also kind of off to the side. One of the things that caused her the most amount of discomfort was the fact that because of her curvature her ribs were actually touching her hip and was just kind of digging in to that and she just was in excruciating pain from that and it was getting worse.
Is this the worst case you've ever seen?
Dr. Acosta: It was a pretty severe case yes, one of the worst cases I've ever seen. Usually people I've interventioned before it gets to this degree but she unfortunately didn't and so it was a pretty extreme case.
Her mother said she didn't have it because the doctor told her she was going to do spinal fusion. What would that do to her, her ribs are already touching?
Dr. Acosta: I think that she didn't have it because she has an underlying kind of dystonia where her muscles are really hyperactive that almost caused her spine to contort in to an S shape. And I think because of that no one really wanted to do anything about her scoliosis thinking that whatever they did would fail because of her muscle spasms. So by the time she got to me it had gotten totally out of control and there really wasn't a way that she could go on living like that.
Is there anything they could have been done before?
Dr. Acosta: Someone might have been able to do the surgery a little earlier on; it might have been a smaller surgery. But because of her underlying muscle spasm I think the concern was that whatever screws or rod they put in would fail because her muscles would just pull on it so much.
She had two operations, take me though the steps.
Dr. Acosta: When you're looking at her spine you're thinking how am I going to straighten that out and so the first thing you do is you have to put screws really up and down her spine. The first step is really putting all the screws up and down her spine and because her so twisted it's not as straight forward as putting in screws in a normal spine. So just instrumenting her spine took all of one day. We used computer navigation kind of like entropic CT scanner to put the screws in because they're in these weird very abnormal trajectories so that was really just all of one day. Then the second day was making some cuts in her bone, in her spine to be able to colostomies, to be able to loosen things up a little bit and then straighten out her spine as much as we could.
Did you think that this was going to be successful or there were times when you were not so sure?
Dr. Acosta: I mean I thought it was going to be successful otherwise I wouldn't have put her through it. But you know in surgery like this there are no guarantees. But I thought if we could get the instrumentation in and get these cuts done and loosen up her spine a little bit I thought she'd do well. And quite honestly for her I think any correction whether it's just the slightest bit would be a significant improvement in her quality of life just because she was so miserable.
You said other doctors wouldn't do this because of her muscle spasm, so why did you decide to do it?
Dr. Acosta: Well first of all we had to do something I think that I couldn't just let her go on like that or just kind of brush her off so I felt an obligation to do something. And also this is really a big interest of mine is scoliosis and spinal deformities so it's very challenging and it really kind of one of my professional passions and it got me sort of excited to take on this challenge. So I was cautiously optimistic when I went in to it but she had to have something done. She really couldn't go on like that.
She's standing straight right now and I think that's amazing what do you think about it?
Dr. Acosta: I think it's great. One of things that I like about spine surgery that's a very tangible thing so you can look at an x-ray after surgery and if it looks better you think I did a great job but for her I think that's absolutely true. Even more so when I think of her and just her as a person and her face and her smile, I'm just so happy for her. That's really what kind of makes it all worthwhile to think about her and the fact that she's doing so well.
Did you take any different approaches to surgery other than the traditional surgery for scoliosis?
Dr. Acosta: We used a lot of the navigation and she just had to have a lot of bone cuts much more than a standard scoliosis procedure because her spine was so rigid, it was so stiff. So we took those kinds of intraoperative things and she had to have full medical workup beforehand. I spent a lot of time with her and her mom counseling them about sort of the risks of surgery and really explaining to them that it's definitely a risky thing. She had a little bit of a rocky course afterward so I wanted to make sure they understood what they were getting themselves in to.
What was the rocky part?
Dr. Acosta: She developed pneumonia after surgery and had a tough time getting off of the ventilator but when she overcame that she kind of took off.
Is it just up from here, is she going to just get better, is there any chance she could get worse?
Dr. Acosta: I think she'll get better, a surgery like this where you have so many screws and rods and you rely on the bones to heal ultimately so she'll have bone up and down her spine connecting segments and ultimately once that happens successfully that's called a fusion she should be okay. But until that happens there's always a risk of some of the screws and rods failing or your herniating revision. So obviously the further along she goes after surgery the better off she is but there's still some potential landmines that we have to look out for.
So two surgeries in thirty nine years all she needed was to come to you?
Dr. Acosta: I guess you can put it that way, it didn't seem like that much but I'm just so happy for her she deserves to have a better life.
She calls you her angel, a miracle worker do you consider yourself something like that?
Dr. Acosta: That's very flattering, very humbling. I don't know if I'm an angel I would just say I was at the right place at the right time and had the right training to be able to do this and I'm just lucky, I'm very fortunate that I got to able to help her.
Why do you suppose so many people turned her away and let her live her life in misery?
Dr. Acosta: Her spine is extremely complex so I think there aren't that many people who know how to fix a spine like that so they sort of just referred her for maybe some pain medication or something like that. And I think the other thing she doesn't have any insurance, she doesn't have great insurance at all and her family comes from a very humble background. I think it's tough to find a specialist especially when you're starting off from such a disadvantage.
Was this a breakthrough surgery and will there be more, was the technology a breakthrough? Or was it just the combination of technology and you and her coming together?
Dr. Acosta: I think it's a combination of the technology and her being such a strong patient and the team that we have here in the nurshary department and all coming together and working hard to have such a great outcome. But I think there are very few places where those things come together to be successful. You certainly couldn't do this operation in a smaller community hospital you really need a lot of specialized physicians and equipment to do this.
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